PANCREATIC DISEASES IN CHILDREN

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CONGENITAL ABNORMALITIES OF THE EXOCRINE PANCREAS

Congenital abnormalities of the pancreas are uncommon and usually asymptomatic. Most congenital anomalies of the pancreas are related to the two critical events during morphologic development, rotation and fusion.56, 67, 101 A brief review of the embryology of the pancreas will help in the understanding of the origin and pathogenesis of the congenital abnormalities.

The pancreas first appears in the fourth-week embryo as two outpouches originating from the primitive foregut just distal to the

APLASIA, HYPOPLASIA, AND DYSPLASIA

Complete agenesis of the pancreas is rare and probably incompatible with life. In partial agenesis, the pancreas is defective in size and shape, usually involving an abnormal development of the dorsal bud. Although in hypoplasia the pancreas is normal in size and shape, the normal epithelial cells are replaced by fatty tissue, and the duct system is reduced in number of ducts and their terminal differentiation. Dysplasia involves disorganized parenchyma, duct dilatation, and fibromuscular

DUCTAL ANOMALIES

The normal ductal architecture of main pancreatic duct of Wirsung and accessory duct of Santorini exists in 60% to 70% of individuals.126 In the rest of the population, numerous variations exist.56 Pancreas divisum is implicated as a possible important contributing factor to acute pancreatitis in patients of all ages. In addition, the association of choledochal cyst, pancreatic duct changes, and pancreatitis is presented.

Pancreas Divisum

Pancreas divisum results from incomplete fusion of the dorsal and ventral pancreatic ductal systems and their respective pancreatic tissues. In this anatomic aberration, the major part of the pancreas is drained by the accessory papilla, which may be too small to drain the secretions.77 The associated anatomic or functional stenosis, at the accessory papilla, may account for the increased incidence of acute or recurrent pancreatitis in these patients who lack an alternate pancreatic outflow.

HETEROTOPIC PANCREAS

Heterotopic pancreas is synonymous to ectopic aberrant or accessory pancreas. It is defined as pancreatic tissue lacking anatomic and vascular continuity with the main body of the pancreas.56 Heterotopic pancreas is found in 0.55% to 15% of autopsies in different localizations. In 70% of instances the ectopic pancreas is found in the upper gastrointestinal tract; antrum 25% within 5 cm of the pylorus; duodenum 30%; and jejunum 15%.101 0dominal organs such as colon appendixi ileum, rectum,

ANNULAR PANCREAS

Annular pancreas consists of a flat band of pancreatic tissue encircling the second portion of the duodenum. The incidence is not known, but the Mayo Clinic revealed 15 cases observed during a 20-year period.65 Pathogenically, three main theories are favored, including56(1) hypertrophy of both the ventral and dorsal anlagen, resulting in complete constriction around the duodenum; (2) persistence and enlargement of the left bud of the paired ventral primordium; and (3) fixation of the tip of the

PANCREATIC INSUFFICIENCY

Several hereditary disorders of the exocrine pancreas, which have been described as causes for pancreatic insufficiency, maldigestion, and steatorrhea, are as follows:

  • Cystic fibrosis

  • Schwachman-Diamond syndrome

  • Johanson-Blizzard syndrome

  • Exocrine pancreatic dysfunction with refractory sideroblastic anemia

  • Pancreatic aplasia/hypoplasia

  • Isolated exocrine pancreatic enzyme deficiencies

  • Lipase

  • Lipase-colipase

  • Colipase

  • Amylase

  • Trypsinogen

Cystic fibrosis is, by far, the most common. The ensuing exocrine

Nutritional Management

The failure to thrive encountered in pancreatic insufficiency is the result of a combination of inadequate caloric intake, maldigestion, malabsorption, abnormal nutrient losses, and increased requirements. Because children with pancreatic insufficiency do not grow until the percentage of energy absorption exceeds 100% to 110% that of the recommended daily allowance (RDA), the current recommended caloric intake is 120% to 150% of RDA.73, 90 Although low-fat diets were previously used,

ACUTE PANCREATITIS IN CHILDREN

Acute pancreatitis is not a common cause of abdominal pain and represents a diagnostic challenge in the pediatric age group. It has numerous causes, an obscure pathogenesis, few effective remedies, and a sometimes unpredictable outcome.130 In contrast with adults, trauma, drugs, and viral infections are the three leading causes in children. The most frequently used diagnostic tools are serum and urinary amylase, abdominal ultrasonography, and CT. Lately, ERCP is more frequently used in selected

HEREDITARY PANCREATITIS

Hereditary pancreatitis (HP) is the second most common hereditary disease of the pancreas. It is a form of chronic pancreatitis presenting in childhood but often unrecognized until years later. It represents the second most common cause of chronic or recurrent pancreatitis in childhood, the first being juvenile tropical pancreatitis.

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    Address reprint requests to Aaron Lerner, MD, Pediatric Gastroenterology and Nutrition Unit, Department of Pediatrics, Carmel Medical Center, 7 Michal Street, Haifa 34362, Israel

    *

    From the Pediatric Gastroenterology and Nutrition Unit, Department of Pediatrics, Carmel Medical Center, B. Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa (AL); the Department of Pediatrics, Shaare Zedek Medical Center, Hebrew University Medical Center (DB); and the Department of Pediatrics, Mount Scopus, Hadassah-Hebrew University Hospital, Jerusalem, Israel (EL)

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    Copyright © 1996 W. B. Saunders Company. Published by Elsevier Inc. All rights reserved.