Resection of Over 290 Polyps During Emergency Surgery for Four Intussusceptions with Peutz–Jeghers Syndrome: Report of a Case

Abstract

A 41-year-old male patient with aggravated epigastralgia and nausea was admitted to Central Aizu General Hospital in February 1997. His past history showed a colonic polyp and anemia in the fourth decade. The patient looked healthy, but showed abdominal distension and tenderness, and pigmented lips. A plain abdominal X-ray revealed a dilation of the small intestine with niveau. Computed tomography disclosed multiple target signs. An emergency laparotomy clarified four intussusceptions of the small intestine with numerous polyps. Three were successfully reduced, while one jejunal intussusception was resected. Due to a fear of recurrence, a total of over 290 polyps were removed. His illness was diagnosed to be Peutz-Jeghers syndrome with a histology of hamartomatous polyps. He thereafter did well for 6 years, when he underwent an ileal resection for another intussusception caused by a newly grown lipoma. He was able to retain his job, but anemia and hypoproteinemia due to the proliferation of polyps necessitated treatments at the outpatient clinic. In May 2005, he underwent a third emergency laparotomy for an intussusception, followed by a resection of the ileum and 54 polyps. Since then he has been able to lead a normal life.

Seven-Year Follow-Up of Peutz-Jeghers Syndrome

“…Some consider these polyps as premalignant lesions, which should be paid considerable attention and removed by surgery or endoscopy. However, others found out that rate of cancer in these polyps is very low, so periodic endoscopic surveillance is enough [ 5 , 10 , 11 ]. As a limitation in this case report, there was not mutation analysis to find out a defect in the STK11 gene is responsible for the PJS phenotype in this patient.…”

Clinical Presentations and Surgical Approach of Acute Intussusception Caused by Peutz–Jeghers Syndrome in Adults

“…Although surgical resection is recommended, there is still no consensus regarding optimal management of adult intussusception 3,19,21 . Definitive surgical resection remains the recommended treatment in most cases of PJS associated with intussusception because of the large proportion of structural causes and the relatively high incidence of malignancy 19,[22][23][24][25] . Previous studies have also shown that the majority of the patients suffer from recurrent episodes of intussusception induced by polyps who require laparotomy 5,26 .…”

Double intussusception in a teenage child with Peutz-Jeghers syndrome: A case report

“…Definitive surgical intervention is recommended in PJS patients complicated by intestinal obstruction, evidence of malignancy, or considerable gastrointestinal hemorrhage[ 10 - 12 ]. In our case, the patient was complicated by intestinal obstruction caused by intussusceptions due to intestinal polyps.…”