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Side-to-side bowel anastomosis mimicking intussusception in a 2-year-old child with Peutz-Jeghers syndrome

机译:2岁大Peutz-Jeghers综合征儿童的肠套叠模拟肠套叠

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摘要

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant inherited condition characterized by hamartomatous gastrointestinal polyps, mucocutaneous pigmentation, and a predisposition for malignancy. Most patients with PJS are diagnosed in the second or third decade of life, and commonly have intussusception as a complication. This report describes an unusual case of a 2-year-old male known to have PJS, who had previously developed a small bowel intussusception caused by a polyp requiring a very short segmental small bowel resection. The patient remained asymptomatic several months after the surgery and then presented with acute abdominal discomfort. On abdominal ultrasound, a target sign measuring 2.7 cm was noted in the left upper quadrant of the abdomen, suggesting a small bowel-small bowel intussusception. There was no evidence of intussusception or bowel obstruction otherwise on diagnostic laparoscopy. It was thought that the previous side-to-side anastomosis had mimicked intussusception on the ultrasound examination. A repeat abdominal ultrasound was performed 1 week after the laparoscopy when the patient was asymptomatic. This again demonstrated a target sign identical in appearance to the previous ultrasound and confirmed that the side-to side anastomosis had in fact mimicked intussusception. It is important that the pediatric gastroenterology, radiology, and surgery communities are aware of this ultrasound finding; it could impact the decision on whether to operate emergently. To our knowledge this is the first report describing this unusual scenario in humans.
机译:Peutz-Jeghers综合征(PJS)是一种罕见的常染色体显性遗传疾病,其特征是错构肠胃息肉,皮肤粘膜色素沉着和恶性肿瘤易感性。大多数PJS患者是在生命的第二个或第三个十年中被诊断出的,并且通常以肠套叠为并发症。该报告描述了一个不寻常的病例,该病例为2岁的男性,患有PJS,该男性先前因息肉而需要进行非常短的小肠段切除而形成了小肠肠套叠。术后几个月,患者仍无症状,然后出现腹部剧烈不适。腹部超声检查时,在腹部左上象限发现目标征兆为2.7 cm,表明肠小肠小肠套叠。诊断性腹腔镜检查无肠套叠或肠梗阻的证据。据认为,先前的侧向吻合在超声检查中模仿了肠套叠。当患者无症状时,在腹腔镜检查后1周重复进行腹部超声检查。这再次证明了目标征象在外观上与先前的超声波相同,并证实了从侧面到侧面的吻合实际上模仿了肠套叠。小儿胃肠病学,放射学和手术界必须意识到这一超声发现,这一点很重要。这可能会影响是否紧急运行的决定。据我们所知,这是描述人类这种不寻常情况的第一份报告。

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