摘要：目的 探讨儿童Rapunzel综合征的临床特点及诊治对策。方法 回顾性分析2019年1月至2021年5月丽水市中心医院收治的5例Rapunzel综合征患儿临床表现、治疗过程和转归资料。以检索词“Rapunzel/长发公主、毛发结石”检索万方、维普、中国知网数据库, 以“Rapunzel syndrome、trichobezoar”检索PubMed、Embase数据库相关文献, 剔除文献综述、重复报道病例、成人病例及无法获得详细资料的病例后, 对相关文献进行复习和总结。结果 本院5例均为女性, 4例长发, 1例短发且有秃发。5例均以呕吐、腹痛、腹部包块等胃肠道梗阻症状就诊。5例均于术前经胃镜和(或)CT等检查证实存在消化道异物, 结合有食毛或拔毛史, 经手术确诊为Rapunzel综合征。1例伴电解质紊乱、多发小肠穿孔; 1例伴营养不良、体重下降; 1例并发肠缺血坏死, 2例无明显并发症及合并症。5例均通过小切口剖腹手术取出毛发团块, 平均手术时间2h, 平均住院时间10d, 均恢复良好顺利出院。出院后于本院精神卫生科随诊, 随访时间3个月至3年, 平均随访时间1.7年, 随访期间无一例复发。共收集Rapunzel综合征相关文献28篇(52例), 男女比例为3∶49, 平均年龄11.73岁, 有拔毛史35例(35/52, 67.3%); 均行手术治疗, 以开腹手术为主(39/52, 75%), 以胃肠切开取异物术为主要术式; 51例预后良好, 1例术前合并胃穿孔患儿术后因重度营养不良、持续消化道出血死亡。结论 儿童Rapunzel综合征往往引起营养不良等非特异性表现, 可导致肠梗阻、隐匿性肠穿孔等并发症。CT和胃镜可早期诊断, 手术是该病的主要治疗手段, 长期精神科随诊及心理治疗可预防复发。

Abstract: Objective Rapunzel syndrome is rare in children and delayed diagnosis and treatment may lead to various complications. The purpose of this paper was to explore the clinical characteristics and diagnostic and treatment countermeasures of Rapunzel syndrome in children. Methods From January 2019 to May 2021, retrospective review was conducted for clinical manifestations, treatment courses and outcomes of five hospitalized cases of children with Rapunzel syndrome. The search terms of "Rapunzel/Princess Rapunzel, trichobezoar" were employed for searching the databases of Wanfang, Wipu, CNKI and "Rapunzel syndrome, trichobezoar". The relevant literatures of PubMed and Embase databases were summarized after excluding literature reviews, repeated cases, adult cases and cases for which detailed information was non-available. Results All five children were female, four with long hair and one with short hair and alopecia. Clinical manifestations included gastrointestinal obstruction such as vomiting, abdominal pain and abdominal masses, etc. All five cases were confirmed to have gastrointestinal foreign bodies by gastroscopy and/or CT pre-operation and the diagnosis of Rapunzel's syndrome was finally confirmed by a history of hair eating or plucking. Complications were electrolyte disturbance and multiple small bowel perforations (n=1), malnutrition and weight loss (n=1) and intestinal ischemic necrosis (n=1). The remainders had no significant complications or comorbidities. Hair masses were removed by small incisional dissection. The average operative duration was 2h and the average hospital stay 10 days. All of them recovered well and were discharged successfully. After discharge, consultations with mental health department continued. None of them had recurrence during the mean follow-up period of 1.7 years (3 months to 3 years). Through a literature retrieval, a total of 28 literature reports in Chinese and English were collected, with 52 cases with a male to female ratio of 3 ∶ 49 and 94.2% of females. The mean age was 11.73 years and there were 28 cases with a history of hair extraction (28/52, 53.8%). Fifty-two children were operated and laparotomy (39/52, 75%) predominated with gastroenterostomy for foreign body removal. The outcome was excellent in 51 of them, except for one child with preoperative gastric perforation who died postoperatively due to severe malnutrition and persistent gastrointestinal bleeding. Conclusion Rapunzel syndrome in children often causes non-specific manifestations such as malnutrition and can lead to complications such as intestinal obstruction and occult bowel perforation, and delayed treatment may be life-threatening. CT and gastroscopy may provide an early diagnosis and surgery remains a major treatment. Long-term psychiatric follow-ups and psychotherapy prevents a recurrence.