DIGESTIVE-TRACT DISORDERS ASSOCIATED WITH ASPLENIA POLYSPLENIA SYNDROME/

The authors reviewed experience gained over a 20-year period of asplen ia or polysplenia syndrome, focusing on patients with associated diges tive tract disorders (DTDs). Eleven of 27 patients (40%) with asplenia /polyspenia had associated DTDs The DTDs comprised malrotation of the intestine in nine, both preduodenal portal vein and gastric volvulus i n three, esophageal hiatal hernia in two, and biliary atresia in one. Laparotomy was carried out on four patients with symptoms of acute bow el obstruction and on one patient with biliary atresia. One patient wi th both malrotation and gastric volvulus, and another with only associ ated malrotation survived. Nine patients died, eight of cardiac insuff iciency and one because of hepatic insufficiency. When infants are dia gnosed with heterotaxia, they should be examined for other combined DT Ds, because they may have a chance for Survival if they undergo surger y when their condition is still stable. Copyright (C) 1997 by W.B. Sau nders Company