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 Oncology Unit, General Surgery Department, Tanta University Hospital, Egypt and Hamad General Hospital, Hamad Medical Corporation, Qatar, between October 2011 and March 2016. Patients’ demographics were collected. After thorough clinical examination, abdominal X-ray, US, & CT scan were done. All the patients were submitted to midline laparotomy with resection anastomosis of the affected bowel segment. Results: The mean age was 28.4 years. Female: male ratio was 3:2. Abdominal pain was the most common presenting complaint with or without intestinal obstruction manifestations. Palpable abdominal mass was found in 3 patients (60%). Intussusception was proved pre-operatively in all the cases by abdominal ultrasound and CT scan. The intussusception was found in the jejunum in 3 patients, ileum in 1 patient, & in 1 patient, there was double intussusception (one jejunal & one ileo-cecal). Histopathological examination revealed the presence of typical Peutz-Jeghers hamartomatous polyp. No morbidity or mortality was reported at a mean follow-up period of 32 months. Conclusion: Family history, physical examination, abdominal ultrasound and CT scan were important in the diagnosis of acute intussusception caused by PJS. Surgical management of PJS associated intussusception is the recommended treatment to relieve patient’s symptoms and to avoid missing underlying malignancy. Patients with PJS should be followed up throughout their lives because of the increased risk of malignant changes.
Highlights
Peutz-Jeghers syndrome (PJS) is a rare inherited autosomal dominant disorder, first described by Peutz in 1921 and Jeghers in 1944 and 1949
In this study; we presented 5 cases with intussusception caused by Peutz-Jeghers syndrome who had presented to the Surgical Oncology Unit at General Surgery Department, Tanta University Hospital, Egypt and Hamad General Hospital, Hamad Medical Corporation, Qatar, between October 2011 and March 2016
Case 2 had a prior laparotomy for a previous intussusception and small intestinal polypectomy
Summary
Peutz-Jeghers syndrome (PJS) is a rare inherited autosomal dominant disorder, first described by Peutz in 1921 and Jeghers in 1944 and 1949. Peutz-Jeghers syndrome is characterized by gastrointestinal hamartomatous polyps and mucocutaneous pigmentations in the oral cavity, lips, peri-oral area, facial skin, hands or feet [3] [4]. These polyps are found in approximately 80% of the cases predominantly in the jejunum, followed by the ileum, the stomach and large intestine. 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. Patients with PJS should be followed up throughout their lives because of the increased risk of malignant changes
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