CASE REPORT   

Minerva Chirurgica 2003 February;58(1):101-4

Copyright © 2003 EDIZIONI MINERVA MEDICA

language: English

Acute abdomen in a 15-year-old patient with Peutz-Jeghers syndrome. Surgical approach

Caronna R., Catinelli S., Di Martino M., Prezioso G., Scozzafava S., Sassayannis P. G., Sinibaldi G., Chirletti P.

The natural history of Peutz-Jeghers syndrome (PJS) is characterized by gastrointestinal complications (occlusion, invagination or bleeding), often the first clinical manifestation in young patients. Surgical treatment consists of treating the complication, exploring the bowel and cleaning out all polyps to prevent further emergency operations at brief intervals. For this purpose both the laparotomic and laparoscopic approaches have been proposed, especially in young patients. A 15-year-old girl was admitted for investigation of colicky abdominal pains. When she was 5 years old, PJS was diagnosed. On admission to our department, the patient underwent emergency esophagogastroduodenoscopy and colonoscopy, both negative. At 24 hours after admission peritonitis developed. Given her clinical history, we rejected the laparoscopic approach proposed at admission and decided for an open laparotomy. Laparotomy disclosed a long jejunoileal invagination that caused irreversible ischemic damage of the bowel. We resected about 130 cm of the ileum and did an end-to-end ileo-ileal anastomosis. Meticulous palpation and transillumination of the residual bowel identified no other polyps. In young patients with acute abdomen and with proven or suspected PJS instead of laparoscopy, open laparotomy is a unique occasion to explore the residual bowel thoroughly, manually and, if possible, endoscopically.