Familial chronic recurrent pancreatitis in identical twins. Case report and review of the literature
E. Freud, R. Barak, N. Ziv, A. Leiser, G. Dinari, C. Mor and M. Zer
Department of Pediatric Surgery, Beilinson Medical Center, Petah-Tiqva, Israel.
Familial presentation of chronic recurrent pancreatitis in childhood is
rare. The etiology of this illness is obscure, and its hereditary
properties are not well defined. Simultaneous occurrence of chronic
recurrent pancreatitis in identical twins with the same clinical
presentation and similar typical pancreatographic abnormalities is
exceptional. Twin sisters, aged 9 years, were admitted to the hospital
because of recurrent attacks of pancreatitis. Ultrasound examination
revealed an enlarged irregular pancreatic duct in both girls, and
endoscopic retrograde cholangiopancreatography showed a distorted duct with
multiple strictures and dilatations similar to a "chain of lakes" pattern.
Both patients underwent longitudinal pancreatojejunostomy within a month.
The therapeutic regimen and preoperative and surgical treatment of such
patients are discussed, as is the optimal timing of intervention.