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

Area of Science:

• Gastroenterology
• Surgical Oncology
• Pediatric Surgery

Background:

• Peutz-Jeghers syndrome (PJS) is a hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract.
• Gastrointestinal complications such as intussusception, obstruction, and bleeding are common initial manifestations, particularly in pediatric patients.
• Current surgical management focuses on addressing acute complications and prophylactic polyp removal to prevent recurrence.

Observation:

• A 15-year-old female with a history of PJS presented with acute abdominal pain, progressing to peritonitis.
• Initial endoscopic evaluations (esophagogastroduodenoscopy and colonoscopy) were negative.
• Despite a previously considered laparoscopic approach, an emergency open laparotomy was performed due to the patient's clinical deterioration and PJS history.

Findings:

• The laparotomy revealed a significant jejunoileal intussusception causing ischemic bowel damage.
• A 130 cm segment of the ileum was resected, followed by an end-to-end ileo-ileal anastomosis.
• Post-resection, careful manual palpation and transillumination of the remaining bowel confirmed the absence of further polyps.

Implications:

• Open laparotomy offers a superior opportunity for thorough intraoperative exploration and polyp eradication in pediatric PJS patients with acute abdominal emergencies compared to laparoscopy.
• This approach may reduce the need for subsequent emergency surgeries by addressing the underlying polyposis comprehensively.
• The case highlights the critical importance of considering open laparotomy in young patients with suspected or confirmed PJS presenting with acute abdominal symptoms.