Adult Intussusception in Patients with Peutz-Jeghers Syndrome: Case Series and Review of Literature

Abstract

Background: Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterized by gastrointestinal hamartomatous polyps and mucocutaneous pigmentations in the mouth, facial skin, hands & feet. Small bowel obstruction, intussusception, bleeding, intestinal and extra-intestinal malignancies are the major complications of PJS. The aim of this study is to analyze the clinical characteristics, preoperative diagnosis, and surgical management of PJS associated-intussusception in adults. Patients and Methods: This study included 5 cases with intussusception in PJS patients presented to Surgical

An unusual presentation revealing Peutz-Jeghers syndrome in adult

“…Peutz-Jeghers syndrome (PJS) is a rare inherited autosomal dominant disease, with an estimated prevalence from 1 in 100,000 people [ 1 ]. It was first described by Peutz in 1921 and Jeghers in 1944 and 1949 [ 2 ]. This disorder is seen equally in both male and female patients and usually diagnosed during childhood or early adulthood [ 3 , 4 ].…”

Peutz–Jeghers Syndrome: A Rare Genetic Cause of Intestinal Obstruction

“…polyps are found in approximately 80% of the cases predominantly in the jejunum, followed by the ileum, the stomach and large intestine 6. It can also be found in extraintestinal sites including the CBMJ 2023 January: Vol.…”

“…12 No. 01 nasal passages, bronchial tree, lung, breast, kidney, ureter, pancreatic, gallbladder, uterine, ovarian and testicular tumors 5,6. Although the hamartomatous polyps are known with low malignant potential, individuals with this disease are at elevated risk of acquiring malignancies.3 Overall lifetime GI cancer risks of 55-85% and the reported lifetime risk for any cancer varied according to the literature between 37% and 93% at the age of 60-70 years.…”