Visceral pain results from the activation of nociceptors in the thoracic, pelvic, or abdominal organs. Studies have demonstrated a prevalence rate of 25% for intermittent abdominal pain and 20% for chest pain among adults. Approximately 25% of women suffer pelvic pain at some point in time.
Visceral pain has a temporal course and clinical characteristics that vary with the different phases of its evolution. In general, it commences as a diffuse and poorly defined midline body sensation in the lower sternum or upper abdomen. Different visceral organs project pain to specific somatic territories as exemplified by bladder pathologies to the perineum, the heart to the left arm, jaw, and neck, the left ureter to the left lower quadrant and groin. The diffuse nature of visceral pain and its consequent difficulty in localization has been ascribed to a low density of visceral sensory innervation and divergence of visceral nociceptive input within the central nervous system. As the pain evolves, pain may be referred to parietal structures within the same metameric field somewhat analogous to sclerotomal pain that occurs within the somatic system. As noted earlier, visceral somatic convergence of nociceptive input to dorsal horn pain projecting neurons and interneurons is a major mechanism of referred pain. Spatial discrimination of visceral pain entails referral to superficial (somatic) structures that produces secondary hyperalgesia of either superficial or deep body wall tissue. Referred pain may be sharper, better localized, and less often associated with autonomic manifestations than somatic pain.
Visceral pain is often associated with autonomic signs and symptoms that include:
1.Pallor
2.Hyperhidrosis
3.Nausea and vomiting
4.Alterations in temperature
5.Changes in heart rate and blood pressure.
The afferent fibers that innervate the viscera course to the CNS by autonomic sympathetic and parasympathetic nerves. A proportion of spinal afferents course with hypogastric, lumbar, colonic and splanchnic nerves and traverse prevertebral and paravertebral sympathetic ganglia to synapse in the thoracolumbar dorsal horn of the spinal cord.
Vagal afferents terminate in the brainstem and pelvic afferents synapse in the lumbosacral cord. These are major components of the parasympathetic innervation. Some visceral afferent fibers evoke conscious sensation while other afferences regulate autonomic functions and are not consciously perceived. These afferences are exemplified by hepatic chemoreceptors or pulmonary stretch receptors. Sensory afferents that innervate the GI tract and urinary tract have regulatory functions and contribute to evoked sensations of pain and fullness.
Visceral sensory afferents are primarily A-delta thinly myelinated (1-4μ) fibers and unmyelinated (1μ) C-fibers. There is a functional division of mechanosensitive visceral receptors into two physiological subgroups. High-threshold receptors that are in the heart, esophagus, colon, ureter, and uterus are only activated by noxious mechanical stimuli. Low-threshold receptors encode intensity and respond to a spectrum of innocuous to noxious stimuli. Under normal conditions, low-threshold AΒ-fibers only transmit innocuous mechanical modalities of sensation. Visceral organs are innervated by mechanically insensitive afferents that acquire mechanosensitivity following inflammation. They have been well characterized in rodent pelvic and splanchnic innervations as well as in cutaneous C-afferents by microneurography.
The convergence of visceral and somatic afferent inputs to the dorsal root ganglia and spinal cord dorsal horn pain transmitting neurons causes the referral of visceral pain to specific somatic sites on the body surface. It has been posited that viscerosomatic convergence may occur because of the low density of visceral afferent fibers that account for less than 10% of the total dorsal horn spinal cord afferent input. There is extensive divergence and intraspinal segmental distribution of visceral afferent terminals compared to cutaneous C-afferents that are usually restricted to one or two above or below segments due to their projections through Lissauer’s tract. Viscerosomatic convergence may also cause maladaptive neuroplasticity of central nociceptive processing in both the spinal cord and other components of the pain matrix. The convergence of nociceptive visceral and somatic inputs is one mechanism that causes concomitant visceral and somatic pain. There may also be viscero-visceral convergence in which pain from one organ can be referred to another organ.
There is bidirectional neuronal connectivity between cognitive, affective, and autonomic nuclei in the brain and neuro-endocrine nuclei, the enteric nervous system and the immune system. Visceral functions require complex regulation that involves CNS integration with the peripheral and enteric nervous system.
Vagal afferents project to the nucleus tractus solitarius in the medulla whose cell bodies are in the nodose ganglion. Convergent spinal visceral afferents synapse in the dorsal horn with second order pain transmitting neurons that project to higher centers via spinothalamic, parabrachial, and dorsal column pathways.
Lesion studies in animal models demonstrate:
1.Dorsal column lesions cause suppressed inhibition of exploratory behavior induced by noxious visceral stimulation and inhibition of potentiated visceromotor reflexes induced by colorectal distention after bowel inflammation;
2.The spinoparabrachial pathway is formed by projections from the superficial dorsal horn and is associated with autonomic and affective responses to painful stimuli.
The spinoparabrachial and nucleus tractus solitarious projections from vagal afferents synapse in the amygdala, hypothalamus and periaqueductal grey nuclei that are important in limbic and cognitive function. Spinothalamic projections from the deep dorsal horn along with a subset of lamina I neurons project to the contralateral ventroposterior lateral and ventroposterior medial thalamic nuclei. They in turn project to the insular and somatosensory cortices for sensory discrimination. Medial thalamic nuclei are primary for the affective and motivational components of pain processing projecting to the prefrontal and anterior cingulate cortex (ACC).
Descending modulation (descending nociceptive inhibitory controls in experimental protocols) is a neuronal system primarily from the periaqueductal grey of the midbrain and the rostral ventral medullary nuclei that has both inhibitory and facilitatory actions on the pain transmission neurons of the spinal dorsal horn. This modulatory system activates specific neurons of the RVM that can facilitate or suppress ascending nociceptive transmission. In animal pain models, it has been demonstrated that RVM neurons respond divergently to visceral and cutaneous stimuli. Dorsal horn pain transmission neurons that are depolarized by somatic stimuli can be inhibited by visceral inputs and vice versa. Human functional MRI studies demonstrate cortical activation after subliminal visceral stimulation and that cortical and subcortical circuitry can modulate brainstem pain processing.
Peripheral sensitization of visceral nociceptors occurs from persistent stimulation from inflammatory mediators (cytokines, chemokines, and prostanoids) which produces ectopic activity and lowered discharge thresholds. The chemical milieu at the site of injury is an “inflammatory soup” composed of immune cells (that secrete cytokines, chemokines and prostaglandins), mast cells, enzymes and protons all of which act upon nociceptive receptors. An important mechanism for peripheral sensitization of visceral nociceptors may be lowered firing thresholds of voltage-gated sodium channels of visceral pain afferents. Tetrodotoxin-resistant currents are present on small nociceptive afferents and have been identified on DRG neurons that innervate the colon. Nav1.8 channels have also been shown to be important in the sensitization of visceral nociceptors. The TRPV1 nociceptor is a non-selective cation channel that is gated by noxious heat, low pH and endogenous lipids and is widely expressed in visceral afferents. TRPV1 channels are upregulated in tissue samples from patients with irritable bowel syndrome (IBS) which has been correlated with pain intensity.
As occurs with somatic pain processing, persistent nociceptive drive causes central sensitization of pain transmission neurons in the visceral pain matrix. In patients with chronic visceral pain, secondary hyperalgesia in specific dermatomes has been demonstrated. Central sensitization has been clearly demonstrated in patients with esophageal and colon pathologies. Central changes derived from the affective-emotional and motivational circuitry, a top-down mechanism, is very important in the production of visceral hyperalgesia.
Chronic abdominal pain often arises from a local inflammatory process in the lower gastrointestinal tract that occurs with inflammatory bowel disease. Approximately 50 to 70% of patients with ulcerative colitis (UC) and Crohn’s disease (CD) present with abdominal pain.
The intestinal wall of the esophagus, stomach, small intestine, and colon are innervated by a dense neuronal network, the enteric nervous system. This intrinsic network is composed of enteric nerve cell bodies (sensory, motor, and interneurons) and their processes that form plexuses characterized by the myenteric plexus (Auerbach’s plexus) and the submucosal plexus (Meissner’s plexus). The enteric nervous system (ENS) regulates the functions of the gut that include:
1.Motility
2.Mucosal secretion
3.Absorption
4.Mucosal growth
5.Local blood flow
6.Immune function
There are bidirectional connections between the CNS and the ENS. Intrinsic enteric afferents release serotonin, substance P, and calcitonin gene related peptide (CGRP) that can initiate or exacerbate neurogenic inflammation that may sensitize gut nociceptive afferents.
The vagal nerve innervates the entire gut except for the transverse and distal portion of the colon. Its cell bodies are in the nodose ganglion whose central afferents synapse in the nucleus tractus solitarius of the dorsal medulla. Vagal afferents regulate feeding by modulation of upper gut reflexes that include gastric accommodation, gastric emptying, pancreatic secretion, and emesis as well as the perceptions of hunger, fullness, bloating, and nausea.
Experimental studies in rodents demonstrate three types of vagal fibers:
1.Mechanoreceptors
2.Tension receptors
3.Chemoreceptors that are activated by bile
Intramuscular vagal mechanical afferents are parallel to either the longitudinal or circular muscle layers and respond to muscle stretch. Intraganglionic laminar afferents innervate the connective tissue surrounding the myenteric ganglia and convey afferences that signal distention and muscle contraction. Tension-sensitive afferents are activated maximally at distensions within the physiologic range and respond to peristaltic contractions. It is posited that vagal afferents code the physiologic perception of mechanical stimuli while pain from distension of the upper GI tract is carried by splanchnic nociceptive afferents. Vagal nociceptors may be a component of pain from gastric acid. There is evidence that low intensity stimulation of vagal nociceptors may facilitate pain and high intensity stimulation may be inhibitory. The vagal nerve may regulate the cholinergic tone of the immune response of macrophages and immunocytes that is anti-inflammatory.
The pelvic nerves innervate the colorectum, the bladder, and the reproductive organs. Pelvic nerves contain afferents from serosal, mucosal, intralaminar, and intramuscular arrays that project to the DRG of spinal segments L5-S2. These afferences primarily detect circular stretch generated by low-intensity colorectal distension. The pelvic nerves are posited to be a component of normal physiology similar to the vagal afferent system (physiologic sensations such as urinary urgency and the desire to defecate) as well as acute rather than inflammatory pain that is the domain of splanchnic afferents.
Splanchnic nerves innervate the entire GI tract and visceral afferents in splanchnic nerves (demonstrated in rodents) from the colorectum synapse in T10-L2 spinal segments. Splanchnic afferent fibers traverse the celiac, superior, and inferior mesenteric ganglia where they form “en passant” synapses with efferent sympathetic neurons. Splanchnic afferents are posited to convey the major proportion of nociceptive afferences from the gut and signal different modalities of mechanosensibility. In the upper GI tract, splanchnic afferents code gastric mechano-nociception while gastric chemo-nociception is mediated by vagal pain transmission neurons. Splanchnic afferents mediate abdominal pain from the lower GI tract. In rodents, splanchnic afferents of the colon are located in the serosa (36%) and mesenteric membranes (50%) often associated with the mesenteric blood supply. Mucosal spinal afferents are found associated with submucosal blood vessels where they form varicose branching axons. The naked nerve endings of submucosal and mesenteric afferents respond to gut distortion during stretch or contraction at tissue destructive levels.
Extrinsic primary afferents that innervate the lower GI tract synapse with DH neurons of the thoraco- and lumbosacral spinal cord primarily in laminae I and II but also in laminae V and X. Ascending pathways of the contralateral anterior quadrant are composed of the spinoreticular, spinomesencephalic, spinohypothalamic and spinothalamic tracts. The spinoreticular tract synapses in the dorsal reticular nucleus and is thought to be involved in the affective-motivational components of visceral pain. The spinomesencephalic tract synapses in the PAG (periaqueductal grey) and other midbrain areas, which is extremely important in descending pain modulation. The spinohypothalamic tract in conjunction with the amygdala, medial thalamus, anterior cingulate cortex, locus coeruleus, and PAG modulate arousal, emotional and autonomic components of pain. Much of the sensory information conveyed by these pathways does not reach consciousness. The spinothalamic tract mediates pain, cold, heat, and a component of light touch. The posterolateral nucleus of the thalamus processes visceral innocuous and noxious inputs. The thalamic relays project to the pregenual anterior cingulate cortex (pACC), the midcingulate cortex, the insula, and the somatosensory cortex. Visceral sensation has a heavy representation in the secondary somatosensory cortex (SII). There is evidence from functional MRI studies that the cerebellum is activated during visceral nociception (as it is with painful somatic stimuli).
Recent studies support a role of the dorsal columns in visceral sensory transmission and nociception. The human limited midline myelotomy surgical procedure for intractable pelvic cancer pain is effective. The dorsal columns receive collateral branches of primary afferent fibers that arise from the dorsal root entry zone en route to the medulla as well as to the projecting axons from the deep lamina of the dorsal horn both of which synapse in the dorsal column nuclei. In experimental animals, there is input from the colon, the ureter, the pancreas, and the epigastric structures to dorsal column nuclei.
Visceral pain is modulated by descending projections from the nucleus magnus raphus, the PAG and the periventricular grey of the hypothalamus. In the cortex, the anterior cingulate area projects to the amygdala and the PAG. These descending projections can be either facilitatory or inhibitory to visceral ascending pain transmission. As is true of the somatic pain system the major circuitry of this system is from the PAG to the rostral ventromedial medulla (RVM) to the dorsal horn. Endogenous opioids are the major transmitter of this circuitry. Other well-characterized neurotransmitters of the system include:
1.Noradrenaline
2.Dopamine
3.Serotonin
The pACC projects to the pontomedullary networks that are posited to be inhibitory due to their high content of opioids. In contrast, stimulation of the RVM that projects to the dorsal horn includes the raphe magnus nucleus that is serotonergic increases dorsal horn excitability.
Visceral pain has many of the same underlying mechanisms that have been characterized for somatic pain. These include peripheral sensitization of nociceptors, central sensitization, and descending pain modulation. The specificity of segmental organization and its innervation density by nociceptors is less than that of the somatic system.
1.Approximately ten million Americans are estimated to suffer angina annually. There are approximately 500,000 new patients per year.
2.Angina is more often the presenting symptom of coronary artery disease in women than men with a female-to-male ratio of 1.7:1
3.Angina pectoris is caused by an imbalance between myocardial blood supply and oxygen demand
4.Definitions:
a.Stable angina:
i.Classic angina related to myocardial ischemia
ii.Chest pain or discomfort that is precipitated by a physical activity. There are minimal or no symptoms with rest. Symptoms recur with physical activity
iii.Precipitants of stable angina are cold weather, large meals and emotional stress
b.Unstable angina (“crescendo angina”):
i.Angina pectoris that is exacerbated by or occurs:
1.At rest or with minimal exertion and lasts for more than 10 minutes
2.It is severe and of recent onset
3.It has a crescendo pattern with more severe, prolonged or increase in frequency
ii.The pathophysiology is reduction of coronary blood flow due to transient platelet aggregation on normal endothelium, coronary artery spasm or thrombosis
iii.The process is initiated by atherosclerosis, evolves through an inflammatory stage to produce an active unstable plaque which may thrombose to cause myocardial ischemia
iv.Approximately 60% of unstable angina attacks occur between 10pm and 8am
c.Microvascular angina (cardiac syndrome X):
i.Microvascular angina is manifest by angina-like chest pain with normal epicardial coronary arteries
ii.Possible pathogenic mechanisms are endothelial dysfunction and decreased blood flow in heart small resistance blood vessels
1.In general, angina is seen in a man older than 50 years of age or a woman older than 60.
2.The chief complain is most often of chest discomfort rather than frank pain. It is described most often as pressure, heaviness, tightness, squeezing, burning, or a choking sensation.
3.Patients may localize the pain to the sternum with a clenched fist (Levine’s sign).
4.The pain is usually crescendo – decrescendo and most often lasts for two to five minutes.
5.Radiations are to either shoulder or both arms: most often medially to the humerus and to the medial forearm. Angina pain rarely crosses the wrist but if it does, it affects the ulnar side of the hand.
6.Less frequent radiations are to the interscapular areas, epigastrium, neck, jaw, teeth, back shoulder, and left medial humerus.
7.Rarely radiates below the umbilicus or above the mandible
8.Radiations to the trapezius muscle are more typical of pericarditis. Angina does not radiate to the trapezius muscles.
9.Typically angina is triggered by exercise, sexual activity, emotional stress, specific tasks, cold, or a large meal; it also occurs with rest and while the patient is recumbent (angina decubitus). The patient may be awakened from sleep with chest discomfort and dyspnea.
10. Angina equivalents are symptoms of myocardial ischemia that are more frequent in diabetic patients and the elderly. They include dyspnea, fatigue, hyperhidrosis, nausea, pallor, and presyncope.
1.Major angina risk factors:
a.Age > than 45 years for men and > 55 for women
b.Cigarette smoking
c.Diabetes mellitus
d.Dyslipidemia
e.Family history of premature cardiovascular disease
f.Hypertension
g.Kidney disease (microalbuminuria or GFR < 60 ml/min)
h.BMI > 30 kg /m2
i.Nonphysical life style
j.Severe emotional stress (prolonged)
2.Myocardial ischemia may be caused by:
a.Stenosis, spasm or acute occlusion of coronary vessels by emboli
b.Increased resistance of coronary and microvessels: blood flow is proportional to the radius of the artery to the fourth power
c.Reduced oxygen-carrying capacity of the blood from decreased oxygen tension and hemoglobin concentration
d.Rheological factors that increase blood viscosity and decrease arterial blood flow
3.Angina pain:
a.The extrinsic autonomic nerves access the heart via arterial routes and extend from the atria into the ventricular epicardium
b.In addition to the ganglia around the coronary sinus and nodal areas, extrinsic nerves synapse with cell bodies of intrinsic cardiac nerves. The majority of intrinsic nerve cell bodies are located on the epicardial surface and within epicardial fat pads. Their axons are connected to adjacent neurons that form networks delineated as “ganglionic plexuses” (GPs). There are both afferent and efferent neurons within the intrinsic cardiac nervous system the majority of whose neurons may be local circuit neurons. Integration of sensory inputs from the heart occurs both in the intrinsic cardiac nervous system as well as in mediastinal, middle cervical and stellate ganglia. Cardiac afferent receptors have their cell bodies in the dorsal root, the nodose ganglia, and the intrathoracic ganglia. There is no topographic spatial representation of the myocardium in these ganglia. It is proposed that during myocardial ischemia ATP is degraded to adenosine that diffuses into the extracellular space. There it causes both arterial dilatation and also pain by stimulating A1 receptors of the cardiac nociceptive afferents. If adenosine is given as an intravenous bolus to healthy volunteers and patients with ischemic heart disease, it provokes angina-like pain in the absence of EKG changes. Patients with asymptomatic myocardial ischemia are less sensitive to adenosine while those with Syndrome X are more sensitive. Intra-arterial adenosine provokes pain in the corresponding vascular bed.
1.Graded exercise stress testing
1.Chest radiography:
a.Is usually normal with angina but may demonstrate cardiomegaly in patients with cardiomyopathy, prior myocardial infarction, pericardial effusion
b.Myocardial perfusion scintigraphy
c.Coronary artery calcium (CAC) scoring by fast CT; electron-beam CT (EBCT) and multidetector CT (MDCT)
1.Acute pericarditis is seen in 1 per thousand hospital admissions
2.Chronic pericarditis occurs in approximately 6-10% of patients with advanced renal failure
3.Malignant disease is the most common cause of pericardial effusion with tamponade in Western countries. Tuberculosis is commonly causative in endemic areas
4.Acute pericarditis is more common in men than women and adolescents are affected more than young adults
5.The pericardium is composed of the parietal pericardium, its outer fibrous layer, and the visceral pericardium, an inner serous membrane that is composed of a single layer of mesothelial cells.
6.The fibrous layer attaches to the diaphragm, sternum and the costal cartilage
7.The visceral layer is adjacent to the heart and is attached to the epicardial fat. It reflects on itself to form the parietal pericardial layer
8.The pericardium contains between 20-50 ml of an ultrafiltrate of plasma. During tamponade there may be an additional 90-120 ml of pericardial fluid after which there is increased pericardial pressure which reduces stroke volume and cardiac output with consequent hypotension
1.Acute pericarditis is the most common pathological process in a wide spectrum of diseases
2.It’s cardinal manifestations are:
a.Pain
b.A pericardial friction rub
c.A pericardial effusion
d.Tamponade and paradoxical pulse
3.Chest pain:
a.Present in acute infection and autoimmune etiologies
b.Pain may be absent in insidious development of tubercular, post radiation, neoplastic and uremic forms
c.The pain is severe and typically retrosternal and left precordial. It is referred to the root of the neck, arms left shoulder and trapezius ridge.
d.It has a pleuritic component due to concomitant pleural inflammation that is sharp and exacerbated by coughing and change of position. Less frequently, the pain may be steady and constricting with bilateral medial arm radiations
e.The pain is relieved by sitting up and leaning forward and is made worse by lying down
f.An audible pericardial friction rub is present in 85% of patients. It is heard throughout the respiratory cycle.
g.Associated signs and symptoms include:
i.Low grade intermittent fever
ii.Dyspnea and tachypnea
iii.If tuberculosis is etiologic, fever, night sweats and weight loss occur in 80% of patients
1.Rheumatoid arthritis:
a.Pericarditis is associated with arthritis and pleuritis
b.Often while the patient is being treated with anti-inflammatory agents that include corticosteroids, gold and antimalarials
2.Chronic patients:
a.The heart rate may be bradycardic in association with tamponade, fever and hypotension that are due to autonomic involvement
3.Malignant pericarditis:
a.Symptoms develop over days to weeks
b.Dyspnea is often the most prominent symptom
4.Cardiac tamponade:
a.May present subacutely with:
i.Anxiety
ii.Dyspnea
iii.Fatigue
iv.Altered mental status
b.A waxing and waning course occurs with intermittently decompressing tamponade
c.Traumatic tamponade
i.Presents with acute dyspnea and altered mental status
5.Physical signs with pericardial inflammation of any cause which are:
a.Pericardial friction rub
b.Dyspnea and tachycardia (usually with associated effusion)
c.Ewart sign (dullness and bronchial breathing between the tip of the left scapula and the vertebral column)
d.Cyanosis
e.Altered mental status (if there is hypotension from tamponade)
f.Rare hepatomegaly and ascites
g.Tachycardia and cardiac arrhythmia
1.Acute pericarditis:
a.Serous pericarditis is most often caused by rheumatoid arthritis and systemic lupus erythematosus
b.Rarely these processes are associated with fibrous adhesions
c.Fibrous and non-fibrous pericarditis are the most frequent causes of pericarditis and include:
i.Myocardial infarction
ii.Post infarction
iii.Dressler’s syndrome:
1.Persistent low-grade fever
2.Pleuritic chest pain
3.Pericarditis often with a friction rub and or a pericardial effusion
4.Signs and symptoms occur 2-3 weeks following a myocardial infarction but may be delayed for several months
5.Usually subside in several days and is not often associated with pericardial tamponade
6.Posited to be an autoimmune inflammatory process (may be associated with an elevated sed rate)
2.Purulent pericarditis:
a.May arise from direct extension from an inflammatory chest process, hematogenous seeding or by lymphatic spread of infection
b.Infection during cardiotomy procedures particularly in immunosuppressed patients
c.Clinical manifestations include fever, chills and spiking temperatures
3.Specific causes of pericarditis include:
a.Infections (viral, bacterial and tuberculous)
b.Autoimmune disorders (RA, SLE, scleroderma, rheumatic fever)
c.Metabolic disorders (hypothyroidism, uremia and hypercholesterolemia)
d.Acute myocardial, Dressler’s syndrome and aortic dissection
e.Neoplasm
f.Drugs
g.Irradiation
h.Trauma
i.Cardiac surgical procedures
j.Idiopathic
4.The pericardium is acutely inflamed and is infiltrated with polymorphonuclear leukocytes associated with vascularization
5.There is an associated fibrous reaction with exudates and adhesion. There may also be a serious or hemorrhagic effusion
1.CBC, serum electrolytes, blood urea nitrogen and creatinine, erythrocyte sedimentation rate and C-reactive protein
2.Cardiac biomarkers
3.Lactate dehydrogenase
4.SGOT and AST
5.EKG:
a.Typically demonstrates ST elevation in all leads
6.Echocardiography
7.Chest radiograph is helpful in patients with effusions larger than 250 ml
1.Definition:
a.Acute aortic syndrome (AAS) is the acute presentation of patients with “aortic pain” that includes:
i.Aortic dissection
ii.Intramural hematoma
iii.Penetrating atherosclerotic ulcer
iv.Aneurysmal leak
v.Traumatic transection
1.Evidence of aortic dissection is seen in 1 to 3% of autopsies
2.The incidence is estimated to be 5 to 30 patients per 1 million of the population
3.Aortic dissection is more frequent in black than white patients and males than females
4.Approximately 75% of dissections are seen in patients 40 to 70 years of age, the peak incidence is between 50 to 65 years; patients with connective tissue disorders present at an earlier age
1.Medical:
a.Hypertension:
i.A catecholamine surge or underlying hypertension
b.Hypotension:
i.Excessive vagal tone
ii.Cardiac tamponade
iii.Rupture of the dissection
c.Blood pressure inter arm differential of more than 20 mmHg
d.Signs of cardiac tamponade:
i.Distant or soft heart sounds
ii.Hypotension
iii.Pulsus paradoxus
iv.Jugular venous distension
v.Kussmaul sign (paradoxical rise in jugular venous pressure on inspiration)
vi.Superior vena cava syndrome (compression from a large distorted aorta)
vii.Right coronary artery ostial dissection may cause acute myocardial infarction
2.Neurologic deficits:
a.Neurologic signs and symptoms occur in approximately 20% of patients and include:
i.Syncope and altered mental status
ii.Chest pain that is described as severe, acute, ripping and in some patients migratory
iii.Radiation to the anterior chest or neck from the ascending aorta; rarely to the face and jaw
iv.Pain radiates to the back when the pathology is in the descending aorta; flank pain with renal artery involvement
v.There is no pain in approximately 10% of patients
vi.Ascending aorta dissection into the great vessels causes a cerebrovascular accident with:
1.Hemispheric symptoms of hemiplegia, hemianesthesia and aphasia (if left carotid artery is involved)
2.Horner’s syndrome with carotid involvement
3.Dysphagia
4.Syncope
5.Difference in upper extremity blood pressure
6.Pulse deficits
7.Anxiety
8.Feelings of impending doom
9.Paraparesis (descending aortic dissection)
1.An intimal tear that penetrates the aortic media
2.The entry tear occurs at the sites of greatest wall tension which is usually a few centimeters close to the aortic valve on the right lateral wall of the ascending aorta (type A dissection); it may also occur where the aorta is fixed near the ligamentum arteriosum of the descending aorta
3.Blood under high pressure dissects through the media to form a second or false channel that parallels the true lumen
4.The re-entrance tear allows blood to circulate through the false lumen
5.Organ ischemia occurs when the dissection flap occludes an aortic branch artery
6.Many aortic dissections are not associated with atherosclerosis as the fibrosis and calcification of that process may limit the progression of the dissection
7.Associated or underlying medical conditions include:
a.Marfan syndrome
b.Ehlers-Danlos type IV
c.Adult polycystic disease
d.Turner syndrome
e.Osteogenesis imperfecta
f.Bicuspid aortic valve
g.Noonan syndrome
h.Coarctation of the aorta
i.Homocystinuria
j.Familial hypercholesterolemia
k.Familial aortic dissection
l.Annuloaortic ectasia
m.Cystic medial necrosis
8.Acquired causes of aortic dissection:
i.Surgical procedures:
1.Aortic and mitral valve replacement
2.Coronary artery bypass graft surgery
3.Cardiac catheterization
4.Percutaneous transluminal coronary angioplasty
ii.Syphilitic aortitis
iii.Deceleration injury with related blunt chest trauma
iv.Cocaine
1.Leukocytosis and decreased hemoglobin
2.Elevated BUN and creatinine (renal artery involvement)
3.Elevated myocardial creatine kinase, myoglobin and troponin I and T levels (myocardial infarctions)
4.Lactate dehydrogenase (hemolysis of RBCs in the false lumen)
5.Smooth muscle myosin heavy-chain (90% sensitive and 97% specific) in the first 24 hours
6.Fibrin degradation products
1.Chest radiography:
a.Widened mediastinum is the classic finding:
2.Other radiographic features include:
a.Pleural effusion
b.Left apical cap
c.Tracheal deviation to the right
d.Depression of the left mainstem bronchus
e.Esophageal deviation
f.Loss of paratracheal stripe
3.Spiral (helical) CT has a higher rate of detection and better resolution than incremental CT scanning
4.CT angiography delineates detailed anatomic definition of the dissection as well as plaque formation
5.MRI and transesophageal echocardiography are often limited by expense, availability and the need for MR compatible monitoring equipment
6.In many institutions contrast enhanced CT is utilized as the imaging procedure of choice as it is capable of providing 3D reconstructed images and assesses other thoracic structures
1.Definition:
a.PAU is a focal defect or lesion that occurs at the site of an intimal atherosclerotic plaque
b.Patients are older and often have significant cardiovascular and pulmonary comorbidities. The most common patient profile is an elderly man with hypertension, coronary artery disease who smokes
1.Most often PAU involves the descending aorta (rarely it occurs in the ascending aorta) and may cause intramural hemorrhage, aortic aneurysm, pseudoaneurysm or dissection
2.It may be asymptomatic and is often incidentally diagnosed usually when confined to the intimal layer
3.The second stage of the lesion is when the atheromatous ulcer penetrates through the elastic lamina into the media
4.The major symptom is severe acute chest pain that radiates to the interscapular area
1.The most common location is in the descending aorta
2.Progressive intimal erosion leads to pulsatile blood that enters the media that results in hemorrhage
3.Erosion of the aortic vasa vasorum by the ulcer causes intramural hemorrhage, aortic dissection and aortic rupture
4.PAU represents 2 to 7% of acute aortic syndrome
1.Spiral computed tomography CT provides two- and three-dimensional image reconstruction
2.Penetration into the adventitia causes:
a.Pseudoaneurysm
b.Aneurysm
c.Aneurysm rupture
1.Intramural hematomas are approximately 10 to 30% of all patients with acute aortic syndrome
2.By definition it is a variation of dissection in which blood collects within the aortic media without an intimal flap
3.If the IMH involves the aortic arch and ascending aorta, its complications and mortality rates are high and are similar to type A aortic dissections. Type B IMH are more benign
4.Symptomatic IMH has an incidence of rupture at presentation of up to 38%
1.The natural history of IMH and its rate of progression are not well delineated but demonstrate that they may rupture, stabilize, regress or progress to dissection or aneurysm formation
2.Risk factors that predict IMH type A progression to dissection include:
a.A dilated aorta
b.An ascending aorta diameter of >5cm
c.Less frequently PAU causes pleural and pericardial effusion, aortic regurgitation and mediastinal hematoma
3.Chest pain that is severe, ripping and acute:
a.Radiates to the anterior chest or neck if in the ascending aorta
b.If in the descending aorta the pain radiates to the interscapular area of the back
c.Progression is unpredictable and sudden and may present as an acute aortic dissection
1.Blood collects within the aortic media due to rupture of the vasa vasorum which are located in the media
2.IMH may also occur from hemorrhage within an atherosclerotic plaque
3.IMH occurs close to the adventitia which causes its high rate of rupture (35%) compared to aortic dissection
4.The hemorrhage may extend toward the lumen to cause a dissection
5.Associated pleural, pericardial and mediastinal hemorrhage are posited to occur from increased aortic wall permeability
1.Contrast enhanced computed tomography
2.The concept of “a micro-tear” that cannot be visualized with conventional imaging modalities is controversial
3.“Echo-free space” demonstrated by TEE and “focal contrast enhancement” on ultra-fast CT are being evaluated
4.C-reactive protein may be a method to predict adverse complications with acute aortic intramural hematoma
1.Aortic aneurysm leak:
a.The acute aortic syndrome can be caused by an acute expansion of an aneurysm
b.Progressive vessel dilatation causes increasing vessel wall tension (LaPlace’s Law) with more rapid dilatation and risk of rupture
2.Traumatic aortic transection:
a.The aortic transection is caused by rapid deceleration forces or direct chest blunt trauma
b.Most patients succumb immediately
c.The location of the transection is just distal to the left subclavian artery of the aortic isthmus where the aorta is fixed by the ligamentum arteriosum
d.Rarely patients present with an incomplete rupture that causes dissection, IMH or pseudo-aneurysm formation
e.The pain is dependent on location of the pathology
1.The incidence in the USA is 1 per 1000 persons per year; it is the third commonest cause of death in hospitalized patients
2.An acute pulmonary embolus is situated centrally within the vascular lumen or if it occludes a vessel (vessel cut off sign). It often causes distention of the involved vessel
3.Chronic emboli are eccentric and are contiguous with the vessel wall. It reduces the arterial diameter more than 50% and there is evidence of recanalization within the thrombus; an arterial web is present
4.A pulmonary embolus is characterized as central or peripheral depending on the branch occlusion. It is massive when it involves both pulmonary arteries or when it causes severe hemodynamic compromise
1.The classical presentation is the abrupt onset of pleuritic chest pain, dyspnea and hypoxia
2.Commonly patients have complained of bothersome symptoms prior to sudden death
3.Most often clinical signs and symptoms are non-specific and include dyspnea, tachypnea and /or chest pain
4.A cramp in the calf several days prior to an embolic event; clinical signs and symptoms of deep vein thrombosis
5.Heart rate > 100 beats / minute
6.Immobilization > 3 days; surgery within 4 weeks
7.Prior DVT or pulmonary embolus
8.Hemoptysis
9.Cancer
10. Pain:
a.Pleuritic chest pain is more common with small peripheral emboli
b.Patients with pleuritic pain may have mechanical hyperalgesia and allodynia in the somatic innervation over the area of lung peripheral infarction
11. Atypical signs and symptoms include:
a.Seizure
b.Syncope
c.Abdominal pain
d.New onset atrial fibrillation
e.Flank pain
f.Delirium in elderly patients
1.Gas exchange abnormalities include hypoxemia (decreased arterial pO2) and increased alveolar-arterial O2 tension
2.Increased anatomic dead space
3.Increased pulmonary vascular resistance due to obstruction or platelet release of serotonin and other vasoactive mediators
1.Blood screen:
a.A hypercoagulable workup if there is no obvious cause for an embolus
b.D-dimer testing
c.Arterial blood gas
d.Serum troponin levels
e.Brain natriuretic peptide
1.Multidetector-row CTA (MDCTA):
a.MDCTA is the criterion standard
b.Pulmonary angiography if MDCTA is unavailable
2.MRI:
a.Gated spin-echo techniques reveal increased signal intensity within the pulmonary artery
1.A pneumothorax is air or gas that accumulates in the pleural cavity
2.A pneumothorax may be spontaneous or is caused by trauma to the lung or chest wall. It is also divided into tension and non-tension subgroups. Non-tension pneumothorax is divided into open or closed (partial) type
3.Spontaneous pneumothorax is classified as either primary or secondary:
a.Primary spontaneous pneumothorax is seen in young thin males and is caused by ruptured pleural blebs or bullae
b.Secondary spontaneous pneumothorax occurs in older patients who have emphysema, asthma, chronic infections, lung cancer, and congenital diseases. These include cystic fibrosis, catamenial pneumothorax, and lymphangioleiomyomatosis.
1.The age-adjusted incidence of PSP is between 7.4 to 18 patients per 100,000 of the population per year in males and from 1.2 to 6 patients per 100,000 of the population for females
2.It is primarily a disorder of tall, thin males who are between 10 and 30 years of age. It is rarely seen in patients over 40 years of age
3.Approximately 10% of patients have a family history
4.Some patients have been described with mutations of the FLCN gene that maps to chromosome 17p11.2 that causes Hogg-Dube syndrome (hair follicle hamartomas, kidney tumors, and spontaneous pneumothorax)
5.Smoking is a precipitating factor
a.PSP usually occurs at rest
b.Presents with the acute onset of local pleuritic chest pain:
i.The pain may be mild to severe
ii.Its quality may be sharp or it has been described as a steady ache. It often resolves within 24 hours while the pneumothorax persists
iii.The pain is associated with shortness of breath
iv.The physical examination of large pneumothorax reveals:
1.Decreased breath sounds
2.Decreased chest wall movement
3.Hyper-resonance with percussion
4.Decreased tactile fremitus
These findings are seen if free air occupies more than 15%-20% of the hemithorax. There may be associated reflex tachycardia.
5.Tension pneumothorax causes severe tachycardia, cold hyperhidrosis, hypotension and/or cyanosis
1.It is posited that most patients have had the spontaneous rupture of a subpleural bleb or bulla
2.An alternative mechanism may be an increase in pleural porosity that occurs with inflammation. Only a subgroup of patients are found with blebs or bullae at surgery
3.Spontaneous hemopneumothorax are 0.5% to 2.6% of patients with spontaneous pneumothorax. It consists of air and blood > 400 ml. It is posited that the bleeding is caused by aberrant blood vessels that arise from the chest wall and grow into the pleural lesions
1.Upright posteroanterior chest radiograph which demonstrates a pleural line with or without an air-fluid level
2.CT of the chest is utilized to delineate a small pneumothorax (less than 15% of the hemithorax area). It may also delineate the number, size and location of bullae / blebs, pleural adhesions, fluid and underlying pulmonary disease
1.Pleurodynia is a rare complication of coxsackievirus B infection. It may also occur with ECHO viruses
2.Coxsackievirus B is an RNA Enterovirus that usually causes an asymptomatic or short duration upper respiratory or gastrointestinal infection. It rarely causes meningitis and carditis
1.Pain:
a.The onset of chest pain is acute
b.Attacks cause severe, intense pain that is paroxysmal, lasts seconds to a minute that occur as attacks. They are associated with dyspnea
c.The thoracic pain is usually located over the lower ribs and is unilateral. It can also occur over the back or substernally
d.Between attacks patients may suffer a constant, dull, pleuritic type chest pain
e.Attacks last for 3 to 5 days and rarely persist longer than a month. There may be remissions and exacerbations
2.Associated signs and symptoms:
a.Constitutional symptoms that include headache (50%) of patients, fever and malaise
b.Gastrointestinal symptoms of nausea, vomiting and diarrhea occur in 50% of patients
c.Abdominal pain, primarily epigastric particularly in children
d.Testicular pain (orchitis) in 10% of males
1.Striated muscle is the primary target of the Coxsackie B virus and is the cause of the severe chest pain. A minority of patients have pleuritis
2.In patients with pleurodynia, the intercostal muscles are involved which is associated with increases in the serum creatine kinase
3.The virus has an incubation time of 1 week in the GI tract and then spreads hematogenously
4.The major target of the virus is skeletal muscle but it also can infect the CNS (meningitis or encephalitis) or the myocardium and pericardium
1.The virus can be recovered from the stool or pharynx for up to 2 weeks following resolution of symptoms
2.Reverse transcriptase (PCR) is now replacing viral culture and neutralization assays
Superior sulcus tumors have a wide spectrum that are often painful as they invade the apical chest wall, the lower portion of the brachial plexus, the first rib, vertebrae, subclavian vessels or stellate ganglion. In general, they cause severe and unrelenting shoulder and arm pain in addition to C8, T1 and T2 thoracic spinal nerve involvement. If the tumor invades the intercostobrachial nerve (that arises from the medial cord of the brachial plexus), there is pain in the axilla which radiates to the anterior chest wall and sternum.
The anatomy of the region that is involved by Pancoast tumors is delineated by the thoracic inlet that is divided into three compartments. The first rib is the base of the thoracic inlet that is defined anteriorly by the sternum and posteriorly by the attachment of the anterior scalene muscle on the first rib. The compartment contains the sternocleidomastoid and omohyoid muscles, the subclavian and jugular veins and the scalene fat pad. The middle compartment lies between the anterior and middle scalene muscles and is crossed by the subclavian artery, phrenic nerve, and trunks of the brachial plexus. The posterior compartment is located behind the middle scalene muscle and contains the posterior scalene muscle, posterior scapular artery, stellate ganglion, sympathetic chain, long thoracic nerve, accessary nerve, neural foramina and vertebral bodies
1.Shoulder pain is often the initial symptom
2.Progression of the growth causes paresthesias and burning pain in C8, T1 distributions often accompanied by weakness of ulnar innervated muscles and atrophy of intrinsic hand muscles (primarily T1 root)
3.A Horner’s syndrome is seen in 15-50% of patients (ptosis, meiosis, anhidrosis and apparent enophthalmos)
4.If the medial cord is involved, there is frequent invasion of the intercostobrachial nerve with axillary and anterior chest pain
1.A wide spectrum of disorders cause Pancoast syndrome and include primary neoplasms of the lung and pleura, metastases, hematologic malignancies, inflammatory and infectious processes
2.The definition of a Pancoast tumor as proffered by the American College of Chest Physicians is:
a.A lung cancer that arises from the apex of the lung that invades structures of the apical chest wall at the level of the first rib or above. A Horner’s syndrome or arm pain is not required. The lesions frequently invade the brachial plexus, subclavian vessels, or spine.
3.The great majority of Pancoast tumors are bronchogenic carcinomas with adenocarcinoma the histologic type
4.Small cell bronchogenic tumors in this location are rare (less than 5%)
5.Pancoast tumors often demonstrate different biology than other non-small cell lung tumors as demonstrated by local invasion rather than lymphatic or hematogenous metastasis
1.Routine CBC, liver enzymes, coagulation parameters and electrolytes
2.Elevated oncofetal carcinoembryonic antigen and beta-2 microglobulins are often associated with small cell lung cancers and are related to the stage of the disease
3.Molecular analysis:
a.Oncogenes associated with lung cancers include:
i.K-ras
ii.C-myc
iii.TP53
iv.HER-2/neu
4.Tumor markers that include bombesin, neuron-specific enolase and other peptides occur with small cell cancers and are related to the stage of the disease and its state of differentiation
5.Percutaneous transthoracic needle biopsy is definitive for diagnosis
6.Rarer tumors that may involve the thoracic inlet include:
a.Askin tumor
b.Ewing sarcoma and neurectodermal tumors of the mediastinum
c.Giant solitary fibrous tumor of the pleura
d.Langerhans cell histiocytosis
1.Chest radiographs may demonstrate a mass or pleural thickening at the apex of the lung that is associated with invasion or destruction of ribs or vertebrae
2.CT of the chest delineates the apical mass and its invasion of the bony thorax and the other structures of the thoracic inlet
3.MRI is the best modality to characterize the brachial plexus, subclavian vessels, spine and the neural foramina
1.The incidence of malignant mesothelioma reported from Germany is 20 patients per million of the population
2.Due to extensive removal of asbestos the incidence is expected to decrease between 2015-2030
3.Greater than 80% of mesotheliomas arise from the mesothelial or submesothelial cells of the pleura
4.Approximately 80% of pleural mesotheliomas occur in men
5.It is a disease caused by occupational asbestos exposure
6.Asbestos is the commercial term for:
a.Different forms of naturally occurring mineral fibers that include:
i.“White asbestos” and amphibole asbestos (crocidolite, amosite, anthophyllite, actinolite and tremolite)
7.Non-asbestos-related mesothelioma comprises 10% to 20% of afflicted patients. Mesotheliomas may be caused by zeolite, an asbestos-like mineral fiber. Other possible etiologic factors include SV-40 viruses, genetic predisposition, and recurrent infection.
1.Dyspnea is the first symptom in 90% of patients
2.It is a disease of the elderly with a median age of 74 years:
a.Approximately 15% of patients are younger than 40 years of age
b.Other series demonstrate a smaller percentage of 2% to 5.8% of patients under the age of 40 years
c.There is no sex difference in younger patients.
3.Patients usually develop mesothelioma 20 to 40 years after asbestos exposure
4.Non-pleuritic chest wall pain is a common early complaint. It is thought to arise from irritation of the intercostal nerves or by infiltration of the malignancy into the chest wall
5.A proportion of patients are asymptomatic diagnosed by a pleural effusion by x-ray or physical examination
6.Rarer manifestations of malignant pleural mesothelioma include:
a.Phrenic nerve involvement
b.Irritative cough
c.Paraneoplastic signs and symptoms
d.Spontaneous pneumothorax
e.Rare to have symptomatic metastasis
1.Mesothelioma is subdivided into epitheliod, biphasic and sarcomatoid subtypes by their histomorphological growth pattern
2.The pleural surfaces are seeded with malignant mesothelioma cells that form nodules. As the disease progresses the pleural space is covered and the malignancy invades the chest wall, mediastinum and diaphragm
3.Approximately 90% of patients present with a pleural effusion. Only 32% are diagnostic. Thoracoscopically guided biopsy is diagnostic in 98% of patients.
1.A new marker is serum mesothelin protein (SMRP) which is elevated in 84% of patients. Megakaryocyte potentiating factor may also be elevated
1.Chest CT:
a.Obliteration of the diaphragm
b.Nodular pleural thickening
c.Decreased size of the involved thorax
d.Radiolucent sheetlike encasement of the pleura
e.A loculated effusion with opacification of the pleura
1.Pleural vasculitides:
a.Asbestos exposure is associated with the onset of microscopic polyangiitis that has been described in the pleura
2.Severe chest wall toxicity from cryo ablation in the setting of prior ablative radiotherapy:
a.A consequence of focal therapy for metastatic cancers
3.Pleuritic chest pain from portal hypertensive gastropathy (PHG):
a.PHG may present with chest pain and hematemesis
1.The esophagus is divided into cervical, thoracic and abdominal sections
2.Its body is composed of an inner circular and an outer longitudinal array of muscular layers. The proximal esophagus is composed of striated muscle that transitions to smooth muscle in its distal 2/3s. The proximal esophagus contains the upper esophageal sphincter which is composed of the cricopharyngeus muscle (cranial nerve IX) and the thyropharyngeus muscle (from the cervical plexus)
3.The distal esophagus is located to the left of the midline and, through the esophageal hiatus in the diaphragm, passes into the abdomen. The right crus of the diaphragm forms a sling around the esophagus from right and left pillars that narrow it when the diaphragm contracts
4.The phrenoesophageal ligament is the reflection of the subdiaphragmatic fascia onto transversalis fascia of the anterior abdominal wall that also encircles the esophagus. The phrenoesophageal ligament marks the esophogastric junction that lies in the abdomen and forms the angle of His. This angle and the length of the intra-abdominal portion of the esophagus determine the closure of the esophagus when there is increased intragastric or abdominal pressure
5.The lower esophageal sphincter or the distal esophageal high-pressure zone is the most distal segment of the esophagus (3.5 cm in adults) and is crucial in preventing GERD. GERD can be caused by malfunction of any of the above structures
6.It is estimated that 25 to 40% of Americans have symptomatic GERD at some time during their lifetime
7.GERD occurs in all age groups, has no sex preference and increases in people over 40 years of age
1.Burning pain in the lower sternal area after eating
2.Regurgitation
3.Dysphagia
4.An acidic taste in the mouth
5.Increased salivation
6.Coughing
7.Hoarseness
1.Major mechanisms for acid reflux include:
a.Poor esophageal motility with decreased clearance of acidic material
b.A dysfunctional lower esophageal sphincter which allows gastric juice to reflux upwards into the esophagus
c.Delayed gastric emptying
2.Associated complications of GERD:
a.Esophagitis:
i.Esophageal mucosal damage which occurs in approximately 50% of patients
ii.Stricture:
1.An advanced form of esophagitis due to circumferential fibrosis. They are usually demonstrated in the mid-to-distal esophagus
iii.Barrett esophagus:
1.The most serious complication of long-standing and severe GERD. It is defined by the metaplastic conversion of the normal distal squamous esophageal epithelium to columnar epithelium which may demonstrate dysplasia
2.Barrett esophagus with intestinal type metaplasia has malignant potential risk for the development of esophageal adenocarcinoma (30-40 times that of normal individuals).
1.Upper GI endoscopy and manometry
2.Manometry delineates lower esophageal sphincter pressure and determines motility disorders
3.Less frequently used studies:
a.24-hour pH probe test
b.Upper GI series
c.Nuclear medicine gastric emptying studies
4.Micro RNA expression of exfoliated tongue cells
a.Validation demonstrates a sensitivity of miR-203 at 91% and a specificity of 87%
1.The esophagus is composed of two muscle types. It is 25cm in length and has an upper proximal and distal sphincter. The upper sphincter is composed of the cricopharyngeus and thyropharyngeus muscles. The lower esophageal sphincter is composed entirely of smooth muscle
2.The proximal esophagus is composed of striated muscle; the mid esophagus contains a graded transition of striated to smooth muscle while the distal esophagus is solely smooth muscle.
3.The longitudinal muscle shortens the esophagus and the circular muscle causes lumen-occluding ring contractions
4.Peristalsis is the sequential coordinated contraction wave that travels the length of the esophagus and propels the food bolus to the stomach
5.The lower esophageal sphincter is relaxed during swallowing and remains open until the peristaltic wave passes. It then contracts and resumes its basic tone
6.Primary peristalsis is defined as a peristaltic wave triggered by the swallowing center and travels at a speed of 2cm/s (correlates with manometry recorded contractions)
7.A secondary peristaltic wave is caused by esophageal distention from a retained food bolus, refluxed material or swallowed air
8.Tertiary contractions are simultaneous dysfunctional contractions that are not peristaltic. Their physiologic function is not known.
1.The incidence of achalasia is approximately 1 per 100,000 persons per year
2.It is a primary esophageal motility disorder characterized by the absence of esophageal peristalsis and decreased relaxation of the lower esophageal sphincter
3.The incidence of achalasia in the USA is 1-3 patients per 100,000 of the population per year
1.Progressive dysphagia for both liquids and solids
2.Chest pain characterized by a squeezing quality located retrosternally that may radiate to the neck, jaw, arms, or back. The chest pain may be exacerbated by food and may awaken the patient from sleep
3.A sensation of heart burn is reported by 30% of patients
4.Regurgitation of food retained in the proximal esophagus particularly at night. Patients may utilize multiple pillows or sleep upright in a chair
5.Symptoms may be present for a long time prior to diagnosis
6.Emotional stress and rapid eating exacerbate all symptoms
7.Slight weight loss is common
1.The major cause of achalasia is the loss of ganglion cells from the wall of the esophagus that starts at the lower esophageal sphincter and progresses proximally. The degree of ganglion cell loss is concomitant with the disease duration. Immunohistochemical analysis reveals loss of nitric oxide synthase and vasoactive intestinal peptide (VIP) in inhibitory nerves. Inflammatory cells and inhibitory fiber loss are seen in the myenteric plexus. Acetylcholine intrinsic nerves are lost in the esophageal body. Wallerian degeneration may be demonstrated within the vagus nerve and dorsal motor nucleus. The circular muscle layer at the lower esophageal sphincter may be thickened. Physiologically, achalasia is most correlated with the loss of inhibitory nerves of the lower esophageal sphincter (LES) that decreases its ability to relax causing relative obstruction at the gastroesophageal junction.
2.Chagas disease secondary to Trypanosoma cruzi:
a.Is transmitted by a reduviid bug bite
b.Is endemic in Central and South America (rarely has been described as far north as Texas)
c.Widespread ganglionic cell loss involves the esophagus, heart, gut, urinary and respiratory tract. Symptoms and signs may take years to develop
3.Rare causes of achalasia:
a.Familial adrenal insufficiency with alacrima
b.Pseudoachalasia from gastroesophageal junction obstruction:
i.Tumor
ii.Neurofibromatosis
iii.Pancreatic pseudocyst
c.Infiltrative disorders causing pseudo-obstruction include:
i.Amyloidosis
ii.Sphingolipidosis
iii.Eosinophilic gastritis
iv.Sarcoid
d.A possible autoimmune mechanism has been suggested due to single nucleotide polymorphisms (SNPs) in the histocompatibility region of chromosome 6 that is associated with other autoimmune disorders
1.Chest radiographs:
a.Dilated esophagus with a sigmoid shape
b.An air-fluid level
c.Widened mediastinum
d.Absence of a gastric air-bubble
2.Esophagography:
a.Tapering of the LES causing the “bird-beak” appearance
b.Loss of peristalsis and transient stasis above the gastroesophageal junction
c.Epiphrenic diverticula are noted (occasionally) above the LES
1.Manometry:
a.Elevated LES pressure greater than 40 mm Hg in > 60% of patients
b.Aperistalsis with stasis of food and esophageal dilatation
c.Nonperistaltic isolated contractions or low amplitude simultaneous contractions of the esophageal body
d.High-amplitude >60 mmHg simultaneous contractions termed vigorous achalasia may be an early stage of the disorder
1.Nutcracker esophagus is a motility disorder of the esophagus in which contractions of its smooth muscle are normal in sequence but are too long or occur at increased amplitude
2.The disorder affects patients of all ages and is most common in the sixth and seventh decades
3.The disorder is not progressive and is not associated with complications
1.Chest pain is the hallmark symptom that is similar to that of achalasia and may mimic angina. The mechanism of the pain may be muscle ischemia, luminal distension or abnormal visceral sensation
2.Dysphagia for solids and liquids that may not be related to pain, is often intermittent and is not associated with weight loss
3.Patients may present with a sudden obstruction of the esophagus after eating food (“steakhouse syndrome”)
4.The illness does not progress or have complications
5.A subgroup of patients are asymptomatic but may demonstrate the same motility findings as “nutcracker esophagus”
1.There are no specific anatomical correlations but the disorder has been associated with the metabolic syndrome
2.The pathology may be related to alterations between excitatory and inhibitory transmitters in the distal esophagus
1.Endoscopy is typically normal although abnormalities associated with GERD may be present
2.Manometry:
a.Peristaltic contractions with an amplitude of greater that 180 mmHg taken 3 and 8 cm above the lower esophageal sphincter are demonstrated
1.Diffuse esophageal spasms are manifested by contractions that are of normal amplitude but are uncoordinated, simultaneous or too rapidly propagated
2.Hypertensive and nutcracker esophagus have coordinated sequential contractions whose amplitude is excessive (see above)
3.Jackhammer esophagus or hypercontractive esophagus is termed when contractions are of very high amplitude involve a major portion of the esophagus with a prolonged duration. It has a jackhammer appearance on high-resolution manometry
1.Esophageal spasm accounts for less than 10% of noncardiac chest pain:
a.The pain is usually retrosternal and may radiate to the back, neck, arms and jaw
b.It may be more severe than angina
2.Globus (a feeling that food or a object is trapped in the throat)
3.Dysphagia is seen in 33% to 66% of patients with diffuse esophageal spasm
4.Regurgitation
5.Heartburn is reported in approximately 20% of patients
6.Symptoms are intermittent and vary from day to day. They usually last for minutes to hours
7.As noted, patients with nutcracker esophagus usually present with chest pain. Only 10% have dysphagia
1.The pathophysiology of DES has not been determined
2.It has been posited that there is increased release of or sensitivity to acetylcholine
3.Body mass index and total cholesterol may be predictive; BMI and glucose may be predictive of LES function
4.DES occurs when there is alteration of the sequential propagation of contractions
1.The classic esophagram is a “cork screw” or “rosary bead” esophagus
1.Scleroderma involves the esophagus in greater than 75% of patients in both progressive systemic sclerosis or the CREST syndrome
1.Symptoms usually reflect the severity of acid reflux
2.Heartburn, regurgitation and dysphagia
3.The severity of esophageal involvement does not correlate with involvement of other organs and may be the presenting complaint
1.Erosive esophagitis is seen in approximately 60% of patients
2.The incidence of Barrett esophagus and adenocarcinoma of the esophagus are increased
3.Esophageal dysmotility develops when the smooth muscle of the distal 2/3 of the esophagus is replaced by fibrosis and scar tissue. Motility is preserved in the striated muscle of the proximal esophagus
1.Esophageal manometry which demonstrates loss of peristalsis in the distal 2/3 of the esophagus
2.Endoscopy in patients with symptoms suggestive of erosive esophagitis
1.Spinal cord injury
2.Anorexia nervosa
3.After several endoscopic sclerotherapy procedures (for esophageal varices)
4.Diabetes mellitus
5.Alcoholism
6.Presbyesophagus
1.Acute gastritis is caused by entities that induce inflammatory changes in the gastric mucosa
2.In general, acute gastritis is divided into erosive and non-erosive forms
3.There is poor correlation between microscopic inflammation and abdominal pain, nausea and vomiting
4.The estimated prevalence is 6.3 patients per 100,000 of the population
1.The pain is often described as gnawing or burning and is located in the epigastrium
2.The pain may be exacerbated or relieved by eating
3.Associated symptoms include: nausea, vomiting, belching and bloating
4.Rarely acute abdominal pain is a presenting symptom which occurs with gangrene of the stomach (usually accompanied by vomiting of purulent gastric contents), fever, chills and hiccups
5.The physical examination is usually normal other than mild epigastric tenderness
1.Acute gastritis is caused by specific drugs, alcohol, bacterial, viral and fungal infection
2.Less frequent causes are shock, radiation therapy, allergy and food poisoning, bile, ischemia and direct trauma
3.Histologic examination of a biopsy establishes the diagnosis in the majority of patients
1.CBC (to assess for anemia from gastric bleeding)
2.Liver and kidney function tests
3.Pregnancy test
4.Stool evaluation for blood
1.The radiologic signs are usually constant regardless of the etiology and include:
a.Thick gastric folds
b.Inflammatory nodules
c.Erosions
d.Coarse area gastrica
2.Endoscopy
3.H. pylori evaluation that is classified as nonendoscopy or endoscopy based
1.Helicobacter pylori is ubiquitous and infects approximately 50% of the world’s population
2.Caucasian patients comprise approximately 29% of cases and Hispanic patients 60%. There is no sex predilection but females have a higher incidence of reinfection
3.Infection may be acquired at any age but some studies suggest infection occurs in childhood
1.Approximately 30% to 35% of patients are asymptomatic
2.No specific signs and symptoms have been described
3.Symptomatic patients may have:
a.Abdominal pain (similar to that of gastritis)
b.Nausea and vomiting
c.Heartburn
d.Diarrhea
e.Hunger in the morning
f.Halitosis
1.The disorder has been staged to follow:
a.Chronic gastritis
b.Atrophic gastritis
c.Intestinal metaplasia that may evolve into dysplasia
d.Gastric adenocarcinoma
e.Gastric MALTomas:
i.Mucosa-associated lymphatic tissue lymphomas
1.EGD (esophagogastroduodenoscopy) in patients with symptoms of peptic ulcer disease
2.EGD plus biopsy from the gastric antrum
3.Echography plus EGD is mandatory in patients with positive biopsy results for MALTomas
4.H. pylori antigen test
5.Carbon 13 urea breath test
6.H. pylori serology
1.Anatomy of the vagal nerve innervation of the abdominal viscera:
a.The left (anterior) and the right (posterior) branches of the vagus nerve descend along the distal esophagus to enter the lower thoracic cavity. They communicate by means of the esophageal plexus
2.Below the plexus, the trunks separate and the anterior trunk branches to form the hepatic, pyloric, and anterior gastric branches. The posterior trunk branches to form the posterior gastric and the celiac branch
3.The terminal branches of the anterior and posterior nerves innervate the parietal cells in a segmental fashion
4.In the USA the lifetime prevalence is approximately 11% to 14% in men and 8% to 11% in women
5.In patients infected with H. pylori the lifetime prevalence is approximately 20% with about 10% in younger patients
1.Gastric and duodenal ulcers are difficult to differentiate by history
2.Epigastric pain is the most common symptom for both conditions.
3.It is manifest by:
a.Gnawing or burning quality which occurs shortly after meals with gastric ulcer and 2 to 5 hours after eating with duodenal ulcer
b.Food and antacids relieve duodenal ulcer pain, but are usually ineffective for gastric ulcers
c.Duodenal ulcer pain may awaken the patient from sleep
d.50 to 80% of patients with duodenal ulcers experience pain at night while only 30 to 40% of patients with gastric ulcers suffer this symptom
e.Pain often follows an individual pattern for each patient
f.If the pain radiates straight through to the back a posterior penetrating gastric ulcer may be causative due to concomitant pancreatitis
g.Patients with gastric outlet obstruction due to chronic duodenal ulcer often complain of:
i.Fullness and bloating with nausea and vomiting several hours after eating
ii.Less frequent clinical manifestations include:
1.Dyspepsia with belching, bloating, distention after fatty food
2.Heart burn
3.Chest pain
4.Hematemesis or melena
5.Symptoms due to anemia (fatigue and dyspnea)
6.Sudden onset of symptoms is a sign of perforation
7.NSAID-induced gastritis may be asymptomatic particularly in elderly patients
8.The physical examination of uncomplicated peptic ulcer disease:
a.Mild epigastric tenderness
b.Rarely right upper quadrant tenderness which is more suggestive of biliary disease
c.Perforated PUD:
i.The sudden onset of severe, sharp abdominal pain sometimes in the epigastrium:
1.Movement exacerbates the pain
2.Fetal position
3.Generalized tenderness, rebound tenderness, guarding and rigidity
4.Signs of septic shock (tachycardia, hypotension and anuria)
1.Peptic ulcers of the gastric or duodenal mucosa extend through the muscularis mucosa
2.The epithelial cells of the gastric and duodenal mucosa secrete mucus from acetylcholine release or stimulation of the epithelial lining which forms a protective gel layer that is impermeable to acid and pepsin
3.Gastric and duodenal cells secrete bicarbonate that buffers acid near the mucosa. Prostaglandin E is secreted which increases the production of both bicarbonate and mucus
4.Ion pumps located in the basolateral cell membrane of the epithelium are a component of the regulation of intracellular pH by removing excess hydrogen ion
5.If there is injury to the barrier, healthy cells migrate to the area in a process called restitution for repair. Mucosal blood flow is restorative by removing acid that had diffused through the mucosa in the area of injury and increases the concentration of bicarbonate
6.Under physiologic conditions there is a balance between gastric acid secretion and mucosal defense mechanisms that prevents injury and include:
a.Tight intercellular junctions
b.Mucus
c.Mucosal blood flow
d.Cellular restitution
e.Epithelial cell renewal
7.NSAIDs, H. pylori, alcohol, bile salts, acid and pepsin allow the back diffusion of hydrogen ions which causes epithelial cell injury
8.H. pylori, acid and pepsin are a major part of the triad that cause peptic ulcer disease
9.H. pylori is able to colonize the gastric mucosa and causes inflammation of the gastric mucosa. Under these circumstances, high levels of gastrin and pepsinogen and reduced levels of somatostatin occur which exposes the duodenum to high levels of acid
10. H. pylori also causes impaired secretion of duodenal bicarbonate which in combination with increased gastric acid secretion lowers the duodenal pH and promotes ulcer formation
1.CBC, liver, amylase and lipase
2.Tests for H. pylori
a.Biopsy:
i.Urease test
ii.Histopathology
iii.Culture
iv.Rapid urease tests are the endoscopic diagnostic test of choice
1.Radiograph of the chest if perforation is suspected to detect free abdominal air
2.Upper GI endoscopy
1.Gastric cancer is the fourth leading cause of cancer mortality in the world
2.Gastric cancer is the 15th most common cancer in the USA
1.There are no associated symptoms in early disease; almost all signs, symptoms and complications are seen in late disease
2.Rare diffuse abdominal pain or pain from an associated gastric ulcer
3.Indigestion, nausea and vomiting
4.Dysphagia
5.Melena and hematemesis
6.Weight loss
7.Enlarged stomach with succession splash
8.Enlarged lymph nodes; Virchow’s node in the left supraclavicular fossa and Irish node in the anterior axilla
1.The frequency of gastric malignancies:
a.Adenocarcinoma – 90% to 95%
b.Lymphoma – 1-5%
c.Stromal tumors (leiomyoma or leiomyosarcoma) – 2%
d.Carcinoids – 1%
e.Adenocanthoma – 1%
f.Squamous cell carcinoma – 1%
1.CBC, electrolytes, liver function tests
2.Tumor markers:
a.Elevation of CEA is seen in 40-50% of patients
b.Elevation of CA-19-9 occurs in 20% of patients
1.Esophagogastroduodenoscopy (EGD):
a.Evaluates the gastric wall and lymph nodes
b.Double-contrast upper GI series
c.Chest radiography (metastatic involvement)
2.CT or MRI of the chest, abdomen and pelvis to assess spread
3.Endoscopic ultrasonography
a.Assess the depth of penetration of the tumor
1.Abdominal abscess
2.Abdominal angina
3.Abdominal hernia
4.Acute intermittent porphyria:
a.Hereditary coproporphyria
b.Doss porphyria
5.Acute mesenteric ischemia
6.Acute pancreatitis and pyelonephritis
7.Adrenal crisis
8.Appendicitis
9.Aortic dissection
10. Biliary disease
11. Cholecystitis
12. Chronic pelvic pain
13. Colonic obstruction
14. Diverticulitis
15. Lead poisoning
16. Esophageal disease
17. Familial Mediterranean fever
18. Gall bladder disease
19. Intestinal motility disorders
20. Ovarian cysts
21. Portal vein obstruction
1.The usual patterns of liver disease are characterized as:
a.Hepatocellular
b.Cholestatic (obstructive)
c.Mixed
2.Hepatocellular disease as exemplified by viral hepatitis or alcohol inflammation and necrosis are the predominant features
3.Cholestatic diseases (gall stone, malignant obstruction, primary biliary cirrhosis and drug induced liver disease) with inhibition of bile flow dominate the symptomatology
4.In mixed pattern both hepatocellular and cholestatic clinical features are manifest
1.Right upper quadrant discomfort or pain (“liver pain”) is demonstrated in many liver diseases
2.Tenderness over the liver area
3.The pain is due to stretch of Glisson’s capsule that encases the liver and is densely innervated with nociceptive afferent receptors
4.Associated signs and symptoms include:
a.Nausea occurs with severe liver disease and may be induced by food odors or eating fatty food
b.Fatigue is the commonest and most characteristic symptom of liver disease. The fatigue arises after exercise and is rare in the morning. It is variable from hour to hour or day to day
c.Itching is seen with acute liver disease particularly in obstructive jaundice and later with hepatocellular disease
d.Jaundice is the hallmark sign of liver disease and is a marker of its severity. Jaundice is usually detectable at a bilirubin level of 2.5 mg/dl
e.Pain radiation to the right upper quadrant, the back and less frequently, to the right shoulder
1.Dependent on the pathology of the liver disease
2.Pain behavior is mediated by vagal afferents. The hepatic branch innervates the liver in addition to the proximal duodenum, pancreas and pylorus
3.Pain behavior mediated by vagal afferents that are activated by lipopolysaccharides or inflammatory cytokines induces the sickness response that is manifested (experimentally in rodents) by:
a.Immobility
b.Decreased social interaction
c.Decreased food intake
d.Anorexia
e.Fever
f.Increased sleep
g.Activation of the hypothalamic-pituitary-adrenal axis
4.A mechanism for the sickness response is that liposaccharides (a component of bacterial cell walls) activate hepatic macrophages (Kupffer cells), dendritic cells and leukocytes that release IL-Β and TNF-α that in turn activate vagal afferents. The proinflammatory cytokines either activate vagal afferents directly or through glomus cells in the abdominal paraganglia:
a.Pain behavior mediated by the sickness response may be responsible for the extreme fatigue seen in many patients with visceral pain
b.Many of the cellular changes induced by the sickness response are abrogated by vagotomy
c.Vagal abdominal afferents carried by the hepatic branch of the abdominal vagus nerves may serve as an interface between the immune system and the brain as proinflammatory cytokines trigger brain stem and hypothalamic illness responses
1.Serum alanine and aspartate aminotransferases (ALT and AST), alkaline phosphatase, direct and total serum bilirubin, albumin and prothrombin time:
a.The pattern of alterations delineates hepatocellular versus cholestatic disease
b.If the process is acute or chronic
c.The degree of hepatic dysfunction
2.Gamma-glutamyl transpeptidase (GGT) which defines if alkaline phosphatase elevations are due to liver disease
3.Hepatitis serology (type of viral hepatitis)
4.Autoimmune biomarkers (antimitochondrial antibody) for primary biliary cirrhosis, P-ANCA for sclerosing cholangitis, and antinuclear, smooth muscle and liver-kidney antibodies to delineate autoimmune hepatitis
5.Liver biopsy:
a.The gold standard for the evaluation of chronic liver disease
1.Ultrasound and CT:
a.Biliary tract dilatation
b.Obstructive jaundice
c.Fatty liver
2.Magnetic resonance cholangiopancreatography (MRCP)
3.Endoscopic retrograde cholangiopancreatography (ERCP):
a.Imaging of the biliary tree
4.Doppler ultrasound and MRI:
a.Assess hepatic vasculature and hemodynamics
5.CT and MRI:
a.Hepatic masses
6.Ultrasound elastography:
a.Measures hepatic stiffness
b.Monitoring fibrosis
1.Anatomy:
a.Bile formed in hepatic lobules is secreted into canaliculi, ductules and larger bile ducts that accompany lymphatics and branches of the portal vein and hepatic artery in portal tracts between hepatic lobules
b.The interlobular bile ducts form larger septal ducts that in turn join and form the right and left hepatic ducts
c.The common bile duct is formed from the hepatic and cystic duct of the gallbladder which enters the duodenum through the ampulla of Vater
1.Gallstone disease:
a.Gallstones migrate into the cystic duct or common bile duct that causes inflammation or obstruction
b.Biliary colic:
i.Begins suddenly and may persist for 30 minutes to 5 hours to subside gradually or rapidly
ii.It is a steady pain; if it lasts more than 5 hr it may be cholecystitis
iii.Nausea and vomiting are frequent with painful episodes
iv.The pain is a steady ache or fullness in the epigastrium or right upper quadrant of the abdomen
v.Radiation of pain is to the interscapular area (often T6 tip of the scapula), right scapula or shoulder
vi.Associated fever or chills suggests cholecystitis, pancreatitis or cholangitis
vii.Precipitants of biliary colic include:
1.A fatty meal
2.A large meal followed by a period of fasting
3.Biliary colic may be nocturnal
2.Acute cholecystitis:
a.Acute inflammation of the gallbladder walls most often is a consequence of cystic duct obstruction by a stone
b.Clinical manifestation:
i.Often acute cholecystitis is initiated by an attack of biliary colic that progressively increases in intensity
ii.60-70% of patients have had spontaneous similar attacks that resolved
iii.Pain becomes more generalized in the right upper abdomen as the attack evolves. The radiations are to the interscapular area, the right scapula and shoulder
iv.Peritoneal signs that include pain with movement or deep inspiration may be associated
v.Patients are anorectic, suffer nausea and vomiting, and become dehydrated. Jaundice can occur when edematous inflammation involves bile ducts
vi.A low-grade fever is common. It may be accompanied by shaking chills or rigors
vii.There is tenderness in the RUQ of the abdomen. In approximately 25 to 50% of patients, an enlarged tense gallbladder can be palpated
viii.Murphy’s sign can be induced by a cough or deep inspiration (exacerbates the pain and causes an inspiratory arrest)
ix.Localized rebound tenderness in the RUQ, abdominal distention, and hypoactive bowel sounds are present. Generalized peritoneal signs and rigidity are absent unless there is perforation
1.The great majority of patients with acute cholecystitis have an obstruction of the cystic duct by a stone
2.Inflammation of the GB wall is caused by:
a.Increased intraluminal pressure and distention that causes ischemia of the GB mucosa and wall
b.The release of lysolecitin from the action of phospholipase on lecithin
c.Bacterial inflammation that occurs in 50-85% of patients. The most frequent organisms are Escherichia coli, Klebsiella, Streptococcus and rarely Clostridium
3.Complications of cholecystitis:
a.Empyema
b.Gangrene and perforation
c.Fistula and gallstone ileus
d.Bile and porcelain gallbladder
1.Gallbladder ultrasound:
a.Procedure of choice for GB stones
2.Radioisotope scans (HIDA, DIDA):
a.Delineates cystic duct obstruction and evaluates bile ducts
3.Elevated serum bilirubin and or alkaline phosphatase supports the diagnosis of a common duct stone
1.The pancreas secretes 1500-3000 ml of isosmotic alkaline (pH >8.0) fluid per day that contains approximately 20 enzymes and zymogens that effect the major digestive functions of the gastrointestinal tract
2.Gastric acid stimulates pancreatic secretion of water and electrolytes
3.Cholecystokinin release from the duodenum and jejunum is triggered by long chain fatty acids, tryptophan, phenylalanine, valine, methionine and gastric acid
4.CCK induces the secretion of enzymes from the pancreas
5.The vagus nerve significantly regulates pancreatic secretion via secretin and CCK that depend on permissive function of a vagal afferent and efferent pathway. This is particularly important for the enzyme secretin
6.Water and bicarbonate pancreatic secretions are primarily regulated by secretin and CCK
7.Vagal stimulation affects the release of vasoactive intestinal peptide (VIP) a secretin agonist
8.Bile salts stimulate pancreatic secretion which integrates the physiology of the biliary tract, pancreas and small intestine
9.Pancreatic exocrine secretion is also regulated by inhibitory neuropeptides
10. Bicarbonate is the most important ion in pancreatic secretions as it helps to neutralize gastric acid and provides the necessary pH for the activity of pancreatic enzymes
1.Abdominal pain is the major symptom of acute pancreatitis:
a.The pain may vary from mild discomfort to overwhelming distress
b.The quality of the pain is steady and boring
c.It is located in the epigastrium and periumbilical area and most often radiates straight through to the back. Less frequently it radiates to the chest, flank and lower abdomen
d.Pain may be more intense in recumbency and patients’ position of comfort is sitting with the trunk flexed and the knees flexed
e.Associated signs and symptoms include:
i.Nausea and vomiting
ii.Abdominal distention that is due to ileus and chemical peritonitis
f.Physical examination demonstrates:
i.An anxious patient in severe distress
ii.Low grade fever
iii.Tachycardia and hypotension
iv.Shock in some patients
v.Abdominal tenderness and rigidity are present to a variable degree but are much less prominent than the severe pain
vi.Bowel sounds are decreased or absent
vii.Rarely an enlarged pancreas or one with a pseudocyst may be palpated in the upper abdomen
viii.In severe necrotizing pancreatitis:
1.Cullen’s sign may be seen as a faint blue discoloration surrounding the umbilicus due to hemoperitoneum
2.Turner’s sign from blood breakdown products (blue-red, purple or green-brown) may be visible in the flank
ix.Systemic effects of pancreatitis occur in all systems
x.Erythematous skin nodules occur in 10-20% of patients
1.Shock may occur from:
a.Hypovolemia due to exudation of blood and plasma proteins into the retroperitoneal space
b.Increased formation and release of kinen peptides that cause increased vascular permeability
c.Systemic effects of proteolytic and lipolytic enzymes
d.Common causes of pancreatitis:
i.Gallstones
ii.Alcohol
iii.Hypertriglyceridemia
iv.Blunt abdominal trauma
v.Abdominal and nonabdominal procedures
vi.Endoscopic retrograde cholangiopancreatography (ERCP) particularly after biliary manometry
vii.Drugs
viii.Sphincter of Oddi dysfunction
e.Less frequent causes of pancreatitis:
i.Vascular causes and vasculitis:
1.Hypoperfusion after cardiac arrest
ii.Connective tissue diseases
iii.Thrombotic thrombocytopenic purpura
iv.Cancer of the pancreas
v.Periampullary diverticulum
vi.Pancreas divisum
vii.Cystic fibrosis
viii.Renal failure
1.Increased serum amylase; values of three times normal are very supportive of acute pancreatitis if salivary gland disease and gut perforation or infarction are ruled out
2.There is no correlation between the severity of pancreatitis and the degree of serum amylase elevation. Pancreatic isoamylase and lipase levels may remain elevated for 7 to 14 days while total serum amylase may return to normal after 2 to 3 days even in the face of active clinical disease
3.Leukocytosis of 15,000 to 20,000 /μL and hemoconcentration may be seen
4.Hyperglycemia due to decreased insulin release, increased glucagon release and excessive secretion of glucocorticoids and catecholamines
5.Hypocalcemia occurs in approximately 25% of patients that is not completely understood
6.Hyper bilirubinemia is demonstrated in approximately 10% of patients which returns to normal in 4 to 7 days
7.Serum alkaline phosphatase and AST are transiently elevated
8.Approximately 25% of patients are hypoxemic
9.The EKG may demonstrate ST segment and T wave abnormalities that simulate myocardial ischemia
10. CT evaluation delineates the severity of the disorder
11. Ultrasonography evaluates the gallbladder
1.Intestinal vascular insufficiency is divided into:
a.Arteriosclerotic mesenteric ischemia
b.Non-occlusive mesenteric ischemia
c.Mesenteric venous thrombosis
2.Acute ischemia is more common than chronic ischemia
3.The blood supply to the intestines includes:
a.Celiac artery
b.The superior and inferior mesenteric arteries and branches of the internal iliac artery
4.There is extensive collateralization between the major mesenteric trunks and branches of the mesenteric arcade
5.Collateral vessels within the small bowel anastomose with vessels in the duodenum and the pancreas
6.Collateral vessels of the colon anastomose with vessels at the splenic plexus and descending / sigmoid colon which are known as Griffiths point and Sudeck’s point respectively which are the most common locations for colonic ischemia
7.This splanchnic circulation may receive up to 30% of the cardiac output
8.Occlusive ischemia occurs if there is blockage of blood flow in a major artery that supplies the intestine
9.Chronic ischemia requires the progressive occlusion of at least two major vessels that supply the intestine (chronic intestinal angina)
10. Non-occlusive ischemia is caused by vasospasm from severe dehydration or shock
1.The usual cause is an arterial embolus in a patient with atrial fibrillation, valvular heart disease, recent myocardial infarction or vascular catheterization
2.The pain is acute in onset (if embolic), is severe and nonremitting
3.The location is abdominal
4.A striking feature is the severity of the pain which is out of proportion to the physical examination
5.Associated signs and symptoms include:
a.Nausea and vomiting
b.Transient diarrhea
c.Bloody stools
6.There may be early abdominal distention and hypoactive bowel sounds
7.As the process evolves there are signs of peritonitis and vascular collapse
1.The embolus is usually found just proximal to the origin of the middle colic artery. The proximal jejunum may be spared while the remainder of the small bowel to the transverse colon is ischemic
1.CBC, serum chemistries, coagulation profile, arterial blood gas, amylase, lactic acid and cardiac enzymes
2.EKG
1.Abdominal radiographs
2.CT of the abdomen
3.Mesenteric angiography
4.Mesenteric duplex scan:
a.High peak velocity of flow in the superior mesenteric artery is correlated with approximately 80% prediction of mesenteric ischemia
b.Laparotomy is the “gold standard” for diagnosis
1.Usually encountered in circumstances of severe physiologic stress that is seen with severe dehydration or shock
1.Generalized abdominal pain
2.Anorexia
3.Bloody stools
4.Abdominal distention
5.A subgroup of patients present with obtundation
1.Early intestinal ischemia is often associated with sequestration of fluid within the bowel wall that causes loss of interstitial volume
2.Decreased mucosal integrity is associated with nonocclusive mesenteric ischemia and less frequently with loss of inferior mesenteric arterial blood flow
1.CBC, electrolytes, amylase, CPK, lactic acid
2.D-dimer, glutathione S-transferase, platelet activating factor (PAF) and mucosal pH monitoring
1.Mesenteric ultrasound
2.Abdominal radiograph
3.CT of the abdomen
4.Mesenteric angiography
1.Ischemic colitis is the most common form of acute ischemia of the intestine other than strangulated small bowel obstruction
2.It is the most frequent form of acute intestinal ischemia that complicates cardiovascular surgery
3.Vascular anatomic features:
a.An index arterial lesion is unusual although many elderly patients have risk factors for atherosclerosis
b.There may be narrowing of small vessels and increased tortuosity of long colic arteries
c.In a great number of instances there is an acute self-limited decrease in intestinal blood flow from diffuse disease of small segmental vessels
d.The colon has a relatively low blood flow that may predispose it to ischemic compromise
e.The blood flow may also be affected by motor activity particularly during straining at stool. Experimental distension of the colon increases intraluminal pressure, reduces total blood flow and decreases the arteriovenous oxygen gradient in the colonic wall
f.The microvasculature plexus is less well developed and is embedded in a thicker wall as compared to the small intestine
g.The end-vessels to the bowel wall (vasa recta) are less developed in the right than left colon
h.The vasa recta are sensitive to vasospasm and there is poor collateral circulation at this level
i.As noted above, the watershed areas of the colon are at the splenic flexure and the rectosigmoid junction
1.Acute onset of a mild crampy abdominal pain with tenderness over the affected bowel segment
2.After 24 hours, there is usually bright red or maroon blood in the stool. There is minimal blood loss that is usually mixed with the stool. There is no hemodynamic compromise (may be seen with diverticulitis or vascular malformation)
3.Anorexia, nausea and vomiting may occur due to ileus
4.Approximately 15% of patients have rebound tenderness, rigidity and absent bowel sounds due to transmural infarction and necrosis
1.Colonoscopy demonstrates:
a.Mild ischemic change of the mucosa that causes minimal erythema
b.Moderate ischemic change with pale mucosal ulcerations and extension to the muscular layer of the bowel wall
c.Severe ischemic colitis presents with ulcerations that are deep and have a black or green discoloration of the mucosa. They represent full thickness bowel wall necrotic lesions
2.Local hypoperfusion and reperfusion injury are thought to be important mechanisms
3.The spectrum of injury includes:
a.Reversible colopathy with subpial hemorrhage and edema
b.Transient colitis
c.Chronic colitis
d.Stricture
e.Gangrene
f.Fulminant universal colitis
4.The distribution of the lesions are segmental:
a.Left sided disease, the inferior mesenteric artery distribution, is more frequently involved than right-sided involvement (superior mesenteric artery)
5.Patients with isolated right colon ischemia have a worse prognosis
1.Colonoscopy (the most accurate study)
2.Serum lactate, lactate dehydrogenase, alkaline phosphatase and systemic acidosis are markers of ischemia
1.CT:
a.The most common finding is segmental circumferential wall thickening
b.May be positive in 89% of patients
1.In left-side colon ischemia, which is the more common type, “watershed” areas are usually the ischemic segments. The limited collateral flow is between the superior mesenteric (SMA) and inferior mesenteric (IMA) artery circulations. Less frequently there is ischemia between the territories of the superior hemorrhoidal artery and the IMA circulations that affect the descending and sigmoid colon
2.Rates of surgery and mortality are greater with right-side colon ischemia that suggest a different pathophysiology in a subset of patients which has been posited to occur from large artery disease
1.Series of 49 patients:
a.Presenting clinical features start less than 10 days prior to diagnosis
b.The median age of patients was approximately 70 (with an 11 year range)
c.Abdominal pain occurs in over 90% of patients:
i.Abdominal angina occurs in a subset of patients
ii.There is a predominance of pain over bleeding with RSCI (right sided colon ischemia)
iii.Higher rates of comorbidities which include:
1.Coronary artery disease
2.Atrial fibrillation
3.Dialysis dependency
iv.Female predominance
1.Role of large artery ischemia is posited to be significant with RSCI
2.The SMA supplies a great proportion of the small bowel and the proximal and transverse colon such that SMA hypoperfusion can cause extensive acute mesenteric ischemia
3.There are often poor collateral vessels in the mesentery of the right colon compared to the left. This is important when the IMA is sacrificed during abdominal aortic aneurysm repair or colon resection
1.Colonoscopy
2.Mesenteric (SMA and IMA) angiography to R/O large vessel disease
1.CT with contrast
1.As noted earlier, the blood flow to the intestines arises from the superior mesenteric artery whose origin is the abdominal aorta. It has branches to the pancreas and duodenum, supplies three branches to the proximal colon, and terminates in the arteriae rectae that supply the jejunum and ileum.
2.Venous blood drains into the venae rectae that then flow into the ileocolic, middle colic and right colic veins. These veins coalesce to form the superior mesenteric vein that merges with the splenic vein which forms the portal vein:
a.The left gastric vein drains the lower part of the esophagus and upper part of the lesser curvature of the stomach via the left gastric vein that enters the portal vein at its origin.
b.The fundus of the stomach is drained by short veins that drain into the splenic vein
c.The left colon venous drainage is through the inferior mesenteric vein to the splenic vein
3.The incidence of MVT is 2.7 patients per 100,000 of the population. It is responsible for 6% to 9% of acute mesenteric ischemia
4.Risk factors for MVT include:
a.Prothrombotic states
b.Inflammatory bowel disease
c.Malignancy
5.Thrombosis of the larger distal portion of the mesenteric veins is usually secondary to malignancy, pancreatitis and infection and is often associated with portal vein thrombosis
6.Thrombosis that originates from the venae rectae that causes MVT is most often related to a prothrombotic state and causes isolated MVT
7.Primary MVT accounts for approximately 21-49% of patients with MVT
8.JAK V16 mutation that maps to chromosome 9p24.1 is found in a subset of patients with MVT often associated with myeloproliferative disorders
1.Patients most commonly present with acute abdominal pain in 90 to 100% of cases
2.Common associations are nausea, vomiting and melena
3.The duration of symptoms before diagnosis is usually 1.5 to 2 days
4.The abdominal pain is out of proportion to the physical findings
5.Approximately 15% of patients have melena, hematemesis, or hematochezia (the passage of fresh blood through the anus). Occult blood is demonstrated in approximately 50% of patients
6.Fever and peritoneal signs signal progression of the infarction and may evolve to a blood pressure of less than 90 mmHg systolic with ascites
7.The physical examination often demonstrates abdominal distention with mild to moderate tenderness
1.Malignancies particularly myeloproliferative disorders are the most common prothrombotic disorder with MVT
2.Oral contraceptive use is causative in 9 to 18% of young women
3.Antithrombin III, protein S, and protein C deficits have been associated with MVT
4.Intra-abdominal surgery, particularly splenectomy, are risk factors for MVT
5.MVT and portal vein thrombosis are more common in patients with advanced cirrhosis
6.Patients awaiting liver transplantation may have portal vein thrombosis (15% of patients)
7.Possible association of MVT has been posited for hyperhomocysteinemia and methylene tetrahydrofolate reductase C677T mutations
1.Serum lactate level does not initially correlate with intestinal infarction. At the time the process demonstrates lactic acidosis it is late in the course of the illness and mortality is 75%
2.Leukocytosis and hemoconcentration are commonly seen
1.Plain abdominal radiographs are usually negative but are helpful in eliminating other disorders
2.Radiographic signs of bowel ischemia include:
a.Thumb printing (semi-opaque indentations in the bowel lumen from edema)
b.Air in the intestines
c.Air in the portal vein and free peritoneal air
3.Contrast enhanced CT:
a.A filling defect in the mesenteric vein is a diagnostic finding
b.Bowel wall ischemia is suggested by:
i.Bowel wall thickening
ii.Portal vein air
iii.Persistent enhancement of the bowel wall
c.The above findings are specific but have low sensitivity
4.Doppler ultrasound
1.Definition: Abdominal angina is postprandial pain that occurs in patients with mesenteric vascular occlusive disease in which blood flow cannot increase enough to meet visceral demands
2.The mean age of patients is slightly older than 60
3.Median arcuate ligament syndrome may be causative in young patients. It has a 3:1 female predominance
1.Abdominal pain that occurs a few minutes after eating and subsides within a few hours
2.Patients report cramping as the quality of the pain
3.Diarrhea may be associated
4.There may be a fear of eating with consequent weight loss
5.An abdominal bruit is occasionally auscultated although in one series it was reported in 60 to 90% of patients
6.The abdomen is scaphoid and soft even during episodes of pain
7.Peripheral vascular disease and smoking are co-morbidities
1.The most common cause is atherosclerosis which most often involves the ostia of mesenteric vessels
2.There are collateral vessels between the celiac artery and the SMA (the pancreaticoduodenal arcades). Fewer collaterals are present between the IMA and SMA
3.Collateral circulation to the hind gut and midgut is also provided by the iliac arteries
4.There is significant increase of blood flow to the celiac and mesenteric arteries after eating under normal circumstances
5.Usually there is severe stenosis of at least two mesenteric vessels
6.Rarely the median arcuate ligament can compress both the celiac axis and the mesenteric artery (the median arcuate ligament syndrome MALS).
7.Rare causes of intestinal angina include:
a.Cardiomyopathy
b.Acute acalculous cholecystitis
1.An evaluation for malignancy and gastric ulcer should be undertaken if the history and examination are suggestive
2.A guiac positive stool occurs in 10% of patients
1.Duplex ultrasound of the mesenteric vessels
2.Mesenteric angiography
1.In approximately 75% of patients, acute intestinal obstruction occurs from previous abdominal surgery that has produced adhesive bands. Internal and external hernias are also major etiologies
2.The incidence of acute intestinal obstruction within the post-operative period is falling; but has been reported as 5 to 25%. The incidence of obstruction is lower with laparoscopic surgery
3.Adynamic ileus and primary intestinal pseudo obstruction may simulate obstruction
4.After postsurgical and internal hernias, disorders of the intestinal wall cause approximately 25% of intestinal obstructions
1.Mechanical intestinal obstruction:
a.The pain is cramping and located in the midabdomen
b.It is worse the higher the obstruction
c.The pain is paroxysmal and the patient is relatively comfortable between paroxysms
d.Patients often note borborygmi concomitantly with episodes of pain
e.The pain may decrease as distention increases (possibly due to decreased intestinal mobility from edema of the intestinal wall)
f.If strangulation occurs, the pain is often more localized, may be steady and may lose its colicky paroxysmal character
g.Vomiting is associated and is earlier and more severe the higher the level of the obstruction. The vomitus is composed of bile and mucus with high obstructions. In ileal obstruction, the vomitus may become feculent and malodorous due to bacterial overgrowth proximal to the obstruction. Singultus is common.
h.If the obstruction is complete, there is obstipation and failure to pass gas by rectum. Diarrhea may occur with partial obstruction
i.Blood in the stool is rare, but may occur with intussuception
j.In both adynamic ileus and pseudo-obstruction (colonic), there is absence of paroxysmal colicky pain but pain does occur from distention. Vomiting does occur but is not as severe as with mechanical obstruction. Singultus commonly occurs.
k.The physical examination demonstrates:
i.Abdominal distention (the predominant sign in all forms of obstruction)
ii.Distention is least with high obstructions in the small intestine and most severe with colonic obstruction
iii.Early obstruction of both the large and small intestine produce minimal tenderness and rigidity and the temperature is rarely > 100oF
iv.Perforation causes shock, tenderness, rigidity and fever from bacterial contamination of the peritoneum
v.Hernial orifices (if they are the cause of the obstruction) will demonstrate a mass
vi.Loud, high-pitched borborygmi are concomitant with paroxysms of pain. This finding may be absent in patients who have strangulation or nonstrangulating lesions
vii.A quiet abdomen may occur with obstruction and does not make the diagnosis of adynamic ileus
viii.A palpable abdominal mass is often a closed-loop strangulating small-bowel obstruction. The palpable lesion is the tense fluid-filled loop
1.The accumulation of gas and fluid proximal to the obstruction causes the abdominal distension. Approximately 70 to 80% of the intestinal gas is from swallowed air which is primarily nitrogen and is not absorbed well from the intestinal lumen
2.The accumulated proximal fluid is a combination of ingested food, saliva, gastric juice, biliary and pancreatic secretions and the failure of physiologic water and ion transport
3.There is an increase of intraluminal pressure from 2-4 cm H20 to 8-10 cm H20
4.Fluid shifts into the intestinal lumen and may be massive which causes systemic hypovolemia, renal failure and shock
5.There is edema of the intestinal wall and peritoneum from blockage of venous return from the intestine which also contributes to fluid and electrolyte loss
6.A closed loop obstruction occurs when the small intestine is occluded at two points by a fascial hernia or adhesive band, which may also compromise the segmental blood supply. If a closed loop is present, peristalsis may increase intraluminal pressure to 30-60 cm H20
7.Decreased blood supply to the gastrointestinal tract is associated with bacterial invasion and peritonitis
1.Leukocytosis with shift to the left is common with strangulation
2.Elevated amylase is seen in all forms of obstruction
1.Roentgenographic studies demonstrate:
a.Distention of fluid- and gas-filled loops of small intestines arranged in a “stepladder” pattern
2.Air-fluid levels are noted in the absence of colonic gas
3.A “ coffee bean” mass may be demonstrated with a strangulating closed loop obstruction
a.CT:
i.The modality of choice to evaluate post-operative patients as it can delineate a dynamic ileus, partial obstruction and complete obstruction
b.Abdominal roentgenograms:
i.May have characteristic features of colonic obstruction:
1.“Birds beak” sign with sigmoid volvulus
2.An enlarged cecum with cecal torsion or bascule (folding of the cecum anteriorly over the ascending colon)
3.Colonic obstruction with a competent ileocecal valve demonstrates gas distention which is confined to the colon
1.Definition:
a.A clinical syndrome characterized by clinical signs supportive of mechanical obstruction without obstruction in the intestine
b.Pseudo-obstruction is divided into acute and chronic forms:
i.Acute colonic pseudo-obstruction (ACPO):
1.The colon is dramatically dilated which may lead to perforation and peritonitis
2.ACPO can recur
ii.Chronic pseudo-obstruction (CCPO):
1.Symptoms of recurrent bowel obstruction within 6 months
2.Abdominal bloating and / or pain within the previous 3 months
c.Suggestive radiographs of bowel obstruction
d.No evidence of anatomic structural lesions
e.In burn and orthopedic patients the prevalence rate of acute colonic pseudo-obstruction (ACPO) is 0.29%
f.ACPO is most often encountered in hospitalized patients:
i.The usual underlying disorders are:
1.Trauma
2.Pregnancy
3.Caesarean delivery
4.Sepsis
5.Cardiothoracic, pelvic and orthopedic surgery
g.This is generally a disease of elderly patients. The mean age is often reported to be in the sixth decade. It is very common in younger patients with spinal cord injury. More recent studies support the mean age to occur in the seventh and eighth decade
1.The usual setting is an elderly debilitated hospitalized patient with multiple medical issues who have undergone medical or surgical procedures
2.Presenting symptoms include:
a.Abdominal pain (80%)
b.Nausea and vomiting (80%)
c.Obstipation (40%)
d.Fever (37%)
3.Physical examination:
a.Abdominal distention (90 to 100%)
b.Abdominal tenderness (66%)
c.Hypoactive high pitched or absent bowel sounds (60%); bowel sounds may be normal or hyperactive in 40% of patients
d.Empty rectum on digital examination
e.Abnormal tenderness in the abdomen occurs with perforation or ischemia but may also occur in patients with viable bowel
1.The three most common causes of acute colonic pseudo-obstruction are:
a.Trauma (particularly retroperitoneal)
b.Systemic serious infections
c.Myocardial infarction and congestive heart failure
d.Commonly associated disorders include:
i.Old age (particularly hospitalized debilitated patients with multiple medical problems)
ii.Hypothyroidism
iii.Hyponatremia, hypokalemia, hypocalcemia, hypercalcemia and hypomagnesemia
iv.Renal failure
v.Medications (narcotics, tricyclic antidepressants, phenothiazines and anesthetics)
vi.Less frequent causes:
1.Megacystis megacolon
2.Amyloidosis
3.GI carcinoma
4.Guillain-Barre syndrome
5.Multiple myeloma
6.Alcohol abuse
7.SLE
8.Systemic sclerosis
2.Mechanisms posited for pseudo-obstruction include:
a.Increased sympathetic tone concomitantly with decreased parasympathetic tone
b.Disruption of sacral parasympathetic innervation to the distal colon
c.Involvement of the transition zone (Tz) between the dilated and nondilated bowel loops that causes proximal distention and smooth muscle hypertrophy. This may be caused by deficiency of the myenteric plexus and ganglia neurons, particularly nitric oxide synthase positive neurons that affect the transitional zone
1.Appendicitis is the most common abdominal surgical emergency in the USA
2.The peak incidence is between the second and third decade of life
3.Perforation is more common in infancy and in the elderly
4.Males and females are affected equally with the exception of the period between puberty and age 25
1.Pain is located periumbilically initially and then localizes to the right lower quadrant. This sequence occurs in approximately 2/3 of patients
2.The pain (visceral in quality) is usually mild, cramping and lasts 4-6 hours
3.When inflammation spreads to involve the parietal peritoneum (primarily somatic) it becomes steady, more severe and is aggravated by movement and cough
4.Anorexia is common; a hungry patient does not have appendicitis
5.Nausea and vomiting occur in 50 to 60% of patients and is usually self-limited
6.Urinary frequency and dysuria may occur if the inflamed appendix is adjacent to the bladder. Similarly, juxtaposition near the sigmoid colon may cause diarrhea. These symptoms may also occur from complex interactions of C-fiber and A-delta fiber interactions, at the level of the dorsal root ganglia or superficial dorsal horn of the spinal cord
7.Recurrent acute appendicitis may rarely recur with complete resolution of signs and symptoms between attacks; it may also recur if a long appendiceal stump is left from the initial operation
8.Physical examination:
a.The signs vary with the time of the examination in relation to the onset of the inflammation and with the anatomical location of the appendix
b.Anatomical locations include:
i.Deep in the pelvic cul-de-sac
ii.In the right lower quadrant adjacent to the peritoneum, cecum and small intestine
iii.In the right upper quadrant particularly during pregnancy
iv.Rarely in the left lower quadrant
c.Tenderness may be absent in the initial stage of the disorder but evolves and is localized to the anatomical position of the appendix
d.The typical location for tenderness is McBurney’s point (located in a line one-third of the way between the anterior iliac crest and the umbilicus).
e.Abdominal tenderness is often absent if the position of the appendix is retrocecal or deep in the pelvis. In these instances, tenderness may be in the flank, or demonstrated on rectal or pelvic examination. Referred rebound tenderness may be elicited
f.Flexion of the right hip and decreased right lower extremity movement are due to parietal peritoneal inflammation
g.Mechanical hyperesthesia of the skin over the right lower quadrant as well as positive psoas and obturator signs occur late in the course
h.The temperature is normal or slightly elevated; perforation is suggested if it is >38.3C (101oF). Tachycardia is concomitant with the elevated temperature
i.Abdominal rigidity and tenderness increase with evolution of the process to perforation with localized or diffuse peritonitis
j.Distension is rare unless there is diffuse peritonitis
k.An abdominal mass may be palpated if there is perforation but usually occurs three days after the event. Earlier detection of a mass is seen with Crohn’s disease or carcinoma of the cecum
l.Perforation rarely occurs prior to 24 hr after onset but occurs commonly after 48 hours
1.Appendicitis most often occurs from luminal obstruction due to a fecalith. This forms from accumulation and inspissation of fecal matter around vegetable fibers
2.Rarer causes of luminal obstruction of the appendix are:
a.Enlarged lymphoid follicles associated with viral illness
b.Pinworms, Ascaris and Taenia
c.Tumors (carcinoid and adenocarcinoma)
d.Barium contrast
3.If there is appendiceal ulceration. Luminal bacteria invade the appendiceal wall; there is an increase of luminal pressure with consequent decrease of arterial blood flow. If the process evolves slowly it may be walled off by the terminal ileum, cecum, and omentum. If this occurs, there is a local abscess. Rupture of appendiceal abscesses may cause fistulas from the appendix to other abdominal organs
1.Moderate leukocytosis (10,000-18,000 cells/μL) with a shift to the left is classic. Leukocytosis > 20,000 μ/L is evidence of perforation
2.Anemia with guiac positive stool suggests carcinoma of the cecum particularly in elderly patients
3.Urinalysis may reveal a few white or red cells without bacteria if the appendix is adjacent to the right ureter or bladder
1.A fecalith in the right lower quadrant is seen in 5% of patients
2.CT demonstrates a thickened appendix, periappendicular stranding, and a fecalith. The sensitivity and specificity are over 90%
1.Appendicitis
2.Small bowel obstruction
3.Gastroenteritis
4.Mesenteric ischemia
1.Gastrointestinal etiologies:
a.Appendicitis
b.Inflammatory bowel disease
c.Diverticulitis (unusual on the right side)
d.Gastroenteritis
e.Inguinal hernia
2.Gynecologic etiologies:
a.Ovarian tumor or torsion
b.Pelvic inflammatory disease
3.Renal etiologies:
a.Pyelonephritis
b.Perinephric abscess
c.Nephrolithiasis
1.Definition:
a.Peritonitis is an inflammation of the peritoneum that may be localized or diffuse, acute or chronic and is due to infection or nonseptic causes
b.Acute peritonitis is most often infectious and is related to a perforated viscus which is defined as secondary peritonitis
c.If no intra-abdominal source is identified, infectious peritonitis is defined as primary or spontaneous
1.The cardinal sign and symptom of peritonitis is acute abdominal pain and tenderness most often associated with fever
2.The location of the pain is dependent on the underlying cause and whether the inflammation is localized or generalized
3.In localized peritonitis from appendicitis or diverticulitis both the pain and physical findings are restricted to the site of inflammation
4.Bowel sounds are most often absent
5.Tachycardia, hypotension and dehydration are common
6.In peritoneal dialysis patients in whom peritonitis is not infrequent approximately, 20% of patients do not have pain or fever. These patients are older than other patients undergoing dialysis
1.A perforated viscus, penetrating wound of the abdominal wall or a foreign object in the abdomen are the main causes of infective peritonitis
2.The most common causes of peritonitis are:
a.Ruptured appendix
b.Ruptured diverticulum
c.Perforated peptic ulcer
d.Incarcerated hernia
e.Gangrenous gall bladder
f.Volvulus
g.Bowel infarction
h.Cancer
i.Inflammatory bowel disease
j.Intestinal obstruction
3.Bacterial peritonitis:
a.May occur in the absence of an intraperitoneal source of bacteria. In 90% of instances, it occurs in a setting of ascites and liver cirrhosis in patients who have a protein concentration of < 1g/L
4.Aseptic peritonitis may be caused by peritoneal irritation from gastric juice, bile, pancreatic enzymes, blood, urine and sterile foreign bodies
5.Medical causes of aseptic peritonitis are SLE, familial Mediterranean fever and porphyria
1.Leukocytosis and marked acidosis are expected
2.Paracentesis with a cell count of >250 neutrophils is usual in the peritoneal fluid. Protein and lactate dehydrogenase may be elevated
1.Plain abdominal radiographs:
a.Dilation of both the small and large intestines with edema of the bowel wall
b.Free air under the diaphragm is almost always diagnostic of a perforated viscus
2.CT:
a.May demonstrate free fluid or an abscess
1.The incidence of CD is 3.1 to 14.6 patients per 100,000 person years
2.Prevalence of CD is 37-246 patients per 100,000 person years
3.The peak age of onset is between 15 and 30 years. A second peak is seen between 60-80 years of age
4.The male to female ratio for CD is 1.1 to 1.8:1
1.Most often presents as acute or chronic bowel inflammation; it evolves toward:
a.A fibrostenotic-obstructive pattern or a penetrating fistulous pattern. The site of the disease determines clinical features
1.The commonest site of inflammation is the terminal ileum
2.Patients complain of right lower quadrant pain and diarrhea
3.The presentation may mimic that of acute appendicitis with severe right lower quadrant pain, a palpable mass with fever and leukocytosis
4.Pain is colicky and is relieved by defecation and is associated with a low grade fever
5.A high-spiking fever is more consistent with an abscess
6.Weight loss occurs from diarrhea, anorexia and fear of eating and may reach 10 to 20% of normal body weight
7.An enlarged mass may be palpated in the right lower quadrant that consists of inflamed bowel, adherent and indurated mesentery and enlarged lymph nodes
8.The mass may extend to obstruct the right ureter or irritate the bladder which is associated with dysuria and fever
9.Early in the disease course, there may be intermittent obstruction from bowel wall edema and spasm that cause postprandial pain. If the process continues over years it evolves into fibrostenotic narrowing and stricture
10. If there is a stricture, diarrhea decreases and is replaced by chronic bowel obstruction. Acute episodes of obstruction are precipitated by bowel inflammation, spasm or impaction of undigested food
11. Severe inflammation of the ileocecal area may lead to fistula with the adjacent bowel, skin, urinary bladder, or abscess within the mesentery. Enterovesicle fistulas are associated with:
a.Dysuria
b.Recurrent bladder infections
c.Pneumaturia (rare)
d.Fecaluria (rare)
12. Enterocutaneous fistulas often drain through abdominal surgical scars
13. Enterovaginal fistulas present as dyspareunia or feculent foul-smelling painful discharge
1.Associated with a loss of digestive and absorptive surface that may manifest as malabsorption or steatorrhea
2.The intestinal malabsorption may be associated with hypoalbuminemia, hypocalcemia, hypomagnesemia, coagulopathy, hyperoxalurea
3.Secondary disorders from the malabsorption are B12 deficiency, vertebral fractures (vitamin D deficiency), hypocalcemia, and steroid use. Pellagra from niacin deficiency has been reported
1.The clinical manifestations of colitis are:
a.Crampy abdominal pain
b.Low grade fever
c.Hematochezia
2.Gross bleeding is not as common as that which occurs with ulcerative colitis and occurs in approximately 50% of patients with exclusive colitis
3.It is posited that pain occurs from irritation by fecal material in inflamed segments of the large bowel
4.Decreased rectal compliance may be another cause of diarrhea
5.Massive gross bleeding and toxic megacolon are rare occurrences
6.Bowel obstruction from strictures occurs in 4 to 16% of patients
7.Fistulas occur into the stomach and duodenum that cause vomiting, and in the proximal and midbowel that are associated with malabsorption
8.Approximately 10% of women develop a rectovaginal fistula
9.Perianal disease is seen in approximately 1/3 of patients and is characterized by:
a.Incontinence
b.Hemorrhoidal tags
c.Strictures
d.Anorectal fistulae
e.Perirectal abscesses
1.The symptom and signs of patients that develop upper gastrointestinal tract disease are:
a.Epigastric pain
b.Nausea
c.Vomiting
2.These patients often have H. pylori–negative gastritis.
3.Disease is usually more severe in the second portion of the duodenum rather than the bulb.
4.Patients may develop fistulae from the large and small bowel and may develop gastric outlet obstruction.
1.Early lesions are aphthoid ulcerations and focal crypt abscesses associated with aggregations of macrophages. These form non caseating granulomas in all layers of the bowel wall as well as in lymph nodes, mesentery, peritoneum, liver and pancreas
2.Granulomas are seen in approximately 50% of patients but are rarely demonstrated by mucosal biopsy
3.Submucosal or subserosal lymphoid aggregates, skip areas, and transmural inflammation are other characteristic microscopic features of CD
4.CD can affect the entire GI tract from the mouth to the anus. The terminal ileum is affected in 90% of patients with small-intestinal disease
5.The inflammation in Crohn’s disease and other inflammatory bowel disorders has been linked to a loss of tolerance to self-antigens which would support the existence of specific autoantibodies in disease phenotypes
6.Genome wide association studies have demonstrated immune-related susceptibility loci
7.Emerging evidence suggests that there is an innate immune response to a low-diversity and altered gut microbiota that initiates and perpetuates the chronic inflammation seen in IBD
1.Elevated ESR and CRP
2.Hypoalbuminemia, anemia and leukocytosis occur in severe patients
3.Endoscopy:
a.Aphthous ulcers, fistulae, and skip areas with rectal sparing. Important for biopsy of affected areas
b.Wireless capsule endoscopy allows visualization directly; visualizes the entire small bowel mucosa
4.Biomarkers:
a.Autoantigenic targets:
i.The major zymogen granule membrane glycoprotein 2 (GP2) is evaluated in CD
1.Radiographic evaluation:
a.Thickened folds and aphthous ulcers in early disease. Strictures, fistulas, inflammatory masses and abscesses are seen in long standing disease
b.The “string sign” is due to long areas of circumferential inflammation
2.CT enterography demonstrates mural enhancement, stratification, engorged vasa recta and perienteric pathology
1.The incidence of UC is 2.2 to 14.3 patients per 100,000 person-years
2.The prevalence is 37 to 246 patients per 100,000 person-years
3.The male to female ratio is 1:1
4.There is increased frequency of both CD and UC in patients of Jewish ancestry
1.Crampy abdominal pain
2.Diarrhea and rectal bleeding
3.Tenesmus
4.Passage of mucus
5.The disorder may present acutely but most often there is an insidious onset over weeks to months
6.Tenesmus (urgency with a feeling of incomplete evacuation) but rarely have severe abdominal pain
7.In patients with distal disease, proctitis or proctosigmoiditis, intestinal transit time slows which may cause constipation
8.Blood mixed stools or grossly bloody diarrhea may occur if the disease spreads beyond the rectum. There is usually increased transit time due to inflammation
9.Diarrhea may be nocturnal or postprandial
10. A subgroup of patients have vague lower abdominal discomfort or mild central abdominal cramping which may be exacerbated by active disease
11. Anorexia, nausea, vomiting, fever and weight loss may be associated
1.Proctitis causes a tender anal canal with blood
2.Extensive disease is associated with abdominal tenderness over the colon
3.Toxic colitis causes severe abdominal pain and bleeding
4.Megacolon patients have hepatic tympany
5.Peritonitis occurs with perforation
1.The disorder is restricted to the mucosa and superficial submucosa. The deeper layers are unaffected other than in fulminant disease
2.The crypt architecture of the colon is disrupted becoming bifid and decreased in number with a gap between the crypt base and the muscularis mucosa
3.A subgroup of patients have plasma cells and basal lymphoid aggregates
4.The mucosa demonstrates vascular congestion, edema, focal hemorrhage and an inflammatory cell infiltrate
5.There is neutrophilic cryptitis and in time cryptic abscess formation
1.Active disease is associated with an increased ESR, C-reactive protein, platelet count and decreased hemoglobin
2.Fecal calprotectin is increased (released by activated neutrophils) and correlates with inflammation
3.Endoscopy
1.Single contrast barium enema:
a.Earliest change is fine mucosal granularity
b.As the disease evolves the mucosa thickens and develops superficial ulcers
c.When the ulcers have penetrated the mucosa they are called “collar-button ulcers”
d.The haustral folds become thickened and edematous and the colon becomes shortened and narrowed
e.There may be post-inflammatory or pseudopolyps, adenomatous polyps as well as carcinoma in long standing disease
f.CT scanning is not as diagnostic as endoscopy or barium enema
1.In some studies of western populations, diverticulosis may affect 50% of the populations > 60 years of age but only 20% of patients with diverticulosis develop symptomatic disease
2.The mean at presentation of the disease is 59 years of age
3.The prevalence between males and females is equal
1.Acute uncomplicated diverticulitis presents with:
a.Left lower quadrant abdominal pain
b.Anorexia
c.Fever
d.Obstipation
2.Perforation:
a.Less than 25% of patients present with generalized peritonitis from diverticular perforation
b.Physical examination reveals abdominal distention and localized or generalized peritonitis
1.The two types of diverticula that occur in the intestines are true or pseudodiverticula
2.A true diverticulum is the herniation of the entire bowel wall while a pseudodiverticulum is the protrusion of only the mucosa through the muscularis propria of the colon. Pseudodiverticula are the most common type that affects the colon
3.The protrusion occurs where the nutrient artery (vasa recti) enters the muscularis propria. The sigmoid colon is most commonly affected
4.The location of the diverticula is posited to be the result of high pressure generated by the muscular sigmoid colon
5.Diverticulitis is inflammation of a diverticulum from material within the protruded diverticular sac and the formation of a fecalith. The vasa recti are either compressed or eroded that causes perforation or bleeding
6.Most bleeds are self-limited, occur in patients > 60 years of age who are hypertensive, atherosclerotic and regularly use nonsteroidal anti-inflammatory drugs
7.The low fiber content of the Western diet is associated with decreased intestinal content and size of the lumen that may cause transmission of muscular contraction pressure to the wall of the colon. Other factors that may be important in pathogenesis are low grade inflammation, altered intestinal microbiota, visceral hypersensitivity and abnormal colonic motility
1.Barium enema and colonoscopy should not be obtained during acute attacks due to the risk of perforation
2.Fever with associated leukocytosis is associated with complicated diverticular disease which includes perforation or abscess formation
1.CT:
a.Sigmoid diverticula
b.Thickened colonic wall (>4mm)
c.Inflammation of the pericolic fat
d.An abscess may be detected in 16% of patients
1.Diverticulitis
2.Salpingitis
3.Inguinal hernia
4.Ectopic pregnancy
5.Nephrolithiasis
6.Irritable bowel syndrome
7.Inflammatory bowel disease
1.Definition:
a.IBS is a disorder with complex symptoms that manifest as abdominal pain or discomfort associated with altered bowel patterns
b.There is an estimated prevalence of 5.8 to 17.5% of the population
c.The Rome III criteria have categorized the disorder into subgroups:
i.IBS with constipation
ii.IBS with diarrhea
iii.Mixed type IBS
iv.IBS unsubtyped
d.Approximately 10% of patients with infective gastroenteritis may develop IBS
1.Abdominal pain:
a.Variable in intensity and location
b.Its location is the hypogastrium in 25%, the right side in 20%, the left side in 20% and the epigastrium in 10% of patients
c.It is episodic and crampy in quality but may be superimposed on a chronic ache. It varies greatly in intensity
d.Pain is usually present only during wakefulness, although severely affected patients have sleep disruption
e.Pain may be exacerbated by eating or emotional distress and may be relieved by defecation or passing flatus
f.Menstrual and premenstrual periods may exacerbate symptoms
2.Altered bowel habits:
a.A change in bowel pattern is the most consistent manifestation of IBS. The most common pattern is constipation that alternates with diarrhea; constipation increases and becomes intractable over time. Many patients suffer tenesmus (a feeling of incomplete evacuation)
b.Diarrhea may be predominant in a subgroup of patients and is characterized by a small volume of loose stools (<200 ml). There is no nocturnal diarrhea. It may be exacerbated by emotional stress or eating. The stool may be associated with a large amount of mucus.
c.Bleeding, malabsorption and weight loss are not features of IBS
d.Abdominal distension and belching are frequent. Many patients have impaired transit and tolerance of an intestinal gas load
e.Approximately 25% of patients have dyspepsia, heartburn, nausea and vomiting
1.IBS is considered to be a multifactorial and heterogeneous disease
2.Possible mechanisms:
a.Inflammation
b.Immunity
c.Infection
d.The gut microbiota
e.Psychosocial factors
f.Stress
g.An abnormal brain-gut axis
3.Recent studies support:
a.Genetic susceptibility
b.Diet / drug intolerance
c.Environmental pollutants as possible associations of IBS
4.Serotonin (5-HT) mediates intercellular signal transmission in the gut; it is synthesized by enterochromaffin (EC) cells (90%) and the serotonergic neurons of the myenteric plexus. EC cells are the primary source of enteric 5-HT in the gastrointestinal tract and 5-HT inactivation may be as important as its release in maintaining gut homeostasis
5.Enteric 5-HT is essential for secretion, motility and bowel sensation; the involvement or alteration of the metabolism of this neurotransmitter is critical in the pathogenesis of IBS. Higher concentrations of 5-HT are found in IBS patients as compared to healthy controls that is associated with depressed serotonin transporter (SERT) mRNA. The regulation of SERT in IBS pathogenesis is under intense investigation as it is now postulated that 5-HT signaling is an essential component of this disorder
1.The bidirectional Brain-Gut Axis:
a.Brain-gut interaction is critical in modulation of gut function. Afferent information from the lumen of the gut is transmitted from enteric, spinal, and vagal inputs to the CNS that trigger homeostatic reflexes in response to physiologic and pathologic visceral stimuli:
i.These reflexes are affected at the level of the:
1.Enteric nervous system
2.Spinal cord
3.Pontomedullary nuclei
4.Limbic CNS areas
ii.Vagal visceral afferent input is a component of modulation of:
1.Emotion
2.Pain sensitivity
3.Satiety
4.Immune response
iii.Descending cortico-limbic modulation can set the gain of enteric reflexes. This top down modulation may occur during:
1.Sleep
2.Stress response
3.Fear
4.Anger
The clinical observations of abdominal pain during palpation of the sigmoid colon and during endoscopic evaluation suggested that IBS patients have visceral hypersensitivity. Barostat controlled balloon distention of the colorectum demonstrates:
1.Lowered mechanical thresholds for perception
2.Increased sensory ratings and viscerosomatic referral patterns.
These findings support:
1.Up-regulation of the sensitivity of visceral afferent pathways,
2.Spinal or brainstem alterations in sensitivity to visceral afferents
3.CNS amplification of visceral input
Evidence supports mucosal immune activation in maintaining a chronic state of visceral afferent sensitization possibly by spino-bulbo-spinal pain amplification through the rostral ventromedullary nucleus (RVM). However a large number of patients with inflammatory bowel disease who suffer similar symptomatology do not have visceral hypersensitivity.
In a subset of IBS patients, there is evidence that enhanced stress responsiveness is important in the development of IBS. In response to emotional or physiologic stress, there is activation of the emotional motor system that includes:
1.Ascending monoaminergic pathways
2.The autonomic system
3.The hypothalamic-pituitary adrenal axis as well as endogenous pain modulatory systems
4.Corticotropin-releasing factor (CRF), a neuropeptide, integrates these outputs in response to stress
5.There are long-term consequences in this system from early aversive events
6.Central stress circuits have been demonstrated to alter visceral nociceptive responses
7.Mild stress associated with anxiety states may cause hyperalgesia
8.Alteration of central pain modulation has been suggested from functional MRI paradigms:
a.IBS patients demonstrate increased activation of the anterior cingulate cortex in response to rectal stimulation; this region is responsive to emotional and attentional stimuli
b.The dorsal ACC also projects to major components of the endogenous pain modulating system. There may be facilitation of pain circuits to a visceral stimulus
c.Some fMRI studies have demonstrated decreased activation of the PAG which suggests less activation of inhibitory pain circuitry
d.IBS patients have also demonstrated increased activation of limbic and paralimbic areas that include the amygdala, hypothalamus, ventral and rostral ACC and dorsomedial prefrontal cortex than ulcerative colitis patients or healthy controls
e.Other fMRI studies have demonstrated that IBS patients have hypervigilance for visceral afferences
1.In patients greater than 40 years of age:
a.Air-contrast barium enema
b.Colonoscopy
2.The diagnostic evaluation suggested by the American Gastroenterological Association is determined by:
a.The duration of symptoms
b.The change in symptoms over time
c.The age and sex of the patient
d.Prior diagnostic studies
e.A family history of colorectal cancer
f.Psychological dysfunction
1.Epigastric or periumbilical pain:
a.Biliary tract disease
b.Peptic ulcer
c.Intestinal ischemia
d.Carcinoma of the stomach or pancreas
2.Pain in the lower abdomen:
a.Colon diverticular disease
b.Inflammatory bowel disease
c.Ulcerative colitis and Crohn’s disease
d.Carcinoma of the colon
3.Postprandial pain with bloating, nausea and vomiting:
a.Gastroparesis
b.Partial intestinal obstruction
c.Intestinal infestation Giardia lamblia
4.Acute intermittent porphyria (painful constipation)
5.Lead poisoning (painful constipation)
1.The spleen is located in the left upper quadrant (LUQ) and is attached to the stomach by the gastrolienal ligament and to the kidney by the lienorenal ligament
2.Accessary spleens are seen in approximately 20% of patients
3.The major functions of the spleen are:
a.Quality control of erythrocytes performed in the red pulp by removal of defective and senescent RBCs
b.Synthesis of antibodies in the white pulp
c.Removal of antibody tagged bacteria and RBCs from the circulation
4.The spleen enlarges from:
a.Increased demand of its basic functions,
b.Abnormal splenic or portal blood flow, and
c.Infiltration
1.The most common symptoms of diseases of the spleen are pain and a heavy sensation in the left upper quadrant
2.Pain may occur from acute splenic swelling due to stretch of its capsule, infarction or inflammation
3.Occlusion of splenic blood flow from thromboembolism may cause LUQ and pleuritic chest pain
4.Vascular occlusion in children with sickle cell disease is painful. In the past it was felt that splenic infarction was painless
5.Rupture of the spleen:
a.From trauma or infiltrative disease may cause a break in the capsule with intraperitoneal bleeding, shock and death
b.Referred pain is often to the cap of the left shoulder (subdiaphragmatic C5 nerve root irritation)
6.A palpable spleen is the major physical finding in disorders of the organ. The normal spleen weighs less than 250 grams, decreases with age and lies entirely within the rib cage
1.Reticuloendothelial cell hyperplasia
2.Immune hyperplasia
3.Disordered immunoregulation
4.Extramedullary hematopoiesis
5.Abnormal splenic or portal blood flow
6.Infiltration by benign and malignant disorders
7.Infectious agents (malaria)
1.The abnormalities are determined by the underlying systemic illness
2.Erythrocyte counts may be decreased with thalassemia major, SLE, cirrhosis with portal hypertension
3.Increased erythrocyte counts are seen with polycythemia vera
4.Granulocyte counts may be decreased with Felty’s syndrome, congestive splenomegaly or leukemia, or increased with infectious or inflammatory diseases
1.MRI or CT of the abdomen
1.Mechanical obstruction can occur at any level of the urinary tract from the renal calyces to the external urethral meatus
2.Areas of narrowing include:
a.The ureteropelvic junction
b.Ureterovesical junction
c.The bladder neck
d.Ureteral meatus
3.Blockage above the level of the bladder causes unilateral dilatation of the ureter and the renal pyelocalyceal system
4.In adults, urinary tract obstruction (UTO) is primarily acquired from pelvic tumors, calculi and urethral stricture
1.Pain is the most prominent symptom and is caused by distention of the collecting system or the renal capsule
2.The rate of distention rather than its degree determine the severity of pain
3.Ureteral stone pain:
a.Is excruciating (10/10 on a visual analogue scale) and is known as renal colic
b.The pain is continuous with little fluctuation in intensity
c.Radiation is to the lower abdomen, testes, tip of the penis and labia
d.In contrast, chronic narrowing of the ureteropelvic junction may be painless but can destroy the affected kidney
e.Flank pain with micturition is strong evidence for vesicoureteral reflux
f.Associated azotemia develops with:
i.Bladder outlet obstruction
ii.Acute renal failure and anuria should be suspected with bilateral obstruction
iii.Polyuria and nocturia are seen with partial obstruction
iv.Fluctuation in urine output in a patient with azotemia is suggestive of intermittent or partial obstruction
v.Obstruction at or below the level of the bladder is associated with hesitancy and difficulty with initiating micturition, post void dribbling, urinary frequency and incontinence
1.UTO is associated with urinary tract infection and urolithiasis
2.Hypertension may occur with acute and subacute unilateral obstruction (due to release of renin by the affected kidney)
3.Prostatic enlargement
4.Congenital lesions
5.Intra-abdominal mass lesions
6.Urinalysis may reveal hematuria, pyuria and bacteriuria
1.Abdominal scout film may demonstrate nephrocalcinosis or a radio-opaque stone
2.Abdominal ultrasonography to evaluate renal and bladder size and pyelocalyceal contour; ultrasonography is approximately 90% specific and sensitive for hydronephrosis
3.Intravenous urography; retrograde or antegrade urography in azotemic patients
4.Voiding cystourethrography is used for the diagnosis of vesicoureteral reflux, bladder neck and urethral obstruction
5.CT is used to evaluate intra-abdominal and retroperitoneal causes of obstruction
1.Renal infarctions are rare. In one large series of 250,000 patients seen in an emergency department 1.4% had an infarction
2.The etiology of acute renal infarction is usually an embolus that arises from the heart or the aorta. The most common underlying disorder is atrial fibrillation
1.Acute flank pain is most typical that is associated with tenderness
2.The pain is continuous
3.Pain may be experienced in lumbar areas; abdominal pain is common
4.Associated symptoms and signs:
a.Acute elevation of blood pressure (renin release in the peri-infarction zone)
b.Nausea and vomiting
c.Elevated temperature > 37.5oC
d.Urinary symptoms
1.Thrombi from the suprarenal aorta
2.Renal artery dissection
3.Hypercoagulable states
4.Endovascular studies
5.Cocaine
6.Sickle cell disease
7.A predilection for infarction of the upper pole
1.Elevated C-reactive protein
2.Elevated lactate dehydrogenase
3.Leukocytosis
4.Microscopic hematuria and proteinuria
5.Elevated serum creatinine and alkaline phosphatase
1.CT angiography
2.Renal angiography
a.A wedge-shaped zone of peripheral hypodensity that does not enhance
1.Urinary tract infections are divided into lower tract that includes the ureters and bladder and the upper tract that includes:
a.Acute pyelonephritis
b.Prostatitis
c.Intrarenal and perinephric abscess
2.Urethral and bladder infections are considered mucosal while tissue invasion occurs with prostatitis, pyelonephritis and renal abscess
3.The infections are categorized as catheter-associated (nosocomial) and non-catheter-associated
4.The majority of acute symptomatic infections occur in young women
5.UTIs are unusual in male patients <50 years of age
1.Urinary tract infections are extremely common in females and cystitis is the major cause of these infections
1.Dysuria
2.Urgency and frequency
3.A sensation of bladder fullness
4.Suprapubic tenderness to palpation
5.Flank pain and costovertebral angle tenderness is more suggestive of upper tract UTI but may be seen with cystitis
6.Rarely there are fever, chills and malaise; these symptoms are much more common with upper UTI
1.Escherichia is the cause of the majority of uncomplicated bladder infections.
2.Other pathogens are:
a.Staphylococcus saprophyticus
b.Proteus mirabilis
c.Klebsiella pneumonia
d.Enterococcus faecalis
1.Urinalysis for pyuria:
a.Positive leukocyte esterase dipstick test
b.Leukocytes in unspun fresh urine (greater than 10 WBCs is abnormal)
c.50% of patients have microscopic hematuria
d.Low grade proteinuria
2.Urine culture is the criterion standard for diagnosis
1.Urethritis is documented in approximately 4 million Americans per year. There are over 700,000 new patients with gonococcal urethritis annually in the USA. The incidence of both gonococcal and non-gonococcal is increasing
2.Risk factors for contracting non-gonococcal urethritis secondary to a sexually transmitted disease are:
a.Contraceptive use
b.Age at first intercourse (younger age is associated with higher risk)
c.Number of sexual partners
d.Sexual preference
e.Previous STDs
1.A large number of patients are asymptomatic and are diagnosed due to partner screening. Approximately 75% of women with Chlamydia trachomatis infections are asymptomatic
2.Symptoms start 4 days to 2 weeks after contact with an infected sexual partner
3.Urethral discharge of yellow, green, brown or blood-tinged material
4.Dysuria is usually localized to the distal penis or urethral meatus. It is most severe during the first morning void. It is exacerbated by alcohol
5.Urinary frequency and urgency are usually absent and suggest prostatitis or cystitis
6.Itching of the urethra or irritation may persist between voidings. Some patients report itching rather than pain or burning with urination
7.Orchialgia may occur in men expressed as heaviness in the genitals. Testicular pain may be epididymitis or orchitis
8.Symptoms may worsen during the menstrual period
9.Systemic symptoms are usually absent (fever, chills, hyperhidrosis, or nausea).
10. If systemic symptoms are present they may be from:
a.Disseminated gonococcemia
b.Pyelonephritis
c.Arthritis
d.Pneumonia
e.Otitis media
f.Reactive arthritis
g.Iritis
h.Rash that involves the palms and soles
1.Neisseria gonorrhoeae
2.Chlamydia trachomatis
3.Ureaplasma urealyticum
4.Mycoplasma hominis
5.Mycoplasma genitalium
6.Trichomonas vaginalis
7.Multiple rare organisms
8.Post traumatic urethritis occurs in 2 to 20% of patients with intermittent catheterization
1.Demonstration of a mucopurulent or purulent discharge
2.A urethral smear with 5 or more leukocytes per oil immersion field
3.A first voided specimen with leukocyte esterase on dipstick
4.More than 30% of patients with NGU have no leukocytes in their urine
5.PCR assays for gonococcal urethritis and Chlamydia infections
1.A population based study in the USA demonstrated a rate of 15-17 patients per 10,000 females and 3-4 male patients per 10,000 of the population
2.Some women who initially present with lower urinary tract symptomatology have pyelonephritis
1.The classic triad of acute pyelonephritis is fever, costovertebral angle pain, nausea, and vomiting. The signs and symptoms may not present concomitantly and all may not occur
2.Signs and symptoms develop rapidly usually over hours to a day. Rarely they are insidious and develop over weeks
3.Symptoms of cystitis may be concomitant
4.Pain:
a.Pain may be mild to severe
b.Flank pain is usually more severe over the affected kidney but may be bilateral
c.Pain may radiate to the lower middle lumbar dermatomes as well as across the surapubic area
d.Suprapubic radiations may be described as:
i.Discomfort
ii.Heaviness
iii.Pressure
e.Upper abdominal pain is unusual
f.Radiation of pain to the groin suggests a ureteral stone
5.Fever is not always present but if present may exceed 103oF (39.4oC). Patients may have rigor and chills without fever
6.Malaise is frequent and severe
7.Anorexia is common; nausea and vomiting vary and diarrhea is rare
8.Elderly patients may present with fever, alteration of mental status or decompensation in another organ system
1.If fever is present it may be greater than 103oF (39.4oC); it may be subnormal in patients with gram negative sepsis
2.Tachycardia is frequent in association with dehydration and sepsis
3.Low blood pressure suggests shock from sepsis or perinephric abscess
4.There is suprapubic tenderness without rebound; there is no rigidity or guarding. Bowel sounds are usually normally active
5.Pelvic examination:
a.Cervical, adnexal and uterine tenderness should be absent
1.Corticomedullary abscess:
a.Patients present with chills, fever, flank and abdominal pain
b.There is often dysuria and nausea and vomiting
c.The abscess is usually associated with vesicoureteral reflux or obstruction
1.The abscess is located between the renal capsule and the renal fascia and is secondary to chronic or recurrent pyelonephritis, rupture of a suppurative process from the kidney, dissemination from the blood
2.The abscess is usually restricted to the perinephric space but can extend to the colon, flank, and groin. Unusual extension is to the lung, paracervical region, peritoneal cavity, psoas muscle, skin or the subphrenic space
3.Symptoms develop insidiously and may include:
a.Fever and chills
b.70% have unilateral flank pain
c.Dysuria (40% of patients)
d.Nausea and vomiting
e.Costovertebral angle tenderness
f.Pain may be referred to the hip, thigh or knee
g.A flank or abdominal mass may be palpated in less than 50% of patients
h.Curvature of the spine away from the involved kidney
1.Patients present with chills, fever and flank or abdominal pain
2.Physical examination may demonstrate a flank or lumbar mass
1.Complications occur in patients with diabetes mellitus, chronic renal disease, all forms of immunosuppression, renal transplantation and HIV infection
2.Complications include:
a.Acute kidney injury
b.Chronic hypertension and renal failure
c.Sepsis (most often gram negative)
d.Renal papillary necrosis
e.Xanthogranulomatous pyelonephritis
3.Corticomedullary abscess:
a.Usually associated with a urinary tract anomaly (vesicoureteral reflux or obstruction)
b.The usual organism is Enterobacteriaceae
4.Perinephric abscess:
a.The abscess is located between the renal capsule and the encompassing renal fascia
b.The suppuration is usually from chronic pyelonephritis
5.Renal cortical abscess
a.Usually caused by hematogenous spread of S. aureus; more common in men than women
1.Urinalysis
2.Urine culture
1.CT to delineate alteration in renal parenchymal perfusion, contrast excretion, perinephric fluid and nonrenal disease
2.MRI for evaluation of infection, masses, obstruction and vasculature
3.Ultrasonography
4.Scintigraphy
1.Ovarian cysts are demonstrated by ultrasound (transvaginal) in a large proportion of premenopausal women and up to 18% of post-menopausal women
2.Mature cystic dermoids are greater than than 10% of all ovarian neoplasms
3.Luteal cysts are seen after ovulation in reproductive-age women and most benign neoplastic cysts occur during this period
1.Most patients with ovarian cysts are asymptomatic and are found during routine pelvic examination or by ultrasonography
2.Malignant ovarian cysts often do not cause symptoms until they are advanced
3.Pain or discomfort in the lower abdomen
4.Discomfort (dyspareunia) with intercourse
5.Difficulty with defecation
6.Tenesmus and desire to defecate if the cyst puts pressure on the large bowel
7.Increased micturition from pressure on the bladder
8.Irregular menstrual periods with abnormal vaginal bleeding
9.Abdominal fullness and bloating
10. Early satiety, indigestion or heartburn
11. Associated with endometriosis
12. Dull bilateral pain (theca-lutein cysts)
13. Adnexal or cervical motion tenderness
14. Cyst rupture or torsion:
a.Severe acute pain
b.Sharp and unilateral pelvic pain that may be associated with trauma, exercise or coitus
c.Abdominal distension
d.Hyperpyrexia
e.Abdominal rebound tenderness and guarding
f.A large cyst may be palpable on abdominal examination
1.An ovarian cyst is a fluid filled sac within the ovary
2.Most ovarian cysts are related to the ovulatory cycle and are either follicular or corpus luteum cysts
3.Other cysts include:
a.Those due to endometriosis
b.Dermoids
c.Cystadenomas
d.Pelvic inflammatory disease
e.Rarely in association with pelvic cancer
1.Urinary pregnancy test
2.CBC
3.Urinalysis
4.Cancer antigens CA125
1.Ultrasonography
2.Definitive diagnosis is based on histologic analysis
1.Ovarian cancer develops from germ cells, stromal cells or epithelial cells:
a.Epithelial ovarian cancer is the most common and the leading cause of death from gynecologic cancer in the USA
b.Epithelial cancers are uncommon prior to age 40 and have a higher incidence in women with a family history, those that have been exposed to asbestos or talc and those with disordered ovarian function
2.Familial cases cause approximately 10% of all ovarian cancer
3.Two types of autosomal dominant familial cancers have been delineated:
a.Breast / ovarian cancer syndrome and
b.Lynch type II cancer family syndrome
4.Two susceptibility genes have been identified in hereditary breast / ovarian cancer, BRCA1 located on chromosome 17q12-21 and BRCA2 that maps to chromosome 13q12-13. Both are tumor suppressor genes that synthesize nuclear proteins that interact with RAD51 that assists in the repair of DNA double strand breaks. RAD51 is overexpressed in many cancers
1.Approximately 70% of patients are diagnosed after the cancer has spread beyond the pelvis
2.Abdominal pain, bloating and urinary symptoms are indications of advanced disease
3.Localized ovarian cancer is most often asymptomatic. If symptomatic, patients complain of:
a.Progressive enlargement of an ovarian tumor may produce urinary frequency or constipation
b.Rarely torsion of the mass causes an acute abdomen
c.There is rare vaginal bleeding
d.Physical examination may reveal a palpable adnexal mass
e.The mass is solid, irregular and fixed; other adnexal masses include:
i.Pedunculated uterine fibroid
ii.Endometriosis
iii.Benign ovarian neoplasms
iv.Inflammatory bowel disease
1.Epithelial tumors account for 85% of ovarian neoplasms, approximately 1/3 of which are malignant. Epithelial tumors of malignant potential have cytological features of malignancy but have not invaded the stroma
2.The other two types of malignancies of the ovary are stromal and germ cell tumors
3.Metastases to the ovary occur from the breast, colon, pancreas, and stomach. The Krukenberg tumor is a bilateral ovarian metastasis from a mucin secreting gastrointestinal cancer
1.CA-125 is relatively insensitive
1.Abdominal CT or MRI scans delineate the tumor and the extent of its spread
2.Ultrasonography of the abdomen and pelvis are useful screening procedures. Transvaginal sonography is frequently used but is associated with significant false-positives
1.Carcinoma of the endometrium is the most common female pelvic malignancy. Approximately 75% of these tumors are restricted to the body of the uterus at the time of diagnosis and are curable
2.It is a disease of postmenopausal women. Approximately 25% of women are affected prior to fifty years of age
3.Risk factors include:
a.Obesity
b.Low fertility index
c.Early menarche
d.Late menopause
e.Chronic anovulation
4.Chronic unopposed estrogen replacement and tamoxifen use have increased risk
1.The cancer occurs most frequently in the sixth and seventh decades
2.Abnormal vaginal discharge (90%), abnormal postmenopausal bleeding (80%), and leucorrhea (10%)
3.Rarely there is pain with micturition, intercourse and in the pelvis
4.Uterine papillary, serous or clear cell cancers may present with abdominal pain and bloating or with symptoms from metastasis
5.Uterine sarcomas present similarly to endometrial carcinomas with vaginal bleeding or pelvic pressure
6.Leiomyosarcoma present most commonly in the sixth decade with irregular menses or postmenopausal bleeding, pelvic pain or pressure and a rapidly expanding pelvic mass. There is a similar presentation for endometrial stromal carcinoma
7.Weight loss, anorexia, pelvic pain or pressure associated with difficulties with micturition and defecation are signs and symptoms of advanced uterine cancer
1.Approximately 80% of endometrial carcinomas are endometrial adenocarcinomas
2.Less frequent tumors include:
a.Clear cell
b.Papillary serous carcinoma
3.Sarcomas account for less than 9% of uterine corpus malignancies and include:
a.Mixed homologous mullerian tumors (40-50%)
b.Leiomyosarcomas (40%)
c.Endometrial stromal tumors (sarcomas) (8-10%)
1.Endometrial biopsy is often the first procedure performed and the degree of malignancy often determines the extent of imaging
1.Ultrasonography helps to exclude other pelvic pathologies
2.Chest radiographs to rule out metastatic disease
3.CT of the chest, abdomen and pelvis in all patients with high grade cancer and those with sarcoma of the uterine body
1.Definition:
a.Leiomyomas of the uterus are benign growths that arise from the smooth muscle and connective tissue of the uterus
b.Rarely a uterine leiomyoma undergoes malignant transformation to a leiomyosarcoma. The incidence of this occurrence is less than 1% and may be as low as 0.2%
c.Approximately 20%-30% of women develop fibroids by the age of 50. They have a genetic tendency
1.Small fibroids are usually asymptomatic. Signs and symptoms depend on both the location and the size of the growth
2.Abnormal uterine bleeding
3.Heavy or painful menstrual periods
4.Abdominal discomfort or bloating
5.Painful defecation
6.Low back pain
7.Urinary frequency or retention
8.Rarely infertility (possibly 3%)
9.Dyspareunia
1.Fibroids are round, well circumscribed but not encapsulated growths
2.Histologically the cells are elongated, spindle-shaped with a cigar-shaped nucleus in a whorled pattern
3.Most fibroids arise in the muscular wall of the uterus. They may be single or multiple
4.Secondary changes in a fibroid include hemorrhage, necrosis, calcification and cyst formation
5.Rarely fibroids metastasize; they enlarge in a benign fashion but cause symptoms due to their location. Extrauterine metastasizing leiomyomas grow in the lungs and lymph nodes. They may invade blood vessels and grow diffusely on the peritoneal and omental surfaces
6.Fibroids are monoclonal tumors and 40-50% demonstrate chromosomal abnormalities
7.In 70% of patients there are specific mutations of the MED12 protein
8.Estrogen and progesterone have a mitogenic influence on leiomyoma cells
1.In palpable growths on pelvic examination gynecologic ultrasound is the standard screening tool that demonstrates focal masses that are heterogenous with shadowing of the ultrasound beam
2.MRI delineates the size and location of the fibroids within the uterus
1.The mortality rate of cervical cancer has declined by 50% over the last 40 years due to the widespread screening with the Pap smear
2.It is more common in women from lower socioeconomic groups, in those with early initial sexual activity and or multiple sexual partners and in smokers
1.Most patients are asymptomatic. The disease is detected on pelvic examination
2.Other presentations are abnormal bleeding or postcoital bleeding or spotting that becomes more profuse intermenstrually or during menstruation
3.Lumbosacral back pain
4.Lower extremity edema
5.Urinary symptoms
6.Yellowish vaginal discharge
1.HPV (human papilloma virus) is an important pathogenic viral infection in cervical cancer. Greater than 66 variants of HPV have been identified and many are associated with genital warts
2.HPV types associated with cervical cancer are 16, 18, 31, 33, 52, and 58; approximately 70% of patients are associated with HPV-16 and HPV-18:
a.The protein product of HPV-16 is the E7 protein which binds and inactivates the tumor-suppressor gene Rb
b.The E6 protein from HPV-18 binds to and inactivates the tumor suppressor gene p53
3.Uncomplicated HPV infection can progress to CIN (cervical intraepithelial neoplasia) which precedes invasive cervical carcinoma
4.Carcinoma in situ demonstrates histologic evidence of neoplasia but does not invade through the basement membrane. It can remain stable for 10 years but usually progresses to invasive carcinoma
1.The Pap smear is 90 to 95% accurate for detecting cervical intraepithelial neoplasia but is less effective in detecting invasive cancer or a fungating mass
2.Colposcopic biopsy is required to evaluate any lesion visible on the cervix
1.Chest x-ray, intravenous pyelograms, and CT are required for staging
2.MRI is important for delineating extracervical extension
1.Biliary tract disease includes active cholelithiasis, choledocholithiasis, calculus and acalculous cholecystitis and ascending cholangitis
2.Approximately 30 to 50% of patients older than 65 years of age have gallstones. Cholecystitis affects approximately 10% of patients with gallstones
3.Biliary disease is one of the most common conditions of elderly patients that present with abdominal pain
4.Diagnostic difficulties occur in the 25% of elderly patients that have no significant right upper quadrant pain, less than 50% have fever, vomiting or leukocytosis
5.The major complications of biliary tract disease include:
a.Gallbladder perforation with consequent peritonitis
b.Emphysematous cholecystitis:
i.An acute infection of the gallbladder caused by Clostridium or Escherichia coli (gas forming organisms)
ii.It is rare, but causes a rapidly progressive acute cholecystitis characterized by early gangrene, perforation of the gallbladder wall
iii.Develops in approximately 1% of all patients with acute cholecystitis and is associated with a mortality of 15 to 25%
c.Symptomatic cholelithiasis
d.Choledocholithiasis
e.Calculus and acalculous cholecystitis
f.Ascending cholangitis
g.Gallstone ileus
i.Associated with approximately 2% of small bowel obstructions in elderly patients
1.Approximately 10% of patients with appendicitis are 60 years of age or older. Fifty percent of deaths from appendicitis occur in this subgroup of patients.
2.The rate of perforation is approximately 5x higher in this group than in younger patients because 75% of elderly patients wait more than 24 hours before seeking medical attention
3.Approximately 50% of patients have no fever or leukocytosis at presentation
4.Approximately 1/3 of patients do not have right lower quadrant pain and 25% do not have tenderness or peritoneal signs in the right lower quadrant
5.Only 20% of elderly patients present with the classic signs of acute appendicitis (fever, anorexia and RLQ pain)
1.Diverticulitis is rare in patients younger than 40 years of age. Diverticula occur in 50-80% of patients older than 65 years of age. Diverticulitis causes microperforation of the colon.
2.Approximately 85% of patients have the disease in the left colon
3.Right-sided diverticulitis is a more benign disorder
4.Elderly patients may be afebrile, the white count is only elevated in 50% and only 25% have a guaiac positive stool
1.Mesenteric ischemia causes approximately less than 1% of abdominal pain in elderly patients.
2.Patients have severe abdominal pain but have minimal tenderness on examination
3.Associated vomiting and diarrhea are common
4.Risk factors are important to make this diagnosis and include severe atherosclerosis, atrial fibrillation and a low cardiac ejection fraction
5.Recurrent postprandial abdominal pain (intestinal angina) is rare
1.Bowel obstruction causes approximately 12% of abdominal pain in elderly patients.
2.Cecal volvulus is rare and usually presents as small bowel obstruction.
3.Sigmoid volvulus is more common and distention of the colon greater than 9cm is a sign of impending perforation. Major risk factors for sigmoid volvulus are inactivity and laxative use.
Approximately 5% of men older than 65 years of age have an AAA. The disorder is much more common in males than females with a ratio of 7:1. If the patient is hemodynamically stable at presentation, the mortality is 25% whereas presentation in shock carries a mortality of 80%.
1.Patients may present with clinical features of renal colic or back pain (often at the T10 level)
1.The incidence of peptic ulcer disease is increasing in elderly patients possibly due to the use of nonsteroidal anti-inflammatory agents. If NSAIDs are used consistently, there is 5 to 10 times an increased incidence of PUD.
2.Approximately 35% of elderly patients with PUD present with no pain
3.The most common presentation is with melena
4.Perforation in elderly patients may be painless
5.Free abdominal air may be absent on abdominal radiographs in more than 68% of patients
Gastroenteritis is a diagnosis of exclusion in elderly patients who present with vomiting and diarrhea.
The usual symptoms of dysuria, frequency, and urgency are less common in elderly patients. Patients may present with alterations of mental status with or without fever.
Both myocardial infarction and pneumonia may rarely present as vague abdominal pain.