Synchronous presentation of acute pancreatitis and splenomegaly with intussusceptions in Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome (PJS) is a rare, autosomal dominant inherited disorder characterized by gastrointestinal hamartomatous polyps and pigmented mucocutaneous lesions. Intussusceptions and gastrointestinal tract bleeding are frequent complications of PJS caused by larger polyps, but acute pancreatitis and splenomegaly are very rare. Previously, surgical intervention was the only curative modality despite the associated complications. In recent years, double-balloon enteroscopy (DBE) has become the alternative method as the ideal treatment. We report the case of a 20-year-old woman diagnosed with PJS, as she suffered from 4-day nausea and vomiting with acute abdominal pain, and clinically manifested acute pancreatitis, splenomegaly and duodenojejunal intussusceptions secondary to a huge proximal jejunal polyp (10 cm × 2 cm). We removed the polyp via DBE and found that the acute pancreatitis, splenomegaly and duodenojejunal intussusceptions were simultaneously ameliorated 2 days after treatment. Currently, the patient has been symptom free for 3 months after polypectomy. Acute pancreatitis, splenomegaly and intussusceptions can synchronously occur in PJS patients, and polypectomy via DBE is the effective treatment alternative to surgical intervention for this disease, thereby obviating some complications associated with the latter.