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CASE REPORT
Peutz-Jeghers syndrome: need for early screening
  1. Kashish Khanna1,
  2. Vikram Khanna2 and
  3. Veereshwar Bhatnagar1
  1. 1 Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, Delhi, India
  2. 2 Department of Pediatric Surgery, Lady Hardinge Medical College, New Delhi, Delhi, India
  1. Correspondence to Dr Kashish Khanna, kash.modern{at}gmail.com

Abstract

Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer-predisposing condition characterised by intestinal hamartomatous polyps and distinct melanin depositions in skin and mucosa. Small intestinal cancer in patients with PJS usually presents by the third decade. A 7-year-old-PJS boy presented with recurrent episodes of colicky abdominal pain and melena requiring repeated blood transfusions. Abdominal CT scan revealed multiple jejunal polyps with jejunoileal intussusception. On exploration, the intussuscepted bowel was resected along with its mesentery and anastomosed. Simultaneously, multiple enterotomies with resection of palpable polyps were performed. The resected bowel showed well-differentiated stage 2A adenocarcinoma with clear resected margins. Postoperatively, the complaints were relieved. On follow-up, he was asymptomatic and is now on yearly cancer surveillance. This is probably the youngest reported case of small bowel cancer in PJS.

  • gi bleeding
  • small intestine
  • small intestine cancer
  • gastrointestinal surgery
  • paediatric surgery
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Footnotes

  • Patient consent for publication Obtained.

  • Contributors The patient was admitted under the care of VB. The child was operated and followed up by VB and VK. The manuscript was proposed and drafted by KK and VK. The final revised and corrected manuscript was approved by all authors before submission.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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