AI Article Synopsis

  • Peutz-Jeghers Syndrome (PJS) is a hereditary disorder marked by dark spots on mucous membranes and the presence of hamartomatous polyps in the gastrointestinal tract, leading to complications like bleeding and obstruction.
  • A 28-year-old female patient presented with abdominal pain and vomiting, revealing hyperpigmented lesions and diagnosed with intussusception, which was confirmed through tomography and surgically addressed via laparoscopy.
  • To minimize surgical risks and the potential for short bowel syndrome, it’s advised to regularly remove polyps endoscopically and use a combination of endoscopic and laparoscopic techniques for management in patients with PJS.

Article Abstract

Peutz-Jeghers Syndrome (PJS) is a rare autosomal dominant disorder which is characterized by hyperpigmentation in mucocutaneous membranes and hamartomatous polyps in the gastrointestinal tract (GIT). Common complications reported in patients with PSJ are bleeding and mechanical intestinal obstruction due to the hamartomatous polyps. There is also an increased risk of gastrointestinal and extra-intestinal malignancies in patients with PJS. A 28-year-old female patient was admitted to the emergency service with complaints of abdominal pain and vomiting. In addition to distention and tenderness on abdominal examination, revealed hyperpigmented lesions on her lips. An abdominal examination did not reveal any scar from the previous abdominal operation. The patient with suspected mechanical intestinal obstruction at pre-diagnosis demonstrated intussusception in the distal jejunal loops on abdominal tomography. In the diagnostic laparoscopy observed intussusception in jejunal loops. After a minimal suprapubic incision, small intestine loops were checked through alexis with bidigital palpation and no other intraluminal mass were detected. Laparoscopy-assisted jejunojejunal re-section and anastomosis was performed for the intussusception segment, where the polyb is located. It has been recommended that endoscopic polyps removal should be performed to avoid multiple surgical resections, which lead to short bowel syndrome. It has been recommended that endoscopic polyps removal should be performed to avoid multiple surgical resections, which lead to short bowel syndrome. By the nature of the disease, there may be multiple polyps simultaneously in the GIT and the associated risk of recurrent intussusception attacks in patients with PJS. To prevent short bowel syndrome and intra-abdominal adhesions due to repeated, laparo-tomies treatment with combined endoscopy and laparoscopic/laparoscopy-assisted surgery should be preferred in patients with PJS.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10443007PMC
http://dx.doi.org/10.14744/tjtes.2021.34560DOI Listing

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