儿童Peutz-Jeghers综合征二例

Two Cases of Peutz-Jeghers Syndrome in Children

目的分析儿童Peutz-Jeghers综合征的临床特点,探讨临床工作中Peutz-Jeghers综合征患儿急诊手术的注意事项和诊疗经验。方法回顾分析2例Peutz-Jeghers综合征患儿的临床资料。结果1例患儿以腹痛、呕吐起病,急诊行剖腹探查,行部分十二指肠切除术,术后腹痛复发,遂行肠套叠复位术,考虑Peutz-Jeghers综合征合并肠套叠;另1例患儿以腹痛、呕吐起病,急诊行剖腹探查,术后恢复良好,考虑Peutz-Jeghers综合征合并肠套叠。结论对于皮肤粘膜黑色斑点且出现外科急腹症的患儿,应考虑Peutz-Jeghers综合征,应首先解决患儿的急腹症,尽可能保留套叠的肠段,术中切除较为明显的息肉,不做预防性息肉切除,术后依据病理检查结果和基因检测结果,定期对患儿进行全胃肠道监测和息肉切除术,提高患儿远期生活质量。

Objective To analyze the clinical characteristics of children with Peutz-Jeghers syndrome,and to explore the precautions and experience in emergency surgery for children with Peutz-Jeghers syndrome in clinical work.Methods The clinical data of 2 children with Peutz-Jeghers syndrome were retrospectively analyzed.Result One patient presented with abdominal pain and vomiting.Emergency exploratory laparotomy and partial duodenectomy were performed.The abdominal pain recurred after operation,and intussusception reduction was performed.Peutz-Jeghers syndrome combined with intussusception was considered.The patient presented with abdominal pain and vomiting,and underwent exploratory laparotomy in emergency.The postoperative recovery was good.Peutz-Jeghers syndrome complicated with intussusception was considered.Conclusion Peutz-Jeghers syndrome should be considered for children with black spots on the skin and mucous membranes and a surgical acute abdomen.The acute abdomen should be resolved first,the intussusception of the intestinal segment should be preserved as much as possible,and the more obvious polyps should be removed during the operation.Prophylactic polypectomy is not performed.According to the results of pathological examination and genetic testing,the children are regularly monitored for total gastrointestinal tract and polypectomy to improve the long-term quality of life of the children.