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Unusual Presentation of Intussusception of the Small Bowel with Peutz Jeghers Syndrome: Report of a Case

机译:Peutz Jeghers综合征小肠肠套叠的异常表现:一例报告

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The Peutz Jeghers syndrome (PJS) is an autosomal dominant disorder which is characterised by hamartomatous polyposes of the gastrointestinal tract, melanin pigmentation of the skin and mucous membranes, and an increased risk for cancer. We are reporting a case of a 15-year-old male with Peutz Jeghers syndrome, who presented to us with features of chronic intestinal obstruction and anaemia. Initially, patient was managed conservatively, but later on, an elective exploratory laparotomy was done for definitive management of intussusception. Laparotomy revealed a jejuno-jejunal intussusception with spontaneous recanalisation of gut which contained a long segment of gangrenous small bowel in the lumen. Resection and anastomosis of the jejunal segment was done. To the best of our knowledge, this might be the first case report on spontaneous recanalisation of small intestine.
机译:Peutz Jeghers综合征(PJS)是常染色体显性遗传疾病,其特征在于胃肠道的错构瘤多处,皮肤和粘膜的黑色素色素沉着以及罹患癌症的风险增加。我们正在报告一例15岁的男性患有Peutz Jeghers综合征的人,该人向我们展示了慢性肠梗阻和贫血的特征。最初,对患者进行了保守治疗,但随后进行了选择性探索性剖腹术以明确处理肠套叠。剖腹发现空肠肠套叠,肠道自发再通,其内腔中含有一长段坏疽性小肠。空肠段切除和吻合。就我们所知,这可能是关于小肠自发再通的首例病例报告。

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