Congenital and Genetic Abnormalities

Pancreas Division (Dominal Dorsal Duct Syndrome): – This problem occurs in up to 10% of the whole population. It results from dorsal and ventral pancreas to unite. Consequently most of the pancreas is drained through accessory papilla that is proximal to papilla of Vater. These types of patients are more prone to pancreatitis for condition appears to be associated with stenosis of opening of one or other papilla.

Diagnosis in made via endoscopic retrograde cholangiopancreatography (ERCP).

Annular Pancreas: – Ventral pancreas surrounds the second part of duodenum and it is frequently associated with other congenital defects such as mal-rotation of intestine, artesian and cardiac defects. Pancreas may constrict duodenum causing obstruction soon after the birth or in adult life. This may predispose to duodenal ulcer and acute pancreatitis. Diagnosis is made by barium studies and treatment is surgical bypass of constriction.

Ectopic Pancreatic Tissue: – It may occur in gastric antrum or duodenum and it takes the form of smooth nodule. It is normally asymptomatic and it does not require any treatment. If it is found coincidentally during surgery, the nodule is excised for it is subject to same conditions including carcinoma, as the pancreas itself.

Pathology: – The pathological findings and clinical features result from obstruction by abnormal viscid secretion of the ducts in salivary glands, digestive and biliary tracts and pancreas causing atrophy

Clinical Features: – The exocrine insufficiency leading to steatorrhoea and diarrhea is almost universal in childhood. If patient survives to adulthood, malabsortion tends to be less troublesome. The intestinal obstruction, commonly called “meconium ileus equivalent”, may occur in the children and in the adults, caused by inspissated food and secretions. Rectal prolapse is frequent in children because of the large bulky stools and frequent coughing. The recurrent episodes of the acute pancreatitis may also occur. The incidence of the diabetes mellitus increases with the age. Chronic pancreatitis may supervene. The disease of liver or biliary tract may occur.

Investigation: – The gastrointestinal problems are investigated according to suspected problems. There may be disorder in the gut epithelium and pancreas. 80% of the patients with cystic fibrosis have both pulmonary disease and pancreatic disease. But 15% patients have lung sepsis with clearly normal pancreatic function.

Management: – Optimal treatment in adolescent and adult depends on a team approach to the complicated respiratory, nutritional and hepatobiliary problems. Many treatment centers have established special clinics for such kind of patients with specialist care.

The patients with cystic fibrosis need 120-150% of recommended daily energy allowance because of malabsorption and catabolism induced by acute and chronic respiratory infections. Nutritional counseling and supervision are very important. Fat restriction is not advised because this limits caloric and fatty acid intake. The supplements of the fat-soluble vitamins are necessary.

All the patients having pancreatic insufficiency need oral pancreatic enzyme supplements – “Creon” or “Neutrizym” 1-2 capsules with the meals. The dose needs to be increased to 30 capsules a day. It is titrated to reduce steatorrhoea to the point at which the stool frequency and offensiveness are reduced. When partial or incomplete intestinal obstruction requires surgery, administration of N-acetylcyteine both orally and rectally may relieve some of the obstructions

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