Multidisciplinary Management of Surgical Disorders of the Pancreas in Childhood

Abstract

Surgery for pancreatic disorders in children is rarely required but may be necessary a) for definitive management of primary pancreatic pathology, b) to treat sequelae of acute or chronic pancreatitis and c) to treat an underlying cause of pancreatitis. There is a broad spectrum of potential pathologies. These patients are best managed by a multidisciplinary team approach.

Pancreatitis in Children

“…10 Acute recurrent pancreatitis can occur in up to 15% to 36% of children who have acute pancreatitis. 22,23 A pseudocyst, one of the more common complications of acute pancreatitis and acute recurrent pancreatitis, is defined as "a collection of amylase-rich pancreatic fluid that lacks an epithelial lining" 19(p417) and includes nonnecrotic tissue. A more heterogeneous collection of fluid with necrotic tissue present is identified as postnecrotic collection or necrotizing pancreatitis.…”

Childhood Pancreatitis

“…Gene sequencing of the 7q35 chromosome region revealed a strong association of the (p.R122 H) mutation of the PRSS1 gene encoding cationic trypsinogen with hereditary pancreatitis (Teich et al, 2006). Developments in genetics have shown that in the majority of patients, hereditary pancreatitis is usually associated with expression of one of two specific mutations in the cationic trypsinogen PRSS1 gene, specifically R122H or N29I.…”

“…In vitro the mutations increase autocatalytic conversion of trypsinogen to active trypsin and thus probably cause premature intrapancreatic trypsinogen activation in vivo. The clinical presentation is highly variable, but most affected mutation carriers have relatively mild disease (Teich et al, 2006).…”

“…The genetic mutations responsible for hereditary pancreatitis have been isolated to chromosome 7q35. The most common hereditary pancreatitis causing mutations in PRSS1 gene are R122H and N29I (Pfutzeret al, 2002;Teich et al, 2006;Nydegger et al, 2006). These two mutations in the cationic trypsinogen gene appeared to allow prematurely activated trypsinogen to cause acinar cell auto-digestion and acute pancreatitis.…”

“…Non-operative management of low grade pancreatic injury is widely accepted. Management of major pancreatic parenchymal or ductal injury in children, however, remains controversial (Haddock et al, 1994;Stringer et al, 2005). Initial nonoperative management of injuries of the proximal pancreatic duct is a common practice and allows for the formation and uneventful delayed drainage of a pseudocyst, rather than the risks of early radical interventions (Jobst et al, 1999).…”