Department of Gastroenterological Surgery, Osaka International Cancer Institute, 3-1-69 Otemae, Chuo-ku, Osaka, 541-8567, Japan.
Department of Surgery, JCHO Hoshigaoka Medical Center, 4-8-1, Hoshigaoka, Hirakata, Osaka, 573-8511, Japan.
World J Surg Oncol. 2021 Jan 21;19(1):22. doi: 10.1186/s12957-021-02133-5.
Peutz-Jeghers syndrome (PJS) is a very rare autosomal dominant genetic disorder characterized by hamartomatous polyps in the gastrointestinal tract and hyperpigmentation of the lips, hands, and feet. The hamartomatous polyps in the small intestine often cause intussusception and bleeding.
A 62-year-old male was hospitalized for treatment of deep vein thrombosis and pulmonary embolism. In the small intestine, computed tomography showed three small polyps with intussusceptions. Since the patient had gastrointestinal polyposis and pigmentation of his lips, fingers, and toes, he was diagnosed with PJS. After an inferior vena cava filter was placed, he underwent laparoscopic-assisted surgery. The polyps causing intussusception were resected as far as possible without intestinal resection, since they had caused progressive anemia and might cause intestinal obstruction in the future. The patient was discharged from the hospital on postoperative day 9 without complications.
Laparoscopic-assisted disinvagination and polypectomy is a useful, minimally invasive treatment for multiple intussusceptions caused by small intestinal polyps in patients with PJS.
Peutz-Jeghers 综合征（PJS）是一种非常罕见的常染色体显性遗传疾病，其特征为胃肠道错构瘤和嘴唇、手和脚的色素沉着。小肠的错构瘤常导致肠套叠和出血。
一名 62 岁男性因深静脉血栓形成和肺栓塞住院治疗。小肠计算机断层扫描显示有三个带肠套叠的小息肉。由于患者有胃肠道息肉和嘴唇、手指和脚趾的色素沉着，因此被诊断为 PJS。在下腔静脉滤器放置后，他接受了腹腔镜辅助手术。尽可能地切除引起肠套叠的息肉，而不进行肠切除术，因为它们已导致进行性贫血，并可能在将来引起肠梗阻。患者术后第 9 天出院，无并发症。
腹腔镜辅助松解和息肉切除术是一种有用的微创治疗方法，适用于 PJS 患者因小肠息肉引起的多发性肠套叠。
