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<p>Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant genetic disorder characterised by gastrointestinal hamartomatous polyps, mucocutaneous hyperpigmentation, and multisystemic tumour susceptibility. We reports a case of a 12-year-old female patient with PJS who presented with acute abdominal pain and vomiting. Abdominal CT revealed small intestinal obstruction with ischaemic necrosis and two distinct intussusceptions. Emergency laparotomy confirmed two segments of intussuscepted small intestine with bowel necrosis. She was successfully treated through emergency surgical resection of the necrotic segments followed by end-to-end anastomosis. Postoperative double-balloon enteroscopy (DBE) at 3 months removed 18 high-risk polyps. The significance of this case lies in its first report of concurrent double intussusception with intestinal necrosis in a paediatric patient with PJS. It underscores the value of early recognition of the specific sign of skin and mucosal pigmentation, providing reference for the early diagnosis, emergency treatment, and clinical management of PJS-related intussusception. This aims to enhance clinicians’ diagnostic and therapeutic awareness of this condition.</p>

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Life-Threatening Manifestations of Peutz-Jeghers Syndrome: A Case of Pediatric Double Intussusception Complicated by Intestinal Necrosis

  • 期刊论文

  • 发表日期：

    2026年2月13日

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  • Ying Luo,
  • Hanlei Li,
  • Shuicai Huang,
  • Junjie Yang,
  • Zhenhuai Yang,
  • Zetian Yang

摘要

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant genetic disorder characterised by gastrointestinal hamartomatous polyps, mucocutaneous hyperpigmentation, and multisystemic tumour susceptibility. We reports a case of a 12-year-old female patient with PJS who presented with acute abdominal pain and vomiting. Abdominal CT revealed small intestinal obstruction with ischaemic necrosis and two distinct intussusceptions. Emergency laparotomy confirmed two segments of intussuscepted small intestine with bowel necrosis. She was successfully treated through emergency surgical resection of the necrotic segments followed by end-to-end anastomosis. Postoperative double-balloon enteroscopy (DBE) at 3 months removed 18 high-risk polyps. The significance of this case lies in its first report of concurrent double intussusception with intestinal necrosis in a paediatric patient with PJS. It underscores the value of early recognition of the specific sign of skin and mucosal pigmentation, providing reference for the early diagnosis, emergency treatment, and clinical management of PJS-related intussusception. This aims to enhance clinicians’ diagnostic and therapeutic awareness of this condition.