Acute intussusception and polyp with malignant transformation in Peutz-Jeghers syndrome: A case report

Abstract

Abstract. Intussusception is one of the most frequent complications of Peutz-Jeghers syndrome, and has been well described in previous studies. More attention has been paid to malignancy, which is another complication of Peutz-Jeghers syndrome and which leads to increased mortality. Few cases of intussusception combined with malignant polyps in Peutz-Jeghers syndrome have been reported to date. In the present study, we report a case of intussusception and malignant polyps occurring in various parts of the small intestine in a 43-year-old male. In addition to repair of the intussusception and partial resection of the small intestine with malignant polyps, we also simultaneously performed polypectomy of as many polyps as possible without resection of the small intestine. Our aim is to make clinicians aware of intussusception and malignant polyps coexisting in Peutz-Jeghers syndrome when performing emergency surgery. Prophylaxis and polypectomy of the entire small bowel is an effective way to reduce the frequency of laparotomies in patients with this disease.

Peutz-Jeghers syndrome: A circumventable emergency

“…[ 5 ] In a recent case report, the patient presented with intussuseption and the polyps turned out to be adenocarcinoma. [ 9 ] The polyps in our case revealed to be a MALToma which is considered to be an extranodal marginal zone B-cell lymphoma. This is very rare and perhaps the third reported case of MALToma in a patient of PJS.…”

“…The more common types of intussusception are jejunojejunal, jejunoileal, ileoileal, ileocecal and rarely duodenojejunal-jejunal type. [ 7 8 9 ] The patient generally presents to the emergency with features of acute abdomen, i.e., pain, constipation/obstipation, nausea, vomiting, abdominal distention, tachycardia, tachypnea, and hypotension. After the initial resuscitation, emergency X-ray abdomen and if possible, computed tomography (CT) abdomen is advisable.…”

“…There are numerous screening tests such as barium enterography, wireless capsule endoscopy, CT/magnetic resonance imaging enteroclysis with oral contrast, double contrast CT colonography to screen gastrointestinal polyps,[ 9 ] but these are expensive and cumbersome for a child with a family history of PJS right from birth. Hence, an annual dermatology referral since birth until the development of mucocutaneous melanosis in a patient with family history of PJS seems an inexpensive first-line screening modality, and this can also determine the time from when the screening for polyps can be started.…”