GI Section
The following are True/False answers:
1.
1 F
2 T
3 T
4 T
5 F
PSEUDOMEMBRANOUS COLITIS
=CLOSTRIDIUM DIFFICILE DISEASE (more appropriate name because pseudomembranes
are uncommon)
Cause: overgrowth of Gram-positive Clostridium difficile in response to a
decrease in normal intestinal flora
Etiologic agent: cytotoxin produced by C. difficile
Predisposed:
(a) complication of antibiotic therapy with tetracycline, penicillin, ampicillin,
clindamycin, lincomycin, amoxicillin, chloramphenicol, cephalosporins
(b) complication of some chemotherapeutic agents: methotrexate, fluorouracil
(c) following surgery / renal transplantation / irradiation; intestinal vascular
insufficiency
(d) shock, uremia
(e) proximal to large bowel obstruction
(f) debilitating diseases: lymphosarcoma, leukemia
(g) immunosuppressive therapy with actinomycin D
Histo: pseudomembranes (exudate composed of leukocytes, fibrin, mucin, sloughed
necrotic epithelium held in columns by strands of mucus) on a partially
denuded colonic edematous mucosa (mucosa generally intact); reactive edema in
lamina propria, submucosa, and eventually subserosa
profuse watery diarrhea, abdominal cramps, tenderness
fever, fecal blood, leukocytosis.
less common: chronic diarrhea, toxic megacolon, hyperpyrexia, leukemoid
reaction, hypoalbuminemia with anasarca,
confluent small yellow plaques (= pseudomembranes) adherent to mucosal surface
seen on endoscopy (50%)
Location: rectum (95%); confined to right + transverse colon (5-27%)
Plain film:
adynamic ileus pattern = moderate gaseous distension of small bowel + colon
"transverse banding" = marked thickening + distortion of haustral folds
"thumbprinting" most prominent in transverse colon
diffusely shaggy + irregular surface (confluent pseudomembranes)
Barium Enema (CONTRAINDICATED in severe cases):
"accordion-like" haustral thickening = contrast material trapped between
distorted thickened closely spaced transverse edematous folds (simulating
intramural
tracts)
pseudoulcerations = barium filling clefts between pseudomembranes
irregular ragged polypoid contour of colonic wall
discrete multiple plaque like lesions of 2-4 mm in size (DDx: polyposis, nodular
form of lymphoma)
N.B.: Risk of colonic perforation!
CT (85% sensitive, 48% specific):
colonic wall thickening of 4-22 mm (61-88%)
smooth circumferential thickening (44%)
accordion sign (51-70%) = alternating bands of edematous haustral folds
separated by intraluminal contrast material
nodular thickening (17%)
homogeneous enhancement due to hyperemia
pericolonic stranding (42%)
ascites (15-25%)
NO colonic abnormality (12-39%)
Dx:(1)Stool assay for Clostridium difficile cytotoxin (detects toxin B):
cumbersome to perform
(2)Enzyme immunoassay test (up to 33% false-negative results): detects toxin A +
B
(3)Stool culture (95% sensitive): not available for 2 days
(4)Pseudomembranes on proctosigmoidoscopy
Cx: peritonitis
Prognosis:15% mortality; most patients recover within 2 weeks
Rx: discontinuation of suspected antibiotic + administration of vancomycin /
metronidazole with attention to fluid and electrolyte balance
2.
Answers:
1. T
2. F
3. T
4. T
5. T
DDX for smooth esophageal narrowing includes: congenital stenosis, web, surgical repair of esophageal atresia, caustic burns, gastric acid-reflux, intubation- reflux, compromise of circulation, XRT of mediastinum, post infection - rare. Lower esophageal narrowing includes: scleroderma, achalasia, diffuse spasm, esophagitis
Classic findings of Barrett's include: high esophageal stricture/ulcer, HH, GER. Strictures are at proximal zone of columnar metaplasia. Can also have strictures in distal esophagus.--peptic stricture. Reticular mucosal pattern is also a finding..
DDX for high esophageal strictures include caustic ingestion, mediastinal XRT, primary or mets, rarely esophageal. involvement by dermatologic disorders such as epidermolysis bullosa dystrophica and benign mucous membrane pemphigoid.
3.
1 T
2 T
3 F
4
TAdult Form Of Hypertrophic Pyloric Stenosis
(secondary to mild infantile form)
acute obstructive symptoms uncommon
nausea, intermittent vomiting
postprandial distress, heartburn
Associated with:
(1) peptic ulcer disease (in 50-74%) (prolonged gastrin production secondary to
stasis of food)
(2)chronic gastritis (54%)
persistent elongation (2-4 cm) + concentric narrowing of pyloric channel
parallel + preserved mucosal folds
antispasmodics show no effect on narrowing
proximal benign ulcer (74%), usually near incisura
4.
1 F
2 T
3 F
4 F
EPIDERMOID CYST OF SPLEEN
= EPITHELIAL CYST = PRIMARY CYST OF SPLEEN
Cause: infolding of peritoneal mesothelium / collection of peritoneal mesothelial
cells trapped within splenic sulci
Histo:(1) mesothelial lining (2) squamous epithelial lining = epidermoid cyst =
squamous metaplasia from embryonic inclusions within preexisting mesothelial
surface epithelium
Age:2nd-3rd decade (average age of 18 years)
May be associated with: polycystic kidney disease
(a) unilocular + solitary (80%)
(b) multiple + multilocular (20%)
average size of 10 cm
peripheral septations / cyst wall trabeculations (in 86%)
curvilinear calcification in wall (9-25%)
may contain cholesterol crystals, fat, blood
Cx: trauma, rupture, infection
5.
1 ?T
2 ?T
3 T
4 T
5 F
Internal Hernia
Incidence:5% of all hernias, responsible for <1% of mechanical small bowel
obstruction
Classification of hernias:
(a) retroperitoneal: usually congenital containing a hernial sac
1.paraduodenal (ligament of Treitz)
2.foramen of Winslow
3.intersigmoid
4.pericecal / ileocolic
5.supravesical
(b) anteperitoneal:
small group of hernias without a peritoneal sac
1.transmesenteric (transverse / sigmoid mesocolon)
2.transomental
3.pelvic (including broad ligament)
A. PARADUODENAL HERNIA (53%)
(a) through fossa of Landzert on left side (3/4)
lateral to 4th portion of duodenum and behind descending + transverse mesocolon
(b) through fossa of Waldeyer on right side (1/4)
caudal to SMA and inferior to 3rd portion of duodenum
B. LESSER SAC HERNIA (<10%)
through foramen of Winslow in retrogastric location
Invaginated gut: ileum > jejunum, cecum, appendix, ascending colon, Meckel
diverticulum, gallbladder, greater omentum
C. HERNIA THROUGH BROAD LIGAMENT (very rare)
after laceration / fenestration from surgery or during pregnancy
6.
1 T
2 T
3 T
4 T
Polyposis Syndromes
1. Adenomatous Polyposis Syndrome
- There is mounting evidence that these (familial polyposis coli, Gardner’s syndrome, and Turcot’s syndrome) are all varying expressions of the same disease - familial adenomatous polyposis syndrome (FAPS). Shared traits:
- relatively rare
- M=F
- autosomal dominant with occasional spontaneous mutations
- 80 -100% penetrance
- responsible gene probably on long arm of chromosome 5 (other genes for colorectal cancer are located here)
- adenomas in the colon are usually tubular or tubulovillous (100 or more adenomas are required for diagnosis)
- usually small (80% <5 mm and sessile)
- most common symptoms are rectal bleeding and diarrhea (because colonic disease predominates.)
- polyps don’t usually develop until puberty, so screening is delayed until patient is 10 y/o.
- untreated patients will develop cancer (and at a much younger age than the general population)
- rectal mucosa must also be removed during total colectomy since there is a high risk of developing cancer in the residual rectal tissue.
- all portions of the GI tract with columnar epithelium may develop adenomas (esophagus has stratified squamous epithelium, so not usually affected.)
- gastric involvement in >50% (fundic (hamartomatous) and adenomatous polyps!)
fundic gland polyps more common in Western countries
fundic polyps gastric adenomas
- not with chronic atrophic gastritis - tubular and villous
- 1-5 mm - sessile, 5-10 mm
- multiple, sessile - >50% multiple
- not premalignant - premalignant (low risk)
- antrum spared (monitor, don’t cut!) - usually distal stomach
- duodenum is 2nd most common site of FAPS
In Japan, 90% have tubular adenomas!! (47-72% Western countries)
- duodenum-periampullary region most frequent site for adenocarcinoma outside of the colon (4% within 5 years S/P colectomy)
- polyps are usually in 2nd portion of duodenum, around the papilla (medial!)
- villous adenomas common, usually periampullary (large, premalignant)
- polyps are also seen in the small bowel, pancreas, and biliary tract
Extraintestinal Manifestations (let the fun begin!)
- skin lesions
- epidermoid cysts frequent
- tend to on face and scalp but can be anywhere
- often appear before GI adenomas!
- lipomas and small fibrous tumors of the skin less common
- bone lesions
- osteomas
- benign, but may be an early marker for FAPS
- angle of mandible, sinuses, outer table of skull ? "screening" Panorex?)
- localized or diffuse cortical thickening of long bones
- dental abnormalities (impacted or supernumerary teeth)
- fibrous lesions
- increased incidence of postoperative peritoneal adhesions
- retroperitoneal fibrosis has been reported
- more commonly, fibroid tumors, especially desmoid tumors of the abdominal wall and mesenteric fibromatosis, occur
- usually seen postoperatively, commonly in women of childbearing age (may grow rapidly and may first appear during pregnancy)
- mets do not occur
- eye lesions
- congenital pigmented lesions of the retina (often bilateral)
- may occur before development of colonic polyposis
- MALIGNANT TUMORS OF THE CNS
- commonly referred to as Turcot’s syndrome (2 cases in 1959!)
- polyps may be less numerous and larger and carcinoma may develop at a younger age
- GBMs/ gliomas most common (most supratentorial), also medulloblastoma
- associated stigmata of FAPS often not seen (can have cafe au lait spots and focal nodular hyperplasia of liver)
- Thyroid carcinoma
- prevalence of thyroid carcinoma is 160X that of general population
- usually papillary, usually multifocal
- usually detected before colonic disease - so consider FAPS in all cases of young female with papillary cancer of thyroid.
2. Hamartomatous Polyposis Syndromes
- Hamartomas of the Cronkhite-Canada, multiple hamartoma (Cowden’s), and juvenile polyposis syndromes have similar histologic features. The Peutz-Jeghers hamartoma has a distinctive morphologic appearance.
- Mucocutaneous and extraintestinal manifestations are common.
Peutz-Jeghers Syndrome
- usually in clusters, carpeting usually doesn’t occur
- treatment is usually polypectomy of troublesome/large polyps
- inherited in autosomal dominant pattern, M=F (40%-50% sporadic, however)
- mucocutaneous pigmentation (95%)
- small brown or bluish-black macules on lips (fades with age) and buccal (doesn’t fade with age) mucosa (less on dorsal fingers, palms, and soles)
- pigment rarely present at birth
- extra-alimentary tract neoplasms (50% - 18X risk of general population, especially pancreatic)
- ovarian sex cord tumor with annular tubules (benign) present in almost all females
- polyps from the stomach to the rectum (95% have small bowel involvement)
- Sertoli cell tumor which produces isosexual precocity unique to Peutz-Jeghers syndrome
- adenoma malignum - well-differentiated adenocarcinoma with aggressive behavior (cervix)
- bilateral breast carcinoma (secondary to estrogen stimulation by ovarian tumors?)
- increased prevalence of GI tract neoplasms (2-16% prevalence of GI tract malignancies)
- most in gastroduodenal area or colon
- polyp is an abnormal mixture of smooth muscle and mucosa (pseudoinvasive appearance)
- can cause recurrent intussusception
- according to Buck's AFIP notes, the polyps rarely occur outside the GI tract.
Cowden’s Syndrome (Multiple Hamartoma Syndrome)
- multiple hamartomas and neoplasms of ectodermal, mesodermal, and endodermal origin
- autosomal dominant with high penetrance
- some features overlap with MEN IIb (mucosal neuroma syndrome - pheochromocytoma, medullary thyroid carcinoma, neuromas (similar to neurofibromatosis))
- mucocutaneous lesions
- occur in all patients and are the hallmark of the disease
- earliest findings (at 20-30 years of age)
- facial papules, oral mucosal papillomatoses, acral keratoses most common
- less characteristic are keratoses located on the dorsum of the hands/ feet/ proximal extremities
- thyroid abnormalities
- most frequent extracutaneous abnormality
- usually goiters and adenomas
- follicular adenocarcinoma in 3-12% (all women)
- breast abnormalities
- 70% have lesions (but 50% was "fibrocystic disease"!!!!)
- cancer prevalence of 30-50%, tends to be bilateral, 30-40 y/o at diagnosis
- GI abnormalities
- multiple nonadenomatous polyps (small and sessile) occur in >35%
- little or no malignant potential
- segmental distribution, most common in rectosigmoid
- usually asymptomatic
- esophageal involvement rare but possible
Cronkhite-Canada Syndrome
- inflammatory polyps in the stomach and colon have no malignant potential
- generalized polyposis syndrome with characteristic ectodermal changes
- occurs sporadically, affected patients are usually around 60 y/o
- innumerable polyps in the stomach, small bowel, and colon in almost all patients
- in the stomach, usually superimposed on thickened rugal folds (may resemble Menetrier’s but the antrum is also involved)
- polyps are usually small and sessile, identical in appearance to the polyps in juvenile polyposis
- GI symptoms followed by abnormalities of the skin (multiple brown macules or confluent areas of hyperpigmentation - hands and feet), hair (abrupt alopecia), and nails (dystrophic changes, possibly loss)
- if patient dies from the disease, it is usually from malnutrition (surgery is usually ineffective, so patients are supportively managed, sometimes with sustained remissions - which may include disappearance of polyps!!)
- 15% of cases progress to malignancy (adenocarcinoma)
Juvenile Polyposis
- two main categories:
- isolated juvenile polyps of childhood (but 50% have more than one polyp)
- usually no family history
- low risk of malignant potential
- most common tumor of the colon in childhood (1% of kids 4-14 y/o)
- painless rectal bleeding most common
- sessile, 2-50 mm diameter
- juvenile polyposis of the colon or entire GI tract (six or more polyps in the colon or rectum or polyps throughout the entire GI tract or family history)
- average age is 32 y/o
- areas of dysplasia in the polyps (therefore, increased risk of cancer)
- 25% of cases are inherited in autosomal dominant fashion
- nonfamilial cases have 25% incidence of congenital abnormalities (hydrocephalus and pulmonary AVMs most common)
- juvenile polyposis of infancy is a subcategory (Infantile Cronkhite-Canada)
- severe diarrhea before 2 y/o
- polyps unevenly distributed throughout GI tract with colon and small bowel most severely involved
- polyps usually 2-3 mm, larger ones are pedunculated
Ruvalcaba-Myhere-Smith Syndrome
(rare, autosomal dominant, pigmented genital lesions, macrocephaly, generalized GI hamartomas, no reported malignancy)
7.
1
2
Celiac Disease
=NONTROPICAL SPRUE = GLUTEN-SENSITIVE ENTEROPATHY
=characterized by malabsorption resulting from atrophy of small intestinal villi
Irritating agent: gliadin polypeptides in wheat, rye, barley, oats
May be hereditary: detected in 15% of 1st-degree relatives
Countries: North America, Europe, Australia, India, Pakistan, Middle East, Cuba
Age: childhood by age 2 years; 30-40 years with M<F; 40-60 years with M>F
Rx: gluten-free diet: corn, rice, tapioca, soya, millet, vitamin supplements
CT:
small bowel dilatation + increased fluid content ± mucosal fold thickening
mild to moderate lymphadenopathy in mesentery / retroperitoneum (up to 12%)
8.
1 T
2 F
3
4 T
5 T
9.
Answers:
1. F
2. T
3. F
4. F
10.
Answer:
1. false
2. true
3. true
4. true
Adenomas are almost always in women. 90% present with mass effect, pain, or hemorrhage. Type V is associated with metabolic disease such as type 1 glycogen storage disease ( von Gierke), DM.
MRI appearance is heterogeneous if there is hemorrhage. Areas of increased T1 signal have fat, methemoglobin hemorrhage. Also increased signal on T2.
Low signal intensity of adenoma on T1 is secondary to necrosis.
11.
Answer:
5. false
6. false
7. false
8. false, true for Sx pt
8a. false
Meckels is a remnant of the vitelline duct. Antimesenteric ileal border w/in 100cm of the ileocecal valve. 15% contain ectopic gastric mucosa
Meckels contains all layers of the bowel; therefore it's a true diverticulum. Symptomatic Meckels occurs more in males than females, BUT asymptomatic Meckel's are in both sexes equally.
Meckel's diverticulum is formed from incomplete invagination of the vitelline duct. Incidence in autopsies is up to 3%. ??? prob doesn't invaginate over time.
Malformation is 3-5X more common in men
12.
Answer:
9. true
10. false
11. true
12. true
13. false, common ( occasional presentation with ulcer in ileal segment-- ?bleeding) prob--false
Ileal dysgenesis is found in adults and is segmental dilatation affecting the distal ileum. Probably developmental in origin, a supposition supported by the presence of ectopic gastric mucosa or other aberrant mucosal linings. There is focal atonicity. Obstructive symptoms. On Barium, see dilated focal segment of ileum and occasionally an ulcer.
Segmental dilatation in kids which is a different disease is thought to be secondary to a neuromuscular disorder.
13.
Answer:
14. True
15. False
16. True
17. False
In an immunocompetent patient, the most common causes of cholangitis are stricture from prior surgery (36%), calculi (30%), sclerosing cholangitis, and an obstructed drainage catheter.
A secondary cholangitis resembling primary sclerosing cholangitis is an uncommon but well-recognized component of AIDS. The condition is thought to be secondary to opportunistic infection by Cryptosporidium, cytomegalovirus, or both. In addition to biliary tract signs and symptoms, patients with AIDS-related cholangitis may suffer abdominal pain and diarrhea from cryptosporidial enteritis.
14.
Answer:
18. false
Duodenal atresia is most commonly associated with Down Syndrome.
15.
Answer:
22. False
23. True
24. ?? false- commonly?-- only 15%
25. True
26. True
27. false????( true diverticula?)
28. false
29. false
In sclerosing cholangitis, chronic obliterative fibrotic inflammation involves the intra- and extrahepatic bile ducts. It appears on cholangiography as multiple strictures in the intra- and extrahepatic biliary tree with pruning of the normal branching pattern, nodular duct walls, and diverticula.
Sclerosing cholangitis can occur either as a primary form (either idiopathic or associated with inflammatory bowel disease or retroperitoneal fibrosis) or may be secondary to cholangitis, surgery, or stone disease. 15% of patients with primary sclerosing cholangitis develop sclerosing cholangiocarcinoma. 70% of patients with primary sclerosing cholangitis have ulcerative colitis. A small percentage of patients with sclerosing cholangitis have an associated fibrosing syndrome involving the retrobulbar area (orbital pseudotumor), salivary gland, thyroid gland, retroperitoneum (retroperitoneal fibrosis), and blood vessels. Associated with sicca complex, Riedel's struma( a chronic proliferating inflammatory fibrosing, process, usually involving both lobes of the Thyroid), retroperitoneal fibrosis, mediastinal fibrosis, but none has been reported consistently.
A greater # of pt are dx earlier asymptomatic secondary to incidentally noted elevated serum alk-phos. Elevation of bilirubin and AST aren't as marked or as consistent as alk-phos. Natural history is variable but usually progressively downhill. No known therapy has been proven effective short of liver transplantation.
Diverticular outpouchings vary in size. Are a characteristic feature of PSC. Appear to develop as herniations adjacent to strictures , whereas others arise as mucosa extensions into a thickened duct wall.
16.
Answer:
30. False
31. True
32. False
33. True
34. True
- exaggeration of normal infolding of luminal epithelium (Rokitansky- Aschoff sinuses)
- associated with proliferation of smooth muscle
- causes marked thickening of GB wall
- can involve entire GB, more commonly segmental (fundus)
- can be confused with cholecystitis or gall bladder carcinoma
- cystic spaces - ring down (comet tail) artifact on ultrasound - projects from nondependent surface of GB wall
- spaces fill on oral cholecystogram
Carcinoma of the gallbladder has been reported anecdotally in adenomyosis but risk of neoplasm is usually not sufficient to indicate prophylactic surgery.
Hyperplastic cholecystoses are benign non neoplastic noninflammatory gallbladder abnormalities which include adenomyomatosis, cholesterolosis, cholesterol polyps.
17.
Answer:
35. True
36. True
37. True
38. False
39. True? is a risk factor for ca., but not necessarily the precursor-- false??
According to Harrison’s, patients with primary sclerosing cholangitis can have signs suggesting acute cholangitis. 70% of patients with primary sclerosing cholangitis can have ulcerative colitis and primary sclerosing cholangitis can precede ulcerative colitis. Primary sclerosing cholangitis usually involves both the intra- and extrahepatic ducts and gives a beaded appearance with strictures. 15% of patients with primary sclerosing cholangitis will get cholangiocarcinoma.
Risk factors for cholangiocarcinoma include PSC, choledochal cyst, familial polyposis, congenital hepatic fibrosis, oriental cholangitis, history of thorotrast exposure.
18.
Answer:
40. False
41. True
42. False
43. True
44. True
- clinical features: fatigue, pruritis, RUQ pain, jaundice, and hepatosplenomegaly
- serum alkaline phosphatase levels are often markedly elevated
- chronic cholestatic liver disease of unknown etiology
- 70% male
- 70% <45 y/o
- 70% have ulcerative colitis
- 40% have gallbladder abnormalities, most common is gallstones
- 15% have gallbladder wall involvement
- 15% have cholangiocarcinoma
- other associated diseases:
sicca complex
Riedel’s struma (chronic thryoiditis)
retroperitoneal fibrosis, mediastinal fibrosis
- specific findings:
cholangiectasis with fibrous obliteration and involvement of intra and extra hepatic ducts
hallmark - multiple segmental strictures involving intra- and extrahepatic ducts with classic beaded appearance
Secondary sclerosing cholangitis is associated with inflammatory bowel disease (ulcerative colitis in 66-75%, occasionally with Crohns disease). However, the term primary is used because there is no convincing evidence that primary sclerosing cholangitis is caused by or is secondary to inflammatory bowel disease.
19.
Answer:
45. False true?-- neonatal hepatitis is a cause.
46. True
47. false
48. True
Causes of enlarged gallbladder:
Obstruction: cystic duct (40%), "cholelithiasis causing obstruction" (37%), cholecystitis with cholelithiasis (11%), Courvoisier phenomenon (10%), pancreatitis
Unobstructed (mostly neuropathic): S/P vagotomy, diabetes mellitus, alcoholism, appendicitis (in children), narcotic analgesia, WDHA syndrome, hyperalimentation, acromegaly, Kawasaki syndrome, anticholinergics, bedridden patient with prolonged illness, AIDS (in 18%)
Hydrops of the gallbladder is defined as massive distention of the gallbladder without evidence of a congenital malformation, stone, or inflammatory etiology. Most cases are idiopathic.
The following are all causes of GB hydrops:
- Kawasaki’s - Sjogren’s - Systemic sclerosis
- nephrotic syndrome - leukemia - familial Mediterranean fever
- TPN - scarlet fever - leptospirosis
- ascariasis - typhoid - staph/strep
In Rumack, hydropic gallbladder may be due to Kawasaki's, pseudomonas, klebsiella, group B strep, neonatal hepatitis, necrotizing enterocolitis, CF with inspissated bile. Can also occur in children on TPN. Transient gallbladder distension can occur in infants or premies and is secondary to transient bile plugging.
20.
Answer:
49. True
50. True
51. False
52. True
Pneumatosis Intestinalis Cystoides represents cystic or arcuate collections of gas located in the subserosa or submucosa - usually in the jejunum. 15% is primary and 85% is secondary
Benign causes:
1) gas dissecting from patients with obstructive lung disease
2) gas entry into the bowel wall from the lumen (ulcer/tear/obstruction)
More malignant causes: (often associated with portal venous gas)
1) ischemic
2) inflammatory (ulcer, inflammatory bowel disease, collagen vascular disease, Whipple disease)
3) infection
21.
Answer:
53. True
54. True
55. True
56. False
57. True
58. False
59. True
Toxic megacolon is defined as acute transmural fulminant colitis with neurogenic loss of motor tone and rapid colonic dilation (>5.5 cm in transverse colon). Ulcerative colitis is the most common cause. BE is contraindicated. The mortality is 20%. Possible causes:
- UC - Crohn’s
- Ischemic colitis - Amebic colitis
- salmonellosis (typhoid fever) - cholera
- shigellosis (bacillary dysentery) - Strongyloides
- Campylobacter - Behcet’s
- pseudomembranous colitis (rarely)
typhoid fever.
22.
Answer:
60. True (if toxic megacolon is present)
61. True
62. False
63. False true
64. False
Other contraindications to BE:
1) immediately following a biopsy with a rigid sigmoidoscope (wait 5 days)
Use single contrast BE for:
1) Hirschsprung’s
2) Acute diverticulitis
3) High grade colonic obstruction
4) colonic fistula
5) ischemic colitis
23.
Answer:
65. True
66. True
67. false
68. false
69. True
The appendix is seen in 10-15% of normals. A normal appendix is small, narrow( < 6mm), blind ending structure with extremely thin wall.
An appendicolith can be an incidental finding and without associated clinical findings, are not indicative of appendicitis. However, of all appendicitis 30% have appendicoliths. US accuracy of dx acute appendicitis is greater than 90%. Sensitivity is 80 - 89%.
Sonographic findings of acute appendicitis include:
noncompressible RLQ tubular structure
target appearance of appendix
appendix diameter > 6mm
lumen distended with anechoic/ hyperechoic material
appendicolith
circumferential loss of submucosal layer of appendicitis
loculated pericecal fluid
prominent pericecal fat.
24.
Answer:
74. True
75. True
76. True
77. True
78. True
Prolonged use of stimulant-irritant cathartics (> 15 years) results in neuromuscular incoordination from clinically increased muscular activity and tonus. This is exclusively a radiographic diagnosis. Involvement is often proximal to distal, with the first changes in the cecum and ileocecal valve. The former often appears conical, while the latter is flattened and gaping. There is general loss of haustration which may extend to the distal colon:
Additional agents which might cause it: phenophthalein, aloin
25.
Answer:
79. True
80. True
81. True
82. True
Kulchitsky cells are argentaffin cells which give rise to carcinoids. Myenteric plexus aka Auerbach's is between the two layers of muscularis. Meissner's is a submucosal plexus. Lymphoid tissue is distributed throughout the GI tract.
26.
Answer:
82. False
83. False
84. False
Rectum, ascending and desc colon are retroperitoneal. Sigmoid and transverse mesocolon are intraperitoneal. The first part of the duodenum is covered by peritoneum. The remainder of the duodenum is retroperitoneal.
27.
Answer:
85. False
86. False
87. False
88. False
89. False
Mallory-Weiss tears are due to sudden increase in intraesophageal pressure from violent projection of gastric contents against a fixed lower esophagus. This results in linear tears at/above/below the gastroesophageal junction (below in 76%). Mallory-Weiss tears are the most common cause of esophagus non-variceal bleeding and account for 5-10% of all acute upper GI bleeds. Most heal spontaneously but some require vasopressin, embolization, or surgery. The tear usually involves the mucosa and submucosa and is self-limited. It is more common in males, usually 30-60 years-old. The tears usually follow binge drinking. The best form of diagnosis is endoscopy, although angiography (left gastric artery injection) can be used. On barium studies the tears appear as linear shallow ulcers (single 77%, multiple 23%) at the gastroesophageal junction. The differential diagnosis is linear ulcers from gastroesophageal reflux. The history is helpful in the differential diagnosis.
28.
Answer:
90. True
91. True
92. False
93. False
94. False
A filiform polyp is a nonspecific histological polyp seen in inflammatory bowel disease. It characteristically has a branching pattern and looks like strands of mucus. It may be the first sign of inflammatory disease. The polyp itself is not precancerous; however, remember that the risk of colon cancer is increased in inflammatory bowel disease. It can cause intussusception in children; however, the most common cause in kids is idiopathic. Filiform polyp is secondary to residual inflammed and/or hyperplastic or reparative tissue protrudes in to the lumen.
Ulcerative Colitis
more common than Crohn’s
4 times more common in whites
2-4 times increased in Jews
peak ages 15-25 and 55-65
urban > rural
familial pattern
Barium Enema findings: acute ulcerative colitis
mucosal granularity and stippling
collar button ulcers
haustral thickening or loss
inflammatory polyps
contiguous circumferential disease
Barium Enema findings: chronic ulcerative colitis
haustral loss
lumen narrowing
loss of rectal valves
widened presacral space
post inflammatory pseudopolyps
complications of ulcerative colitis
cancer significantly higher than general population
annual incidence 10% after first decade of colitis
risk increased with increased extent of disease
toxic megacolon
1.6-13% of patients can be the initial manifestation
most common cause of death directly related to ulcerative colitis
extracolonic manifestations
- liver: hepatitis, sclerosing cholangitis, cholangiocarcinoma, fatty infiltration
- musculoskeletal: arthritis, AS, HOA, AVN
- mucocutaneous: pyoderma gangrenosum, erythema nodosum
- renal: nephrolithiasis, amyloid
- hematologic: anemia, arteritis, thrombocytosis
Crohn's Disease
idiopathic inflammatory disease with granulomatous change
can effect any part of the GI tract
peak 15-25 years old
M=F
familial tendency
Small bowel affected in 80% - Terminal ileum most common location
Small bowel and colon in 50%
isolated to colon in 15- 20%
isolated to perianal area in 2-3%
findings:
aphthoid ulcers
transmural progression
long linear ulcers on the mesenteric border
sacculations anti-mesenteric
inflammatory polyps, pseudopolyps
cobblestone pattern
skip lesions
complications
strictures
abscess
fistulas - terminal ileum most common site
increased incidence of carcinoma (less than ulcerative colitis )
distal ileum preferred site
dismal prognosis
younger age (46 yrs)
Turcot's
Colonic and CNS tumours
29.
Answer:
95. True
96. True
97. False
98. True
99. True
There are approximately 200 deep mucosal glands aligned in parallel rows in the esophagus. Each gland contains several ducts which merge to form a single main duct that extends 2-5 mm in the wall of the esophagus to open at the mucosal surface. Intramural pseudodiverticulosis is due to dilated excretory ducts. Approximately 34-48% of patients with intramural pseudodiverticulosis have Candida colonization. Some feel that Candida predisposes to intramural pseudodiverticulosis; others feel that inflammation around the ducts can cause plugging leading to intramural pseudodiverticulosis. In fact, 80-90% of patients with intramural pseudodiverticulosis have inflammatory disease in the esophagus histologically. Intramural pseudodiverticulosis is therefore a sequela of chronic esophagitis, especially reflux.
40% of patients with intramural pseudodiverticulosis have strictures which can be benign or malignant. Intramural pseudodiverticulosis usually occurs in the elderly, it is twice as common in males, 20% of patients are diabetic, and 15% are alcoholics. Most patients have intermittent or slowly progressive dysphagia which is dramatically relieved with mechanical dilatation.
On esophagography, there are fleck-shaped collections of barium arranged along the axis of the esophagus. They commonly lack communication with the esophageal lumen and thus are differentiated from shallow ulcers. Occasionally they can form bridging structures in the wall of the esophagus resulting in discrete tracks. If the ducts perforate it can lead to intramural pseudodiverticulosis. One half of patients with intramural pseudodiverticulosis have diffuse disease and 1/2 of patients have segmental disease. Many patients with intramural pseudodiverticulosis have high esophageal strictures (40%?).
30.
Answer:
100. True
101. True
102. false
103. True
Our pathologists use nuclear pleomorphism, number of mitoses, and presence of necrosis to distinguish atypical from typical carcinoid. There is a gradation with a lot of inter- (and probably intra-) observer variation. Radiographically, we cannot make a distinction unless metastases are present, which would be atypical.
31.
Answer:
104. False
105. False
106. True
The rectosigmoid is involved in 95%. The mucosa is hyperemic, but sometimes beneath the ulcers, there is a vasculitis raising the question of whether ulcers are caused by an initial vasculitis-induced ischemic necrosis of the mucosa. The ileum is involved in 10-25% ("backwash ileitis").
32.
Answer:
107. True (non propulsive)
108. True (Barrett’s)
109. True
110. False
111. False
Barrett esophagus is an inflammatory condition in which the pseudostratified squamous epithelium of the esophagus is replaced by columnar (gastric) epithelium. It is thought to result from chronic reflux, which is invariably present, and is usually associated with a sliding hiatal hernia. An esophagram reveals a characteristic, finely reticular mucosal pattern. The ulcerations of Barrett esophagus tend to be deep and penetrating, resembling peptic ulcerations in the stomach. They are usually separated from the hiatal hernia by a variable length of normal-appearing mucosa. Strictures form in a small percentage of patients. Technetium pertechnetate examination usually shows increased activity in the affected segment, as the isotope is actively secreted by the gastric-type mucosa. Approximately 10% of patients with this condition have been reported to develop adenocarcinoma. 25-50% pt with GER have abnormal motility. Inflammatory esophagogastric polyps are seen in GER and frequently straddle a HH.
33.
Answer:
112. False
113. True
114. True
115. False
116. False (greater risk-- they have a 10% risk of developing ca rather than a 10% increased risk above baseline)
- 10-20% of esophageal carcinoma is adenocarcinoma
- tylosis = hyperkeratoses on palms and soles
- much higher than 10% of head and neck carcinoma patients actually get esophageal carcinoma
Patients with carcinoma of the esophagus usually present with extensive tumor. They remain asymptomatic during much of their development and therefore often present at a stage which is too advanced to permit cure. The incidence of carcinoma is higher among those with esophagitis, untreated achalasia, lye strictures, diverticula, webs, alcohol, cigarette smoking, hot tea, and aflatoxin (Aspergillus molds found in nuts) exposure. The Plummer-Vinson (Paterson-Kelly) syndrome, which consists of dysphagia, iron deficiency anemia, and mucosal lesions of the mouth, pharynx, and esophagus, has also been linked to esophageal carcinoma. When the cancers become overt, almost 50% of these tumors are located in the middle third, 30% in the distal third, and 20% in the upper third. Histologically, about 60-70% are either poorly or well-differentiated squamous cell carcinoma, and 5-10% are adenocarcinoma. The remainder are undifferentiated. 70% of patients are dead within one year of diagnosis, and the five year survival rate is 5-10%. Metastases occur as a relatively late phenomenon. Between 1-15% of pt with head and neck cancer develop an esophageal SCC., so they have a significantly increased risk of developing a separate primary SCC.
34.
Answer:
117. False
118. True
119. False
120. True
121. True (non caseating)
35.
Answer:
122. False
123. False
124. False
125. False
A Shatzki ring is a constant lower esophageal ring which is due to mucosal thickening or may be secondary to scarring from reflux.. It occurs at the level of the B ring.
Muscular rings of the esophagus are transient rings related to contraction.
A ring = contracted/hypertrophied muscles in response to an incompetent gastroesophageal sphincter
B ring = sling fibers representing U-shaped thickening of inner muscular layers
On a good double contrast exam, the squamocolumnar junction (which is 1-2 cm above the gastric sling fibers) can be seen. It is also called the Z-line
The best technique to detect a muscular ring is a prone single contrast examination.
36.
Answer:
126. True
127. False
128. True
129. True
130. True
- 80% of juvenile polyps are in the rectosigmoid.
- Malignant potential:
< 5 mm --------> 0%
5-9 mm --------> 1%
10-20 mm -----> 10%
> 20 mm -------> 50%
15mm=15% rule
37.
Answer:
131. True
132. True
133. False
134.true
135. True
The small bowel valvulae probably cannot be completely effaced as we count the valvulae/inch when a small bowel enema is done. Jejunum loops can't be effaced but ileum can be.
38.
Answer:
136. True
137. false
138. True
139. True
140. True
Focal nodular hyperplasia is twice as common in females. They can bleed with rupture causing hemoperitoneum. The incidence rises to 14% if the patient is on oral contraceptives. (Is 0-14% common?). Focal nodular hyperplasia is the only tumor that contains Kupffer cells. It is multifocal in 20%. On non-enhanced CT, focal nodular hyperplasia has slightly decreased attenuation - this changes to isodense to hyperdense with contrast injection. Fewer than 1/3 of cases present because of clinical Sx , most are incidentally found.
39.
Answer:
145. False
146. False
147. True
148. False
Esophageal varices arise from the dilated left gastric (coronary) veins and drain into systemic veins of the thorax (hemiazygous, azygous, intercostal). The IMV can shunt blood from the splenic vein to the hemorrhoidal veins in the pelvis.
Collaterals in portal vein hypertension include:
- portal v-> coronary/l gastric, short gastric, vertebral plexus, IVC, hemiazygous
-coronary v->azygous, hemiazygous, vertebral
-IMV->inf and middle hemorrhoidal. v.
40.
Answer:
149. True
150. False
151. True
152. True
153. True
Following discussion is about nontropical sprue. (= celiac disease)
The folds are not thickened in uncomplicated celiac disease. Fold thickening can be seen with malabsorption-related hypoalbuminemia. The Moulage (wax) pattern is seen in 50% - it is a smooth contour with effaced pattern less folds.
Have stunted villi in celiac disease. The moulage sign represents flocculated barium seen in celiac disease. Luminal fluid excess can be due to hypersecretion and is non-specific for the disease. There is increased separation or apparent abscence of folds in the jejunum. ? Can get increased number of folds in the ileum referred to reversal of folds between the ileum and jejunum. Can get painless transient intussusceptions.
41.
Answer:
141. False
142. False
143. False
144. True
Lymphogranuloma venereum is more common in males. It is commonly symptomatic with rectosigmoid narrowing, deep ulcers, and fistulae. It is caused by Chlamydia trachomatis, which is not a retrovirus. Treatment with tetracycline in the acute phase is successful.
Gonococcal proctitis has a similar radiographic appearance.
The clinical spectrum ranges from the asymptomatic carrier state when a primary painless vesicle forms and resolves . The disease then evolves. to inguinal syndrome from lymphadenopathy bloody diarrhea, rectal pain, tenesmus. If left untreated, deep ulceration, stricturing, rectovaginal fistulas may occur.
42.
Answer:
70. False
71. True
72. False
73. True
Glucagon is naturally made by the alpha cells in the islets of Langerhans. Glucagon increases blood glucose and gastric acid release, increases hepatic ketogenesis, and increases lysis of adipose. It acts by binding to cell membrane receptors; it is a smooth muscle spasmolytic: The gallbladder and papilla of Vater relax with injection of glucagon
0.1 mg - stomach and duodenal hypotonia
0.25 mg - small bowel hypotonia
1.0 mg - colonic hypotonia
Glucagon is contraindicated:
Absolute:
1) in patients who have allergy or hypersensitivity to it
2) in patients with pheochromocytoma
3) in patients with insulinoma (precipitous glucose drop)
Relative:
4) in brittle diabetics
Anticholinergics (Buscopan, Hyoscine butylbromide) are contraindicated in patients with glaucoma. They tend to decrease gastric secretions.
43.
Answer:
154. True
155. probably True
156. True
157. False
158. True
ITP has thickened folds secondary to hemorrhage.
SLE probably has thickened folds secondary to hemorrhage due to vasculitis.
Scleroderma doesn't have thickened folds. Get Hidebound bowel, The valvulae are bunched together because of the fibrosis.
44.
Answer:
159. False
160. True
161. False
162. False
163. False
The differential diagnosis for aphthous ulcers includes:
1) Small bowel
a) Yesinia (with nodular mucosal pattern in the terminal ileum)
b) salmonella (produces an endotoxin which irritates the mucosa of the stomach, small bowel, and colon - findings are nonspecific but may resemble those of ulcerative colitis on barium examination.)
c) amebic
d) shigella
e) Crohn’s
f) Behcet’s
g) lymphoma
h) herpes/CMV
??(ankylosing spondylitis, TB, Rickettsiosis)
2) Colon
a) Crohn’s disease
b) amebiasis
c) Yesinia
d) Behcet’s
e) lymphoma
f) ischemia
Aphthous ulcers are shallow ulcers.
45.
Answer:
164. True
165. True
166. False
167. True
168. False
46.
Answer:
169. False
170. False
171. False
172. False
173. True
Lymphoma is first a submucosal process (submucosal nodules). Other presentations include: enlarged folds, polypoid masses, or stricture. The most common presentation is a polypoid mass or an infiltrating stricture indistinguishable from carcinoma. (There is no serosal layer.)
Lymphoma of the esophagus accounts for 1-2% of all cases of lymphoma of the GI tract. (Stomach is the most common site, followed by small bowel.) It is more commonly of the non-Hodgkin’s variety. The typical appearance is distal narrowing due to adjacent spread from the gastric fundus. There can also be direct invasion of the esophagus from mediastinal lymph nodes, or extrinsic compression by mediastinal lymph nodes. Usually NHL and less commonly Hodgkins.
Gastric lymphoma is much more common. Non-Hodgkin lymphoma accounts for the majority of gastric lymphoma and radiographically occurs in four forms: infiltrative, ulcerating, polypoid, and endoexophytic. Hodgkin disease typically produces a desmoplastic reaction, resembling scirrhous carcinoma.
47.
Answer:
174. True
175. True
176. True
177. True
178. True
48.
Answer:
179. True
180. True
181. False
182. False
Neutropenic colitis ("typhlitis") (inflammed cecum) most commonly involves the cecum and terminal ileum. CT findings include: wall thickening (occasionally with low attenuation intramural areas of edema or necrosis), pneumotosis, pericolonic fluid, and thickening of the fascial planes. Untreated can lead to transmural necrosis and perforation.
In pseudomembranous colitis, the inflammation is less pronounced than in typhlitis. Clostridium difficile is seen in pseudomembranous colitis.
49.
Answer:
183. True
184. False
185. False
186. False
Herpes esophagitis is often seen in the immunocompromised patient. CMV and HIV esophagitis are also not uncommon in an immunocompromised patient. All are commonly symptomatic. All most commonly involve the mid-esophagus.
"Candidiasis is by far the most common cause of infectious esophagitis,"
50.
Answer:
187. True
188. False
Glycogenic acanthosis (epithelial hyperplasia) is seen in the mid or distal esophagus. It is a benign disease which is degenerative and age-related. There is increasing frequency with increasing age. In the elderly it is seen in up to 30% of contrast esophagrams.
Acanthosis nigricans is thickening and discoloration of the skin and papillomatosis from idiopathic causes. 60% of cases in adults are associated with gastric adenocarcinoma. It is also associated with lymphoma, Cushing’s syndrome, acromegaly, and Stein-Leventhal syndrome.
51.
Answer:
189. true
190. True
191. prob true-- but can have other etiologies, but is most likely secondary to portal HTN
Gastric varices have been found in all parts of the stomach, although the majority are found in the fundus and proximal body. They cause thickened mucosal folds which vary in size and shape. The presence of gastric varices without esophageal varices usually indicates splenic vein occlusion, which is most commonly caused by pancreatitis or pancreatic carcinoma. CT may be more sensitive than conventional radiologic examination in dx gastric varices because it can delineate not only the luminal varices, but also the deeper intramural and perigastric varices.
When there is splenic vein obstruction, the flow reverses to fill the short gastrics which then drain into the coronary v which drains into the portal v. Splenic v thrombosis may be intrinsic or more commonly extrinsic. Extrinsic compression from chronic pancreatitis, pancreatic pseudocysts, pancreatic. ca., peripancreatic lymphoma, mets, retroperitoneal fibrosis/bleeding. Intrinsic thrombosis may result from polycythemia or myeloproliferative, or idiopathic. In the presence of isolated gastric varices, without esophageal varices, portal HTN is so much more common than splenic v obstruction that most pt with isolated gastric varices are from portal HTN. If necessary, CT or angio can be performed to document the pathophysiology.
"Uphill" varices form when there is increased flow through the coronary veins, usually the result of portal hypertension (most commonly due to alcoholic cirrhosis in the U.S.), with blood flow toward the azygous vein. Therefore, they predominate in the lower half of the esophagus. Normally, the esophageal plexus drains into the coronary v, then to the splenic v. In portal HTN, the esophageal plexus drains into the azygous/hemiazygous and then into the SVC.
"Downhill" varices develop when the superior vena cava is obstructed below the entrance of the azygous vein. The collateral vessels are most developed in the upper half of the esophagus. Mediastinal tumor or inflammatory disease is usually the cause.
52.
Answer:
192 True
193. True
194. True
195. True
Hepatocellular carcinoma can have a variable appearance on ultrasound. Most lesions <5 cm are hypoechoic - larger tumors can be hyperechoic secondary to fibrosis and necrosis. Eighty percent of hepatocellular carcinomas occur in patients with preexisting chronic liver disease such as cirrhosis, hemochromatosis, or alpha-1-antitrypsin deficiency. It occurs in about 10% of patients with chronic active hepatitis, which is most commonly due to hepatitis B. A strong association exists between dietary aflatoxin and hepatocellular carcinoma in southeast Asia and Africa, and it is a well-known complication of Schistosoma japonicum, which is endemic in the Far East. Also the fat within HCC can make the lesion hyperechoic.
53.
Answer:
196. True
197. True
198. False
There is a strong male predominance in post-Billroth carcinoma, perhaps as high as 36:1. Pt who undergo partial gastrectomy are at increased risk for developing gastric ca. Usually BII affected more than BI. The mortality form gastric ca 15yrs or more post gastrectomy is 3-7X greater than general population.
54.
Answer:
199. True
200. True
201. True
202. false
GI duplications are most common in the terminal ileum. Pertechnetate can be useful since some of the duplications contain gastric mucosa.
Enteric duplications are usually on the mesenteric side. The majority of duplication cysts are spherical duplications and have no direct communication with the lumen. They are most frequently in the ileum. The rare tubular duplication lumen may be in communication with the normal bowel at both ends, or at only one end. Occasionally there is no communication. Ectopic gastric mucosal lining can be useful in the dx. 99m-Tc has been reported to have 86% sensitivity.
55.
Answer:
203. true
204. true
205. false
206. false
207. false
60-75% of cases of colonic volvulus involve the sigmoid. In S. America and in Africa volvulus is secondary to dilated and elongated sigmoid from a high fiber diet. In US, usually is in elderly males and residents of nursing homes secondary to chronic constipation and obtundation (bloating) from medication.
Therapy usually consists of emergency sigmoidoscopy and rectal tube placement which is successful in 90%. Recurrence is 50%. Contrast low pressure barium enema may occasionally be required. to demonstrate sigmoid volvulus. F/U barium exam is recommended to evaluate for underlying carcinoma.
Pt present with abdominal pain and distension from obstruction. Obstipation and vomiting are also symptoms. Sx are usually acute but can be chronic.
56.
Answer:
208. false ( more a focal stricture rather than diffuse)
209. true
210. true
DDX for smooth esophageal narrowing includes: congenital stenosis, web, surgical repair of esophageal atresia, caustic burns, gastric acid-reflux, intubation- reflux, compromise of circulation, XRT of mediastinum, post infection - rare. Lower esophageal narrowing includes: scleroderma, achalasia, diffuse spasm, esophagitis
Classic findings of Barrett's include: high esophageal stricture/ulcer, HH, GER. Strictures are at proximal zone of columnar metaplasia. Can also have strictures in distal esophagus.--peptic symptoms. Reticular mucosal pattern is also a finding..
DDX for high esophageal strictures include caustic ingestion, mediastinal XRT, primary or mets, rarely esophageal. involvement by dermatologic disorders such as epidermolysis bullosa dystrophica and benign mucous membrane pemphigoid.
GI Section
Select the single best answer:
57.
Answer: b
58.
Answer: c
CC malignant die from malabsorption. PJ malignant 2-3%GI, 13% breast
59.
Answer: b
60.
Answer: b
61.
Answer: a
62.
Answer: a
63.
Answer: b
64.
Answer:???
65.
Answer: b. leiomyoma
66.
Answer: c
most likely answer for 40y.o man. Etoh cirrhosis is the most common in this age group. Pancreatic calcification.
Advanced gastric ca are seen on plain films as a soft tissue mass indenting on the gastric shadow. Rarely, mucin-producing scirrhous ca contain gross areas of calcification that have a stippled , punctate appearance. Peak incidence of ca is 50-70 y.o. but 3-5% of pt with ca are younger than 35y.o. Pt are only symptoms when have advanced disease. 25,000 new cases per year.
Gastric mets are found in less than 2% of pt with ca. Mets are most commonly melanoma or breast. Can get spread from colon ca along the gastrocolic ligament; so, findings are along the greater curve. Omental mets may spread superiorly to the stomach via the proximal greater omentum aka the gastrocolic ligament.
Leiomyoma are usually in pt over 50y.o. Can be found in the stomach, antrum, body, or fundus. On plain film, rarely, leiomyomas can contain irregular streaks or clumps of calcifications.. The larger the mass, the more epigastric pain and bleeding. Rarely they grow larger than 5cm. Most often their discovery is incidental. Benign gastric tumors (polyps, leiomyomas) have been present in 5% and up to 25% of autopsies.
Gastric varices can be recognized on plain film as a lobulated mass. Depending on the etiology, pancreatic calcification can be seen. usually secondary to patients with portal hypertension secondary to cirrhosis or liver disease. Cirrhosis is one of the 10 leading causes of death. Frequency is 15 per 100,000. In the US, it is the third leading cause of death for men 34-50 y.o.
67.
Answer: a. dysplastic fingernails
68.
Answer: Crohns,. e
ZE has grossly thickened edematous duodenal and jejunal folds. 75% of ulcers are located in the stomach or duodenal bulb. 25% of ulcers are located in the post bulbar duodenum or jejunum.
Duodenal involvement by Crohns is usually assoc with antral involvement. Findings include aphthous ulcers, one or more larger ulcers, thickened nodular folds, cobblestoned appearance. Can eventually get fibrosis, narrowing, sacculations. Can get fistulization. Strictures are usually in the post bulbar duodenum.
Celiac sprue secondary to increased immunoglobulin production secondary to gluten ingestion. The villi are absent in sprue. Lamina is thickened. Changes in the duodenum included decreased number of folds, nodular changes rarely.
Duodenal carcinoids can be seen as polypoid, submucosal masses, or ulcerated lesions.
Giardia is dx with duodenal biopsy or aspirate. Radiographic findings are non specific including irregular fold thickening, hypermotility, luminal narrowing.
69.
Answer: c
95% of bile is resorbed in the ileum. Bile is resorbed by active transport process through the intestinal mucosa in the distal ileum.
70.
Answer: c
Success of initial intubation depends on location--lesions distal to esophagus are more difficult to intubate and get balloon for dilatation to. Proximal esophageal strictures tend to be more difficult to dilate than distal strictures. Once successful intubation and dilatation have been achieved, location does not have a major effect on long term outcome. Malignant strictures are less likely to respond than benign. Patients with anastomotic strictures are reportedly more difficult to intubate. Epidermolysis usually diffusely involves the esophagus. According to S Rubesin, epidermolysis bullosa shouldn't be dilated because there is a high rate of perforation due to the friable mucosa and the diffuse involvement.
71.
Answer: b
Common causes for pneumobilia include post-op, e.g. sphincterotomy or Whipple pancreaticoduodenectomy, biliary enteric fistula, pancreatitis, emphysematous cholecystitis. Most common causes of biliary enteric fistula include gallstone, post-op, uncommon causes include Peptic Ulcer Disease perforation into biliary tract, trauma.
Mesenteric infarction causes portal venous air.
72.
Answer:
a. true
b. true
c. ??true b/c decreased drainage into the hepatic veins --> decreased intrahepatic IVC pressure.
d. true
US chief role in management of cirrhosis is dx of portal HTN. Doppler identifies flow direction.
Stage II cirrhosis on angio is characterized by corkscrew appearance of hepatic artery branches. Portal flow is bidirectional. Stage III has wide spread corkscrewing of arteries and increased arterial flow.
Stage II, wedged hepatic venography, Stage IIA show opacification of portal v for 2-4s but eventual drainage into hepatic v. With Stage IIB, opacification of main or segmental portal v for more than 4s. Normal liver shows drainage into hepatic vein. Wedge hepatic venography gives sinusoidal pressure.
Collateral pathways lead to the IVC. ? increased blood flow to IVC--? increased pressure
73.
Answer: c. trauma
This question has also been asked as "What is the most common cause of fluid collection in the anterior pararenal space in a child who has had blunt trauma?"--answer : pancreatitis
On the other hand, in a child, following blunt trauma, the most common gastrointestinal injury involves the duodenum.
74.
Answer: b
Complications of ERCP.
Pancreatitis 1%
cholangitis 0.8
drug side effects 0.6
pancreatic abscess 0.3
GI injury 0.2
mortality 0.2
75.
Answer: a. hyperplastic
Hyperplastic polyps represent 75-90% of all gastric polyps. They are not premalignant, but they are associated with atrophic gastritis and 8-28% of patients with hyperplastic polyps have synchronous gastric carcinomas. They are usually smooth, sessile, round, and 5-10 mm in size. In addition, they are usually clustered in the fundus and body (i.e., multiple). The differential diagnosis includes leiomyomas, adenomatous polyps, and polyposis syndromes.
Adenomatous polyps (villous, tubular, tubulovillous) account for <20% of all gastric polyps. They are dysplastic lesions which may undergo malignant degeneration. 30-40% of patients with adenomatous polyps have a synchronous gastric carcinoma.
76.
Answer: b. chondroma
They are probably referring to Carney’s syndrome. This condition (24 reported cases, M:F=1:11) includes:
1) gastric leiomyosarcoma
2) functioning extraadrenal paraganglioma
3) pulmonary chondromas
77.
Answer: c. cecum
78.
Answer: e rectum
79.
Answer: b. posterior lower midline pharyngeal diverticulum
Zenker diverticula originate in the midline of the posterior wall of the hypopharynx at an anatomic weak point known as Killian’s dehiscence =posterior hypopharyngeal diverticulum. It is an acquired mucosal herniation. At this location, immediately above the cricopharyngeus, there is a divergence between the fibers of this muscle and the inferior pharyngeal constrictor which is superior to the cricopharyngeus. During swallowing, increased intraluminal pressure forces mucosa to herniate through. The etiology of Zenker diverticula is not firmly established, but premature contraction or motor incoordination of the cricopharyngeus muscle is thought to play a major role. Their development presumably takes time, however, as they have not been reported in children. Carcinoma arises in 0.3% of pt with Zenker's
- relationship between Zenker's and reflux is controversial
- dysphagia, regurgitation of undigested food, halitosis
- found in elderly
80.
Answer: c. contraction of the muscularis mucosa
Contraction of the muscularis mucosa gives transient 1-2 mm horizontal folds. This is often associated with reflux. Feline esophagus alone does not indicate esophagitis.
Secondary contractions of the esophagus are due to thoracic esophageal distention.
81.
Answer:
a. True
b. False
c. False
d. False
e. False
Hemangiomas classically are echogenic with posterior acoustic enhancement. They should not have demonstrable flow on Doppler; they are more common on the right; they are well defined. They occasionally can get a prominent central vein. Hemangiomas are hyperechoic mass with discrete margins, with possible acoustic enhancement. Color doppler demonstrates filling vessels in the periphery of the tumor but not significant doppler flow deep within the hemangioma
- most common benign liver tumor (4% incidence at autopsy), generally asymptomatic and solitary
- calcifications uncommon
- US - well circumscribed, round or oval, uniformly echogenic
- CT - precontrast hypodense, post contrast early peripheral enhancement with centripetal filling and complete filling on delayed images
- MRI - low in T1, high on T2, light bulb lesions
- Tc99m RBC - early photopenic, late iso- to hyperintense
82.
Answer: a
Regenerating nodules can occasionally be appreciated on US and are hypoechoic.
Colon carcinomas are usually hypoechoic on US. If the colon ca is mucinous, can get calcifications.
Lymphoma of the bowel is usually hypoechoic. mural thickening. Lymphoma is usually hypo or anechoic but echogenicity is variable.
Breast ca is hypoechoic to the surrounding tissues. Kopans.
adenoma--adrenal- solid mass. can have calcification
hepatic- can be hyper, hypo, iso or mixed echogenicity.
parathyroid- hypoechoic relative to thyroid
Tumors that calcify include mucinous ca of the colon or pancreas, bronchogenic ca, and breast ca. Mucinous adenocarcinoma of the colon is the most common primary neoplasm associated with calcified liver lesions
83.
Answer: b. pancreatitis
84.
Answer: e. target sign or pseudokidney sign in the right lower quadrant
The sonographic hallmark of acute appendicitis is the recognition of abnormal appendix. The abnormal appendix is noncompressible tubular structure which in the transverse plane has a "target" configuration.
85.
Answer: e. hepatic venoocclusive disease
Many gastrointestinal complications have been reported after transplantation. These include peptic ulcer disease; massive hemorrhage; colon perforation (in association with diverticulitis, CMV infection, or idiopathic); fecal impaction, sometimes leading to obstruction, ileus, or even colonic necrosis; or pancreatitis.
Specifically, for bone marrow transplantation, the following intestinal complications may occur:
1. The induction protocol of high-dose radiotherapy and/or chemotherapy causes mucositis with orophayngeal and abdominal pain, vomiting, and diarrhea. It may last up to 3 weeks.
2. Impairment of the host’s immune status leads to opportunistic bacterial, fungal, viral, and parasitic infection.
3. Acute GVHD develops.
Post bone marrow transplant pt are probably neutropenic and thrombocytopenic; therefore , are unlikely to be hypercoagulable and develop Budd Chiari.
86.
Answer: a. is pathognomonic for gastric carcinoma
No sign in gastrointestinal radiology has generated more confusion or disagreement than the meniscus sign of a malignant ulcer, which was originally described by Carman in 1921 and refined by Kirkland in 1934. The Carman-Kirkland meniscus complex is caused by a cancer straddling the lesser curvature of the gastric antrum or body in which the tumor is broad. Manipulation and compression of the lesion at fluoroscopy may result in the demonstration of a discrete ulcer crater that has a meniscoid configuration. The convex inner border of the meniscus may be quite irregular and is almost always directed toward the gastric lumen, whereas the concave outer border of the meniscus, representing the base of the broad, shallow ulcer, tends to be smoother and usually does not project beyond the expected gastric contour. A radiolucent halo may be seen adjacent to the meniscus as a result of apposition of the elevated edges of the tumor on the anterior and posterior walls. Although the Carman-Kirkland meniscus complex is a reliable radiologic sign of malignancy, it can be demonstrated in only a small percentage of all malignant ulcers of the stomach.
87.
Answer: c. lungs
Clinical manifestations of scleroderma include skin fibrosis and Raynaud’s (peripheral vasospasm). These are the most common and earliest manifestations.
The esophagus is eventually involved in approximately 90% (hypomotility in 50%). The smooth muscle in the distal half to two-thirds of the esophagus is replaced by fibrosis, resulting in diminished esophageal motility. Because the lower esophageal sphincter tone is severely decreased, gastroesophageal reflux commonly occurs, often resulting in peptic esophagitis and stricture formation.
Less common abnormalities are: renal failure, synovitis, basilar interstitial lung disease, and cardiac conduction abnormalities.
88.
Answer: b. adenocarcinoma
Cholangiocarcinoma exists in three distinct forms: as an infiltrative, stricturing process; a bulky exophytic tumor; or a polypoid intraluminal mass. On CT, tumor mass at the level of biliary obstruction is seen in approximately 70% of patients. Klatskin tumor refers to a cholangiocarcinoma occurring at the junction of the right and left hepatic ducts. Except for the rare anaplastic neoplasm, almost all cholangiocarcinomas are adenocarcinomas arising from bile duct epithelium
89.
Answer: a
Tumors that calcify include mucinous ca of the colon or pancreas, bronchogenic ca, and breast ca. Mucinous adenocarcinoma of the colon is the most common primary neoplasm associated with calcified liver lesions
90.
Answer: a. left gastric artery
Mallory Weiss tear
- relatively common
- increased intraesophageal pressure - linear mucosal laceration (longitudinal in 77%)near gastric cardia (at/above/below gastroesophageal junction in 76%) resulting in painless hematemesis
- accounts for 5-10% of all acute upper GI bleeding, usually seen in male alcoholics 30-60 y/o
- most heal spontaneously 48-72 hours
- excellent prognosis
91.
Answer: a. cystic duct
92.
Answer: b. the fundus and c. the body of the stomach
93.
Answer: b. blind loop syndrome
- segment of small bowel that has been bypassed by an enteroanastomosis
- get stagnation of bowel contents
- get malabsorption of Vitamin B12 (?megaloblastic anemia), diarrhea, anemia, abdominal pain
- can be created from anatomic variants
94.
Answer: a. sprue
Celiac disease (nontropical sprue)
- the classic malabsorptive disease
- associated with HLA DR3
- increased production of IgA, IgM antibodies
- gluten intolerance
- steatorrhea, abdominal distention, glossitis, changes in pigmentation, neuropathies
- villi are absent or stunted, thickened lamina
- reversal of jejunal and ileal patterns, duodenum looks like hell ("The jejunum looks like the ileum, the ileum looks like the jejunum and the duodenum looks like hell," Jim Buck)
- transient intussusception sometimes occurs
- nodules
- small bowel has poor peristalsis and becomes flaccid and dilates, especially proximally
- hypersecretion and mucosal atrophy in the jejunum cause dilution and the "moulage" pattern seen on barium examination
- associated with small bowel lymphoma, small bowel carcinoma
- Treatment - gluten restriction or antibiotics
Whipple’s
- rare multisystem disease, probably bacterial
- PAS + macrophages
- predilection for involvement of SI joint, heart valves, CNS, joint capsule
- middle-aged men, USA, Northern Europe
- diarrhea, steatorrhea
- immune defects
- diffuse micronodules 1-2 mm primarily in jejunum
- associated with hypoalbuminemia
- no dilatation or increased secretions
- nodal masses in mesentery, echogenic
- may have sacroiliitis
- differential diagnosis - MAI, lymphangiectasia
- Treatment - Antibiotics
Waldenstrom's macroglobulinemia
- associated with monoclonal IgM (plasma cell dyscrasia)
- lymph nodes, hepatosplenomegaly, anemia
- bleeding from mucous membrane
- associated with lymphoma or lymphatic obstruction
- intestinal involvement is rare - see tiny nodules ("sand like") in small bowel secondary to villi becoming greatly distended
Mastocytosis
- accumulation of mast cells, primarily in skin
- increase output of gastric acid
- diarrhea, malabsorption
- duodenal ulcers and duodenitis
- nodular thickened folds, nodules can be large
- look for bone sclerosis
Scleroderma
- F: M > 3:1
- 3-5th decade
- associated with CREST (Calcinosis of skin, Raynaud's phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia)
- smooth muscle involvement in GI tract in 50%
- Esophagus > small bowel > colon > stomach involvement
- Esophagitis:
incompetent LES, decreased peristalsis, increased reflux, dilated esophagus, patulous gastroesophageal junction
- small bowel atony, dilatation, malabsorption
- pseudoobstruction
- pneumatoses
- hide bound appearance, may mimic transient intussusception
- no increase in fluid
- diverticula (sacculations)
- malabsorption- hypoproteinemia - thickening of valvulae
- fibrosis at lung bases
95.
Answer: b. MAI
Cryptosporidium get dilatation and fluid
Cryptococcus usually spares the small bowel, but can get fold thickening and fluid
MAI (MAC)
- small bowel is most common site of involvement in gut
- similar to Whipple’s
- diffuse nodules
- irregular, thickened folds
- small bowel diffusely involved
- lymph nodes - may have low attenuation centers (like TB)
- hepatosplenomegaly, increased LFT's
Cryptosporidiosis
- 10% of cases of diarrhea in AIDS
- severe and debilitating diarrhea
- dilution of barium compatible with sprue
- fold thickening in duodenum and jejunum
CMV
CMV usually spares the small bowel
- esophageal ulcers, gastritis, isolated intestinal ulcers
- terminal ileitis, spontaneous intestinal perforation, focal or diffuse colitis
- CMV esophagitis, large ulcerations
- CMV gastritis
- small bowel is usually spared
- CMV colitis - diffuse nodular lymphoid hyperplasia
Herpes
- primarily esophagitis:
- scattered diamond or stellate discrete ulcers on a background of normal mucosa
- can see proctitis
96.
Answer: b. extrinsic to the circular muscle
97.
Answer: c. mastocytosis
- bone osteopenia ---> sclerosis
- nausea, vomiting, tarry stools
- urticaria pigmentosa
- alcohol intolerance
- tachycardia, flushing, headache, asthma (all secondary to histamine release)
- associated with peptic ulcer disease
98.
Answer: b. left lateral decubitus
Pneumoperitoneum is best detected with left lateral decubitus (left side down leading to air lateral (actually superior) to the liver or supine cross table lateral (leading to air on the underside of the anterior abdominal wall).
99.
Answer: a. lesser omentum
The lesser sac is also known as the "omental bursa." The lesser omentum slings the lesser curvature of the stomach to the undersurface of the liver. The lesser sac lies behind the lesser omentum, stomach, and more inferiorly, the greater omentum. The lesser sac is anterior to the transverse colon and the transverse mesocolon.
100.
Answer: b. histiocytosis
The different manifestations of histiocytosis:
A) Letterer-Siwe Disease
1) weeks to 2 years old
2) hemorrhage, anemia, fever, FTT, hepatosplenomegaly, lymphadenopathy
3) bones affected in 50% -widespread lytic lesions. ("raindrop" pattern in calvarium)
4) 70% mortality
B) Hand-Schuller-Christian Disease
1) triad: exophthalmos, diabetes insipidus, lytic skull lesions
2) 5-10 year olds
3) lytic lesions in bone, floating teeth
4) blebs in the lungs, pneumothorax in 25%
5) nodular infiltrate may progress to fibrosis and end-stage lung
6) prognosis: spontaneous remissions and exacerbations
C) Eosinophilic Granuloma
1) peak age is 5-10 years-old
2) lytic bone lesions which are monostotic in 50-75%
3) skull has a beveled-edge with "button sequestrum"
4) spine - vertebra plana
5) lung - upper lobe reticulonodular pattern which can progress to honeycomb appearance
6) recurrent pneumothoraces in 25%
7) prognosis: excellent
101.
Answer: c. postbulbar ulcer
In annular pancreas there is stenosis in the periampullary region with extrinsic defect on the medial margin of the 2nd portion of the duodenum.
A duodenal diaphragm (web) occurs in the 2nd portion of the duodenum near the ampulla of Vater. A thin radiolucent line extends across the duodenal lumen. There is proximal duodenal dilatation.
Duodenal diverticulum is an acquired lesion, usually at the medial border of the 2nd portion of the duodenum. (can be in the 3rd or 4th portion, however)
A postbulbar ulcer is usually located on the medial wall of the proximal descending duodenum above the ampulla of Vater. It can be difficult to demonstrate secondary to severe spasm. Edema and spasm often result in a smooth rounded indentation on the lateral wall of the duodenum opposing the ulcer.
102.
Answer: a. esophageal achalasia
Achalasia is due to decreased number of ganglion cells in Auerbach’s plexus. The etiology is unknown. 7% develop esophageal carcinoma. There is esophageal dilatation, stasis of esophageal contents, absence of primary peristalsis, and incomplete or absent lower esophageal sphincter relaxation. The distal esophagus is smooth, tapered, and beak-like. Incomplete emptying of the esophagus occurs, even when the patient is upright. There is slowly progressive dysphagia during the middle decades. Odynophagia and chest pain are uncommon. Most patients are between 20-40 y/o.
103.
Answer: b. most of the gastrin-producing cells are removed
Both the Billroth-I and Billroth-II operations removed the distal 2/3-3/4 of the stomach. Gastrin-producing cells are in the antrum, so all should be removed. Acid-producing (parietal) cells are located in the fundus and body, so only a portion are removed. The goal is to "excise the entire gastrin mechanism as well as a large portion of the parietal cell mass."
104.
Answer: b. secretin
Trypsinogen is secreted by the pancreas.
Secretin is the most potent stimulant for water and bicarbonate secretion.
Enterokinase is secreted by the duodenum and converts trypsinogen into trypsin.
Cholecystokinin is the most potent stimulant for protease secretion by the pancreas.
Gastrin is produced by G cells in the antrum of the stomach - it causes the parietal cells to secrete acid.
105.
Answer: d. hyperplastic polyp (present in at least 40%)
106.
Answer: b
Mastocytosis is characterized by the accumulation of mast cells. These pt have increased local and systemic release of histamine. There is increased output of gastric acid associated with duodenal ulceration and duodenitis. Bone manifestation is secondary to mast cell infiltration leading to fibroblastic activity and granulomatous rx. Can get osteopenia and bone destruction or osteosclerosis which involves mostly the axial skeleton
Multiple osteomas of the mandible, calvarium, or tubular bones can accompany Gardner's. This closely resembles familial polyposis coli. These pt have a 100-200 x increased risk of developing periampullary duodenal ca. Most pt are asymptomatic .
GI Section
The following are matching answers:
107.
1 c
2
3
108.
1 c
2 a
3 b
4 d
109.
Answer:
1. c
2. d ,e
3. b ,e
4. a
5. ??e--2,3,5 are all hamartomatous
(Gardner’s syndrome matched with c because a, b, d, and e don’t match and I guess keratin xanthoma fits with Jim Buck’s comment, "Other subcutaneous -omas also occur." (with FAPS))
Polyposis Syndromes
1. Adenomatous Polyposis Syndrome
- There is mounting evidence that these (familial polyposis coli, Gardner’s syndrome, and Turcot’s syndrome) are all varying expressions of the same disease - familial adenomatous polyposis syndrome (FAPS). Shared traits:
- relatively rare
- M=F
- autosomal dominant with occasional spontaneous mutations
- 80 -100% penetrance
- responsible gene probably on long arm of chromosome 5 (other genes for colorectal cancer are located here)
- adenomas in the colon are usually tubular or tubulovillous (100 or more adenomas are required for diagnosis)
- usually small (80% <5 mm and sessile)
- most common symptoms are rectal bleeding and diarrhea (because colonic disease predominates.)
- polyps don’t usually develop until puberty, so screening is delayed until patient is 10 y/o.
- untreated patients will develop cancer (and at a much younger age than the general population)
- rectal mucosa must also be removed during total colectomy since there is a high risk of developing cancer in the residual rectal tissue.
- all portions of the GI tract with columnar epithelium may develop adenomas (esophagus has stratified squamous epithelium, so not usually affected.)
- gastric involvement in >50% (fundic (hamartomatous) and adenomatous polyps!)
fundic gland polyps more common in Western countries
fundic polyps gastric adenomas
- not with chronic atrophic gastritis - tubular and villous
- 1-5 mm - sessile, 5-10 mm
- multiple, sessile - >50% multiple
- not premalignant - premalignant (low risk)
- antrum spared (monitor, don’t cut!) -
- duodenum is 2nd most common site of FAPS
In Japan, 90% have tubular adenomas!! (47-72% Western countries)
- duodenum-periampullary region most frequent site for adenocarcinoma
outside of the colon (4% within 5 years S/P colectomy)
- polyps are usually in 2nd portion of duodenum, around the papilla (medial!)
- villous adenomas common, usually periampullary (large, premalignant)
- polyps are also seen in the small bowel, pancreas, and biliary tract
Extraintestinal Manifestations (let the fun begin!)
- skin lesions
- epidermoid cysts frequent
- tend to on face and scalp but can be anywhere
- often appear before GI adenomas!
- lipomas and small fibrous tumors of the skin less common
- bone lesions
- osteomas
- benign, but may be an early marker for FAPS
- angle of mandible, sinuses, outer table of skull ? "screening" Panorex?)
- localized or diffuse cortical thickening of long bones
- dental abnormalities (impacted or supernumerary teeth)
- fibrous lesions
- increased incidence of postoperative peritoneal adhesions
- retroperitoneal fibrosis has been reported
- more commonly, fibroid tumors, especially desmoid tumors of the abdominal wall and mesenteric fibromatosis, occur
- usually seen postoperatively, commonly in women of childbearing age (may grow rapidly and may first appear during pregnancy)
- mets do not occur
- eye lesions
- congenital pigmented lesions of the retina (often bilateral)
- may occur before development of colonic polyposis
- MALIGNANT TUMORS OF THE CNS
- commonly referred to as Turcot’s syndrome (2 cases in 1959!)
- polyps may be less numerous and larger and carcinoma may develop at a younger age
- GBMs/ gliomas most common (most supratentorial), also medulloblastoma
- associated stigmata of FAPS often not seen (can have cafe au lait spots and focal nodular hyperplasia of liver)
- Thyroid carcinoma
- prevalence of thyroid carcinoma is 160X that of general population
- usually papillary, usually multifocal
- usually detected before colonic disease - so consider FAPS in all cases of young female with papillary cancer of thyroid.
2. Hamartomatous Polyposis Syndromes
- Hamartomas of the Cronkhite-Canada, multiple hamartoma (Cowden’s), and juvenile polyposis syndromes have similar histologic features. The Peutz-Jeghers hamartoma has a distinctive morphologic appearance.
- Mucocutaneous and extraintestinal manifestations are common.
Peutz-Jeghers Syndrome
- usually in clusters, carpeting usually doesn’t occur
- treatment is usually polypectomy of troublesome/large polyps
- inherited in autosomal dominant pattern, M=F (40%-50% sporadic, however)
- mucocutaneous pigmentation (95%)
- small brown or bluish-black macules on lips (fades with age) and buccal (doesn’t fade with age) mucosa (less on dorsal fingers, palms, and soles)
- pigment rarely present at birth
- extra-alimentary tract neoplasms (50% - 18X risk of general population, especially pancreatic)
- ovarian sex cord tumor with annular tubules (benign) present in almost all females
- polyps from the stomach to the rectum (95% have small bowel involvement)
- Sertoli cell tumor which produces isosexual precocity unique to Peutz-Jeghers syndrome
- adenoma malignum - well-differentiated adenocarcinoma with aggressive behavior (cervix)
- bilateral breast carcinoma (secondary to estrogen stimulation by ovarian tumors?)
- increased prevalence of GI tract neoplasms (2-16% prevalence of GI tract malignancies)
- most in gastroduodenal area or colon
- polyp is an abnormal mixture of smooth muscle and mucosa (pseudoinvasive appearance)
- can cause recurrent intussusception
- according to Buck's AFIP notes, the polyps rarely occur outside the GI tract.
Cowden’s Syndrome (Multiple Hamartoma Syndrome)
- multiple hamartomas and neoplasms of ectodermal, mesodermal, and endodermal origin
- autosomal dominant with high penetrance
- some features overlap with MEN IIb (mucosal neuroma syndrome - pheochromocytoma, medullary thyroid carcinoma, neuromas (similar to neurofibromatosis))
- mucocutaneous lesions
- occur in all patients and are the hallmark of the disease
- earliest findings (at 20-30 years of age)
- facial papules, oral mucosal papillomatoses, acral keratoses most common
- less characteristic are keratoses located on the dorsum of the hands/ feet/ proximal extremities
- thyroid abnormalities
- most frequent extracutaneous abnormality
- usually goiters and adenomas
- follicular adenocarcinoma in 3-12% (all women)
- breast abnormalities
- 70% have lesions (but 50% was "fibrocystic disease"!!!!)
- cancer prevalence of 30-50%, tends to be bilateral, 30-40 y/o at diagnosis
- GI abnormalities
- multiple nonadenomatous polyps (small and sessile) occur in >35%
- little or no malignant potential
- segmental distribution, most common in rectosigmoid
- usually asymptomatic
- esophageal involvement rare but possible
Cronkhite-Canada Syndrome
- inflammatory polyps in the stomach and colon have no malignant potential
- generalized polyposis syndrome with characteristic ectodermal changes
- occurs sporadically, affected patients are usually around 60 y/o
- innumerable polyps in the stomach, small bowel, and colon in almost all patients
- in the stomach, usually superimposed on thickened rugal folds (may resemble Menetrier’s but the antrum is also involved)
- polyps are usually small and sessile, identical in appearance to the polyps in juvenile polyposis
- GI symptoms followed by abnormalities of the skin (multiple brown macules or confluent areas of hyperpigmentation - hands and feet), hair (abrupt alopecia), and nails (dystrophic changes, possibly loss)
- if patient dies from the disease, it is usually from malnutrition (surgery is usually ineffective, so patients are supportively managed, sometimes with sustained remissions - which may include disappearance of polyps!!)
- 15% of cases progress to malignancy (adenocarcinoma)
Juvenile Polyposis
- two main categories:
- isolated juvenile polyps of childhood (but 50% have more than one polyp)
- usually no family history
- low risk of malignant potential
- most common tumor of the colon in childhood (1% of kids 4-14 y/o)
- painless rectal bleeding most common
- sessile, 2-50 mm diameter
- juvenile polyposis of the colon or entire GI tract (six or more polyps in the colon or rectum or polyps throughout the entire GI tract or family history)
- average age is 32 y/o
- areas of dysplasia in the polyps (therefore, increased risk of cancer)
- 25% of cases are inherited in autosomal dominant fashion
- nonfamilial cases have 25% incidence of congenital abnormalities (hydrocephalus and pulmonary AVMs most common)
- juvenile polyposis of infancy is a subcategory (Infantile Cronkhite-Canada)
- severe diarrhea before 2 y/o
- polyps unevenly distributed throughout GI tract with colon and small bowel most severely involved
- polyps usually 2-3 mm, larger ones are pedunculated
Ruvalcaba-Myhere-Smith Syndrome
- something that I know nothing about but it was mentioned at AFIP (rare, autosomal dominant, pigmented genital lesions, macrocephaly, generalized GI hamartomas, no reported malignancy)
110.
6. d
7. a
8. a
9.a
10.c
Inspissated meconium may obstruct the colon causing the mucous plug syndrome. Far fewer of these fetuses have CF than with meconium ileus.
Meconium ileus is obstruction and dilation of ileum from impaction of abnormal sticky and thick meconium of fetuses with cystic fibrosis.
Meconium peritonitis can be caused by volvulus, jejunum or ileal atresia or meconium ileus. Secondary to any GI perforation in utero. Results in dystrophic calcification.
Meconium Plug Syndrome is a form of neonatal intestinal obstruction secondary to failure of passage of meconium from the colon. Meconium plug is a result of colonic inertia rather than a cause of intestinal obstruction. Frequently is a presenting symptoms of Hirschsprung disease. It is rarely due to CF. Show signs of obstruction 2-3 day of life.
Small left colon is a functional disorder. Has a definite association with maternal diabetes. Vomiting, abd distension, and failure to pass meconium are symptoms in the neonatal period. Colon is dilated to the splenic flexure. Meconium is frequently within the left colon and distended transverse colon.
Meconium Aspiration is caused by aspiration of meconium stained fluid. happens 10% of deliveries but ,1% incidence of meconium asp. syndrome.
Persistent Fetal Circulation may be due to many reasons e.g. diaphragmatic hernia, meconium aspiration, unknown etiology. When severe, there is significant r-l shunting and hypoxia and acidosis.
111.
Answer:
11. a
12. b
13. c
14. d
15. e
16. f
Emphysematous cholecystitis:
- clostridium perfringens
- clostridium welchii
- E. coli
- Staph.
- Strep.
Pseudomembranous colitis:
- C. difficile
MAI (MAC) is most likely to cause pseudo-Whipple’s disease which is a disseminated mycobacterial infection with an increased number of mesenteric and retroperitoneal lymph nodes. The lymph nodes are Periodic Schiff stain positive (like Whipple’s disease) but, unlike Whipple’s, the lymph nodes are also acid fast positive. In Whipple’s disease, the macrophages are filled with PAS and glycogen and gram-positive rods are seen - this disease responds to antibiotics.
Strep Bovis bacteremia have a high incidence of colorectal tumors.
112.
Answer:
17. c
18. d
19. e
113.
Answer:
20. c
21. a
22 d
Calcitonin is a biochemical marker for familial medullary carcinoma of the thyroid. MEN 2A consists of pheochromocytoma, medullary thyroid ca, parathyroid hyperplasia. 2B has pheochromocytoma and medullary ca but pt also have dysmorphic features.
Carcinoid tumors excrete multiple monoamines and peptides such as histamine, catecholamines, bradykinins, thachykinins, enkephalins, endorphins, vasopressin, gastrin, adrenocoricotrophin prostaglandins.
Clinical features of VIPoma include diarrhea, hypokalemia, hypochlohydria.
114.
Answer:
23. a
24. a
115.
Answers:
25. b
26. d and f
27. e
28. c
29. a
30. g
The esophagus is involved in 90% of cases of scleroderma. The proximal one third (striated muscle) remains normal, but the distal 2/3 (smooth muscle) is involved. There is a patulous LES with abnormal motility. In the small bowel, there can be atony and subsequent dilation (pseudoobstruction) and possible malabsorption. The small bowel and colon can get sacculations. "Hidebound" describes normal thickness folds with straightened bowel margins.
Crohns disease is characterized by transmural inflammation, fistulae, fissures, and linear ulcers. It is also associated with post-inflammatory polyps, or pseudopolyps.
Zollinger-Ellison syndrome occurs secondary to a gastrinoma which is most often a non-beta islet cell tumor in the pancreas. Most are malignant with metastases to the liver. It presents with severe (multiple and recurrent) peptic ulcer disease. 90% have ulcers and postbulbar ulcers are particularly suspicious. Gastric folds are markedly thickened (especially in the body and the fundus) from the high level of acid secretion. 50% of patients have diarrhea. Hypersecretion of acid leads to a large fluid volume which dilutes barium
Lymphoid hyperplasia is usually found in the ileum (and jejunum). Peyer’s patches are seen in the antimesenteric border and are oriented longitudinally.
Intramural hemorrhage gives the stack of coins appearance secondary to submucosal hemoglobin or edema. Other potential causes of the stack of coins appearance are: trauma, ischemia, XRT, bleeding diathesis, venous obstruction, hypoproteinemia, etc.
116.
Answers:
31. b
32. b
33. a
34. b
35. a and b
Typhlitis represents acute cecal inflammation in immunosuppressed patients (especially AML, ALL, AIDS, renal transplants).
Regional enteritis (Crohns disease) gives fissuring, perirectal and pericolonic abscess, pseudopolyps, etc.
117.
Answer:
36. a
37. c
Impaction of a gallstone in the cystic duct or neck of the gallbladder may cause common hepatic duct stenosis or obstruction. This is known as Mirizzi syndrome. PTC shows stenosis and displacement of the common hepatic duct. Compression usually occurs from the right side of the duct. Gallbladder carcinoma may produce similar defects in the CHD. In most cases of Mirizzi syndrome, there is an anomalous insertion of the cystic duct allowing a parallel arrangement of the cystic duct adjacent to the common hepatic duct.
Hydrops of the gallbladder can be caused by (among others):
1. Kawasaki’s disease (mucocutaneous lymph node syndrome)
2. leptospirosis
3. prolonged hyperalimentation
4. sepsis
118.
Answer:
38. b
39. c
40. a
Twining’s recess is seen in hypertrophic pyloric stenosis. It is associated with Diamond’s sign which is a transient tent-like cleft in the channel.
Valves of Houston are 3 transverse folds in the rectum.
Columns of Morgagni are vertically oriented folds in the anus. Superior to these columns is the pectinate line-- the junction between columnar and squamous mucosa.
119.
Answer:
41. c
42. b
43. a
44. a
45. a
Cystic pancreatic tumors may be classified into two broad categories -- microcystic adenoma (serous) and mucinous cystadenoma (macrocystic). Both are found more commonly in women, but only the microcystic adenomas are associated with von Hippel-Lindau syndrome. The microcystic type is more likely to occur in the head of the pancreas whereas the mucinous type is more commonly found in the body and tail.
The microcystic adenoma has no malignant potential. It is characterized by numerous small cysts, usually under 2 cm, but may have several larger cysts. When the cysts are numerous and very small, the mass may appear echogenic, due to the high number of tissue interfaces. It is usually vascular, and may demonstrate a central stellate scar, with or without calcification.
The mucinous cystadenoma, on the other hand, does possess a malignant potential and may develop into a cystadenocarcinoma. The cysts which characterize this lesion are usually large, and the tumor is typically hypo- or avascular.