Annular pancreas

Pancreatic Disorders in Infants and Children

Pancreatic disorders in infants and children encompass a wide variety of congenital and acquired conditions. Although rare in occurrence, pancreatic disease in children is generally serious and often life-threatening, making prompt diagnosis and treatment mandatory. These disorders may be complex, often necessitating extensive diagnostic evaluation, multiple operations and protracted hospitalization. In some cases, prompt surgical management is the only definitive treatment. The rarity of pancreatic disorders requiring operation in childhood, combined with the complexity of the illness and frequent complications, make treatment both unique and challenging to the physician.

Synn and associates reviewed the medical records of 79 children aged 18 years or younger with pancreatic disease. These patients were seen during a 20-year period. The spectrum of illness changed with the age of the patient; patients with congenital anomalies presented at a median age of one week and those with acquired disorders presented at a median age of 10 years. Thirty of the children were treated medically and 49 underwent a total of 60 surgical procedures.

Forty-eight of the patients had pancreatitis or its complications, 17 had congenital malformations, 12 had hypoglycemia with hyperinsulinism and two had pancreatic carcinoma. The mortality rate for children with pancreatitis was 17 percent and was limited to the 30 patients who were not treated surgically. The predominant types of pancreatitis were idiopathic (16 children) and drug-induced (12 children). All of the 12 children with drug-induced pancreatitis had received corticosteroids. Only rarely should these patients undergo surgery. The postoperative results were uniformly good in patients who underwent operations for various traumatic lesions of the pancreas or for complications of pancreatitis.

The most common congenital malformation of the pancreas was an annular pancreas in association with duodenal atresia; all of the 13 children with this abnormality were successfully treated with bypass procedures. The remaining four patients with congenital abnormalities had an ectopic pancreas and underwent successful wedge resection.

Hyperinsulinism was associated with nesidioblastosis or islet cell hyperplasia in nine infants and with islet cell adenomas in three patients. All of these 12 children were managed unsuccessfully with medical treatment. They subsequently underwent successful resection. No deaths occurred among the nine children with nesidioblastosis or islet cell hyperplasia, although neurologic sequelae from prolonged pre-operative hypoglycemia were common.

Two patients underwent radical resection for pancreatic carcinoma, one of whom has survived 20 years postoperatively. (American Journal of Surgery, September 1988, vol. 156, p. 201.)

COPYRIGHT 1989 American Academy of Family Physicians
COPYRIGHT 2004 Gale Group