A Case of Recurrent Multiple Jejunal and Ileal Intussusceptions in Peutz-Jeghers Syndrome: A Radio-Pathologic Correlation
Anna Marie G. Pineda, RMT, MD
University of Santo Tomas Hospital, Department of Radiological Sciences, Manila, Philippines
To present a comprehensive multi-modality radio-pathologic investigation of the first case of recurrent multiple-site jejunal and ileal intussusceptions in a case of Peutz-Jeghers Syndrome.
A 17 year old male, Filipino, with a family history of familial adenomatous Polyposis and Peutz-Jeghers Syndrome (PJS), was managed in our institution due to recurrent abdominal pain, vomiting and melena. Physical findings include direct tenderness with a palpable mass in the abdomen, pallor, hyperpigmentation of the lips, buccal mucosa with sparing of the tongue, and distal digits (Fig.1).
Non contrast whole abdominal CT scan revealed multiple iso- to slightly hyperdense polyps within the stomach, small and large intestines. Multiple fluid-filled jejunal and proximal ileal segments were appreciated exhibiting “target sign”appearances suggestive of double-site intussusceptions (Fig.2A).Emergency exploratory laparotomy with manual reduction, jejunotomy and polypectomy were done.
Recurrence of symptoms after five years prompted repeat contrast-enhanced whole abdominal CT scan, which again revealed polyps and at least four-site ileal and jejunal intussusceptions (Fig.2B).
Age-specific surveillance for multiple-organ involvement was done to search for PJS-associated carcinoma. Upper GI series with follow-through small intestinal series revealed sessile and pedunculated polyps in the stomach and small intestines with an area of jejunal narrowing (Fig.3). Ultrasound showed polyps in the gastrointestinal tract with blood flow in the pedicles (Fig.4)and, non-specific testicular microlithiases (Fig.5).Endoscopy with polypectomy were done revealing light to dark brown bosselated and rubbery polyps (size range- 0.3 to 1.3 centimeters) (Fig.6).Histologically, one specimen is an adenoma and the rest are hamartoma exhibiting the “arborizing” pattern consistent with PJS. (Fig.7)