Abstract：
BACKGROUND: Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by hamartomatous gastrointestinal polyps along with the characteristic mucocutaneous freckling. Multiple surgeries for recurrent intussusception in these children may lead to short bowel syndrome. Here we present our experience of management in such patients.
METHODS: From January 2015 to December 2023, we reviewed children of PJS, presented with recurrent intussusceptions. Data were collected regarding presentation, management, and follow-up with attention on management dilemma. Diagnosis of PJS was based on criteria laid by World Health Organization (WHO).
RESULTS: A total of nine patients were presented with age ranging from 4 to 17 years (median 9 years). A total of eighteen laparotomies were performed (7 outside, 11 at our centre). Among 11 laparotomies done at our centre, resection and anastomosis of bowel was done 3 times while 8 times enterotomy and polypectomy was done after reduction of intussusception. Upper and lower gastrointestinal endoscopy (UGIE & LGIE) was done in all cases while intraoperative enteroscopy (IOE) performed when required. Follow-up ranged from 2 months to 7 years.
CONCLUSIONS: Children with PJS have a high risk of multiple laparotomies due to polyps\' complications. Considering the diffuse involvement of the gut, early decision of surgery and extensive bowel resection should not be done. Conservative treatment must be tried under close observation whenever there is surgical dilemma. The treatment should be directed in the form of limited resection or polypectomy after reduction of intussusception.
METHODS: From January 2015 to December 2023, we reviewed children of PJS, presented with recurrent intussusceptions. Data were collected regarding presentation, management, and follow-up with attention on management dilemma. Diagnosis of PJS was based on criteria laid by World Health Organization (WHO).
RESULTS: A total of nine patients were presented with age ranging from 4 to 17 years (median 9 years). A total of eighteen laparotomies were performed (7 outside, 11 at our centre). Among 11 laparotomies done at our centre, resection and anastomosis of bowel was done 3 times while 8 times enterotomy and polypectomy was done after reduction of intussusception. Upper and lower gastrointestinal endoscopy (UGIE & LGIE) was done in all cases while intraoperative enteroscopy (IOE) performed when required. Follow-up ranged from 2 months to 7 years.
CONCLUSIONS: Children with PJS have a high risk of multiple laparotomies due to polyps\' complications. Considering the diffuse involvement of the gut, early decision of surgery and extensive bowel resection should not be done. Conservative treatment must be tried under close observation whenever there is surgical dilemma. The treatment should be directed in the form of limited resection or polypectomy after reduction of intussusception.
摘要：
背景：Peutz-Jeghers综合征（PJS）是一种常染色体显性遗传性疾病，其特征是错构瘤性胃肠道息肉以及特征性粘膜皮肤雀斑。这些儿童复发性肠套叠的多次手术可能导致短肠综合征。在这里，我们介绍了我们对此类患者的管理经验。
方法：从2015年1月到2023年12月，我们回顾了PJS的孩子，表现为复发性肠套叠。收集了关于演示的数据，管理,并关注管理困境。PJS的诊断基于世界卫生组织（WHO）制定的标准。
结果：共有9名患者的年龄为4至17岁（中位数为9岁）。共进行了18次开腹手术（7次开腹手术，11在我们的中心）。在我们中心做的11例开腹手术中，肠套叠复位后肠切除吻合3次，肠切开术和息肉切除术8次。在所有情况下都进行了上消化道和下消化道内窥镜检查（UGIE和LGIE），而在需要时进行了术中肠镜检查（IOE）。随访2个月至7年。
结论：患有PJS的儿童由于息肉并发症而发生多次开腹手术的风险很高。考虑到肠道的扩散参与，不应早期决定手术和广泛的肠切除术.每当出现手术困境时，必须在密切观察下尝试保守治疗。肠套叠复位后，应以有限切除或息肉切除术的形式进行治疗。
方法：从2015年1月到2023年12月，我们回顾了PJS的孩子，表现为复发性肠套叠。收集了关于演示的数据，管理,并关注管理困境。PJS的诊断基于世界卫生组织（WHO）制定的标准。
结果：共有9名患者的年龄为4至17岁（中位数为9岁）。共进行了18次开腹手术（7次开腹手术，11在我们的中心）。在我们中心做的11例开腹手术中，肠套叠复位后肠切除吻合3次，肠切开术和息肉切除术8次。在所有情况下都进行了上消化道和下消化道内窥镜检查（UGIE和LGIE），而在需要时进行了术中肠镜检查（IOE）。随访2个月至7年。
结论：患有PJS的儿童由于息肉并发症而发生多次开腹手术的风险很高。考虑到肠道的扩散参与，不应早期决定手术和广泛的肠切除术.每当出现手术困境时，必须在密切观察下尝试保守治疗。肠套叠复位后，应以有限切除或息肉切除术的形式进行治疗。
