Q71) Duplication of the intestine associated with
A. Heterotopic mucosa
B. Smooth muscle component
C. Associated with spinal / vertebral defects
D. All are correct
Q 72) Choledochocele is type
Q73) . WDHA syndrome is associated with
Q74) DVT, Depression, Dermatitis, Diarrhoea seen in
Q 75) Which of the following is a type of Familial Adenomatous Polyposis
A. Gardner syndrome
C. Juvenille polyps
D. Hamartomatous polyp
Q 76) Perineal procedure in rectal prolapse
C. Resection rectopexy
D. Frykman Goldberg procedure
Q77 ) Intussusception in children
A. Mc type is Ileocolic
B. Mc type is colo colic
C. Entering tube is intususcepiens
D. Contrast enema is not useful
Q78 ) A type of Selective shunt for portal hypertension is
A. PSRS (Proximal spleno renal shunt)
B. Warren shunt
C. SSPCS (Side to side portocaval shunt)
D. Mesocaval shunt
Q 79) Hereditary diffuse Gastric carcinoma is associated with which breast cancer
A. Ductal carcinoma NOS subtype
B. Lobular carcinoma
D. Metaplastic carcinoma
Q 80. Malignant hydatid disease is caused by
A. E. Granulosus
B. E. oligartus
C. E. vogeli
D. E. multilocularis
Duplications of alimentary tract are rare and most are on mesenteric side. Normally they are lined by alimentary tract mucosa but can have heterotopic mucosa.
They can also be associated with spinal anomalies. They present in early childhood with obstruction, hemorrhage, intussusception or perforation. They can even go on to become malignant (especially rectal)
Excision is the treatment
Ref BAiley page
2. Type II and III CC are associated with an extremely low risk of malignant transformation. Diverticulectomy of type II CC followed by primary CBD closure at the diverticulum neck.
Small choledochoceles- endoscopic sphincterotomy.
Transduodenal excision for large choledochoceles with associated complications such as gastric outlet obstruction or pancreatitis
3. Type V (Caroli's disease) management consists of liver resection or orthotopic liver transplant
VIP (Vasoactive Intestinal Peptide) is found in brain, Antral G cells, pancreatic neurons and D cells in pancreas.
Components of WDHA are watery diarrhea, hypokalemia, Achlorhydria
Glucagonoma is characterised with Diabetes, Dermatitis, DVT, Depression. Also associated is weight loss and vitamin deficiency.
Skin lesions- Necrolytic migrating erythema, appear before other symptoms and are often misdiagnosed. These are because of amino acid deficiency and go away after Iv amino acids
Ref Sabiston 957
FAP has phenotype variations. This means there can be many extraintestinal manifestations.
Gardner syndrome- FAP + Osteoma skull + Desmoid mesentery + Periampullary tumors
Associated with FAP are also thyroid papillary tumors, medulloblastomas (turcot) , hypertrophic gastric fundic polyps, and congenital hypertrophy of the retinal pigmented epithelium of the iris (CHRPE)
2. PJS (Peutz Zeghers Syndrome) - Small polyps through out the intestine especially small intestine associated with pigmented lesions of skin and genital tumors, This entity is dift from FAP and has increased risk of pancreatic and
3. Juvenile polyps-
Delorme - Perineal procedure in which there is rectal mucosectomy with muscular plication
Frykman Goldberg - Transabdominal posterior rectopexy with resection of the redundant left colon .
Ripstein - Trans abdominal proctopexy
Resection rectopexy- Trans abbdominal
Thierech- Perineal wire plasty
Altmeier- Perineal Proctosigmoidectomy and levatoroplasty
Intussusception is classified according to the site of the intussusceptum and intussuscipiens. In children, more than 80% are ileocolic, beginning several centimetres proximal to the ileocaecal valve with their apex found in the ascending or transverse colon.
Classically, a previously healthy infant presents with colicky pain and vomiting (milk then bile). Between episodes,
the child initially appears well. Later, they may pass a ‘redcurrant jelly’ stool
Selective shunts only decompress gastroesophageal varices and maintain portal hypertension and flow to the liver
Warren shunt or DSRS is a selective shunt which decompresses esophageal and gastric varices by connecting the splenic vein into the left renal vein. With portal azygous disconnection by ligation of the left and right gastric veins, and interruption of the gastroepiploic arcade, portal hypertension is preserved. Key elements in this procedure are mobilization of the splenic flexure of the colon, mobilization of the pancreas from the superior mesenteric vessels to the hilum of the spleen, adequate dissection of the splenic vein to prevent distortion when it is brought down for anastomosis to the left renal vein.
Germline CDH1 mutations confer a high lifetime risk of developing diffuse gastric (DGC) and lobular breast cancer (LBC)
The updated guidelines include revised CDH1 testing criteria (taking into account first-degree and second-degree relatives):
(1) families with two or more patients with gastric cancer at any age, one confirmed DGC;
(2) individuals with DGC before the age of 40 and
(3) families with diagnoses of both DGC and LBC (one diagnosis before the age of 50).
Additionally, CDH1 testing could be considered in patients with bilateral or familial LBC before the age of 50, patients with DGC and cleft lip/palate, and those with precursor lesions for signet ring cell carcinoma. Given the high mortality associated with invasive disease, prophylactic total gastrectomy at a centre of expertise is advised for individuals with pathogenic CDH1 mutations. Breast cancer surveillance with annual breast MRI starting at age 30 for women with a CDH1 mutation is recommended.