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Common bile duct obstruction related to intestinal polyposis in a child with Peutz-Jeghers syndrome
A T Gentile1, S W Bickler, M W Harrison
1Department of Surgery, School of Medicine, Oregon Health Sciences University, Portland.
Insights
Peutz-Jeghers syndrome can cause duodenal polyps leading to complications like bowel obstruction. This case highlights how these polyps can distort anatomy and cause biliary obstruction, requiring surgical intervention.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Genetics
Background:
- Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder.
- PJS is characterized by hamartomatous polyposis in the gastrointestinal tract and mucocutaneous pigmentation.
- Gastrointestinal manifestations include intussusception, obstruction, and bleeding.
Observation:
- A 9-year-old girl with PJS presented with small bowel obstruction due to duodenal intussusception.
- Polyps in the fourth portion of the duodenum caused the intussusception.
- Postoperative complications included biliary obstruction, necessitating further surgical intervention.
Findings:
- Duodenal polyposis in PJS can lead to significant anatomical distortion.
- Ampullary obstruction occurred secondary to duodenal polyposis.
- Pancreatitis and portal fibrosis were noted in this patient.
Implications:
- Early recognition and management of duodenal polyps in PJS are crucial.
- Surgical intervention may be required to address complications like intussusception and ampullary obstruction.
- PJS requires long-term monitoring for gastrointestinal and extra-intestinal manifestations.
Abstract:
Peutz-Jeghers syndrome is characterized by hamartomatous polyposis of the small and large bowel and mucocutaneous pigmentation. The authors describe a 9-year-old girl with small bowel obstruction related to duodenal intussusception caused by polyposis in the fourth portion of the duodenum. Operative reduction of the intussusception and excision of the polyps were performed, at which time the pancreas appeared to have mild pancreatitis. A liver biopsy specimen showed mild portal fibrosis and ductal proliferation. The patient did well postoperatively, but later presented with symptoms consistent with biliary obstruction. Percutaneous transhepatic cholangiography showed pancreatic and biliary duct dilatation as well as obstruction of the common bile duct, which extended into the left upper quadrant. Exploration showed ampullary obstruction several centimeters proximal to the line of resection. Sphincteroplasty was performed. The postoperative course was uncomplicated. The authors conclude that Peutz-Jeghers syndrome with polyps in the duodenum can markedly distort duodenal and ductal anatomy and can lead to ampullary obstruction.