Lin Xiaoyan,Zhang Heng,Zhuo Jinwei,et al.Rapunzel syndrome in children: a report of 5 cases with a review of the literature[J].Journal of Clinical Pediatric Surgery,2023,22(01):92-96.[doi:10.3760/cma.j.cn101785-202106053-018]
儿童Rapunzel综合征诊治分析
- Title:
- Rapunzel syndrome in children: a report of 5 cases with a review of the literature
- Keywords:
- Intestinal Obstruction; Bezoars; Gastroenterostomy; Intestinal Perforation; Surgical Procedures; Operative; Child
- 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:
- ObjectiveRapunzel 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.MethodsFrom 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.ResultsAll 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.ConclusionRapunzel 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.
参考文献/References:
[1] Vaughan ED Jr,Sawyers JL,Scott HW Jr.The Rapunzel syndrome.An unusual complication of intestinal bezoar[J].Surgery,1968,63(2):339-343.
[2] 杨彦,侯中和,陈庆文,等.Rapunzel综合征一例[J].中华放射学杂志,2006,40(10):1030.DOI:10.3760/j.issn:1005-1201.2006.10.027. Yang Y,Hou ZH,Chen QW,et al.A case of Rapunzel syndrome[J].Chin J Radiol,2006,40(10):1030.DOI:10.3760/j.issn:1005-1201.2006.10.027.
[3] 孙有成,刘远梅.小儿胃内巨大毛发结石1例[J].临床小儿外科杂志,2007,6(1):45.DOI:10.3969/j.issn.1671-6353.2007.01.035. Sun YC,Liu YM.A case of huge hair stone in stomach of children[J].J Clin Ped Sur,2007,6(1):45.DOI:10.3969/j.issn.1671-6353.2007.01.035.
[4] Dogra S,Yadav YK,Sharma U,et al.Rapunzel syndrome causing appendicitis in an 8-year-old girl[J].Int J Trichology,2012,4(4):278-279.DOI:10.4103/0974-7753.111203.
[5] Prasanna BK,Sasikumar K,Gurunandan U,et al.Rapunzel syndrome:a rare presentation with multiple small intestinal intussusceptions[J].World J Gastrointest Surg,2013,5(10):282-284.DOI:10.4240/wjgs.v5.i10.282.
[6] Fallon SC,Slater BJ,Larimer EL,et al.The surgical management of Rapunzel syndrome:a case series and literature review[J].J Pediatr Surg,2013,48(4):830-834.DOI:10.1016/j.jpedsurg.2012.07.046.
[7] Islek A,Sayar E,Y?lmaz A,et al.A rare outcome of iron deficiency and pica:Rapunzel syndrome in a 5-year-old child iron deficiency and pica[J].Turk J Gastroenterol,2014,25(1):100-102.DOI:10.5152/tjg.2014.4051.
[8] Marwah S,Pandey S,Raj A,et al.Rapunzel syndrome presenting as jejuno-jejunal intussusception[J].Clin J Gastroenterol,2015,8(4):202-206.DOI:10.1007/s12328-015-0578-7.
[9] Ahmed N,Baloch MA,Baber KM,et al.A rare variant of Rapunzel syndrome-acute small bowel obstruction caused by ball of hairs in distal ileum with its tail extending in caecum and ascending colon[J].J Pak Med Assoc,2016,66(6):761-764.
[10] Koushk Jalali B,Bing l A,Reyad A.Laparoscopic management of acute pancreatitis secondary to Rapunzel syndrome[J].Case Rep Surg,2016,2016:7638504.DOI:10.1155/2016/7638504.
[11] Rojas PG,Paredes EB,Reto CP.Rapunzel syndrome as a cause of obstruction and intestinal perforation[J].Acta Gastroenterol Latinoam,2016,46(2):114-117.
[12] Wolski M,Gawl?owska-Sawosz M,Gogolewski M,et al.Trichotillomania,trichophagia,trichobezoar-summary of three cases.Endoscopic follow up scheme in trichotillomania[J].Psychiatr Pol,2016,50(1):145-152.DOI:10.12740/PP/43636.
[13] 丁媛媛,李稳.内镜下治疗长发公主综合征1例报道[J].胃肠病学和肝病学杂志,2016,25(12):1432-1433.DOI:10.3969/j.issn.1006-5709.2016.12.037. Ding YY,Li W.Endoscopic treatment of Rapunzel syndrome:one case report[J].Chin J Gastroenterol Hepatol,2016,25(12):1432-1433.DOI:10.3969/j.issn.1006-5709.2016.12.037.
[14] 郑静,露娜,马智,等.超声诊断长发公主综合征1例[J].中国医学影像技术,2016,32(1):133.DOI:10.13929/j.1003-3289.2016.01.034. Zheng J,Lu N,Ma Z,et al.Ultrasonographic diagnosis of Rapunzel syndrome:one case report[J].Chin J Med Imaging Technol,2016,32(1):133.DOI:10.13929/j.1003-3289.2016.01.034.
[15] Bolívar-Rodríguez MA,Fierro-López R,Pamanes-Lozano A,et al.Surgical outcome of jejunum-jejunum intussusception secondary to Rapunzel syndrome:a case report[J].J Med Case Rep,2018,12(1):362.DOI:10.1186/s13256-018-1883-9.
[16] Cannalire G,Conti L,Celoni M,et al.Rapunzel syndrome:an infrequent cause of severe iron deficiency anemia and abdominal pain presenting to the pediatric emergency department[J].BMC Pediatr,2018,18(1):125.DOI:10.1186/s12887-018-1097-8.
[17] 郭飞,徐彦磊,张大,等.小儿毛发性胃石症一例[J].临床小儿外科杂志,2018,17(2):159-160.DOI:10.3969/j.issn.1671-6353.2018.02.017. Guo F,Xu YL,Zhang D,et al.A case of hairy gastrolith in a child[J].J Clin Ped Sur,2018,17(2):159-160.DOI:10.3969/j.issn.1671-6353.2018.02.017.
[18] 王峰.罕见巨大毛发胃石症1例[J].中国临床医学影像杂志,2018,29(9):677-678.DOI:10.12117/jccmi.2018.09.020. Wang F.Huge hair gastrolithiasis:one case report[J].J China Clin Med Imaging,2018,29(9):677-678.DOI:10.12117/jccmi.2018.09.020.
[19] Finale E,Franceschini P,Danesino C,et al.Rapunzel syndrome:how to orient the diagnosis[J].Pediatr Rep,2018,10(2):7689.DOI:10.4081/pr.2018.7689.
[20] Iwama I,Nambu R,Hara T.A novel finding of Rapunzel syndrome[J].Clin J Gastroenterol,2018,11(1):19-22.DOI:10.1007/s12328-017-0790-8.
[21] Nour I,Abd Alatef M,Megahed A,et al.Rapunzel syndrome (gastric trichobezoar),a rare presentation with generalised oedema:case report and review of the literature[J].Paediatr Int Child Health,2019,39(1):76-78.DOI:10.1080/20469047.2017.1389809.
[22] Soria Alcívar M,Betancourt Ruiz M,Moyon Gus?ay M,et al.Giant Rapunzel syndrome with atypical complication.Report of a case[J].Rev Gastroenterol Peru,2019,39(1):74-77.
[23] Sotoudeh E,Hussain S,Shafaat O,et al.Fungal peritonitis with fungus balls,a complication of trichobezoars and Rapunzel syndrome[J].Am J Case Rep,2019,20:685-688.DOI:10.12659/AJCR.915517.
[24] 赵国策,樊超强,聂绪彪,等.内镜下多器械组合治疗长发公主综合征1例[J].局解手术学杂志,2019,28(10):847-849.DOI:10.11659/jjssx.07E019059. Zhao GC,Fan CQ,Nie XB,et al.Endoscopic treatment and follow-up for Rapunzel syndrome:one case report[J].J Reg Anat Oper Surg,2019,28(10):847-849.DOI:10.11659/jjssx.07E019059.
[25] 黄一敏,刘江斌,刘海峰,等.胃镜辅助经腹壁小切口胃切开术治疗儿童毛发性胃石症[J].中华小儿外科杂志,2019,40(1):28-31.DOI:10.3760/cma.j.issn.0253-3006.2019.01.006. Huang YM,Liu JB,Liu HF,et al.Guidance of small-incision-gastrotomy by gastroscopy for trichobezoar in children[J].Chin J Pediatr Surg,2019,40(1):28-31.DOI:10.3760/cma.j.issn.0253-3006.2019.01.006.
[26] Ni?ǎ AF,Hill CJ,Lindley RM,et al.Human and doll’s hair in a gastric trichobezoar,endoscopic retrieval hazards[J].J Pediatr Gastroenterol Nutr,2020,71(2):163-170.DOI:10.1097/MPG.0000000000002779.
[27] Stinco M,Montemaggi A,Noccioli B,et al.An unusual case of hypoproteinemia in childhood:keep in mind trichobezoar[J].Front Pediatr,2020,8:82.DOI:10.3389/fped.2020.00082.
[28] Mirza MB,Talat N,Saleem M.Gastrointestinal trichobezoar:an experience with 17 cases[J].J Pediatr Surg,2020,55(11):2504-2509.DOI:10.1016/j.jpedsurg.2020.04.020.
[29] Jackman J,Nana GR,Catton J,et al.Gastric perforation secondary to Rapunzel syndrome[J].BMJ Case Rep,2021,14(2):e240100.DOI:10.1136/bcr-2020-240100.
[30] Grant JE,Chamberlain SR.Trichotillomania[J].Am J Psychiatry,2016,173(9):868-874.DOI:10.1176/appi.ajp.2016.15111432.
[31] Pereyra AD,Saadabadi A.Trichotillomania[M/OL]//Anon.StatPearls[Internet].Treasure Island (FL):StatPearls Publishing,2021.https://pubmed.ncbi.nlm.nih.gov/29630238/.
[32] Flessner CA,Knopik VS,McGeary J.Hair pulling disorder (trichotillomania):genes,neurobiology,and a model for understanding impulsivity and compulsivity[J].Psychiatry Res,2012,199(3):151-158.DOI:10.1016/j.psychres.2012.03.039.
[33] 陈潇,李炳荣,纪建松,等.Rapunzel综合征CT表现两例[J].中华放射学杂志,2019,53(7):623-624.DOI:10.3760/cma.j.issn.1005-1201.2019.07.019. Chen X,Li BR,Ji JS,et al.CT features of Rapunzel syndrome:two cases reports[J].Chin J Radiol,2019,53(7):623-624.DOI:10.3760/cma.j.issn.1005-1201.2019.07.019.
[34] Farhat LC,Olfson E,Nasir M,et al.Pharmacological and behavioral treatment for trichotillomania:an updated systematic review with meta-analysis[J].Depress Anxiety,2020,37(8):715-727.DOI:10.1002/da.23028.
相似文献/References:
[1]张生 金先庆 李晓庆 周德凯 向丽 刘伟. 先天性肠闭锁、肠狭窄120例临床分析[J].临床小儿外科杂志,2011,10(04):273.
[J].Journal of Clinical Pediatric Surgery,2011,10(01):273.
[2]龙雪峰 李强辉 向波 陈小勋 周维模 郭鹏. 腹腔镜手术在小儿急性粪石性小肠梗阻[J].临床小儿外科杂志,2011,10(06):440.
[J].Journal of Clinical Pediatric Surgery,2011,10(01):440.
[3]陈盛,严志龙,梅董昱,等. 极低出生体重儿动力性肠梗阻的外科干预指征与治疗策略研究[J].临床小儿外科杂志,2018,17(05):349.
Chen Sheng,Yan Zhilong,Mei Dongyu,et al.Surgical treatments for verylowbirthweight infants with dynamic intestinal obstruction.[J].Journal of Clinical Pediatric Surgery,2018,17(01):349.
[4]唐华建,郑锦涛,李伟燎,等.V 型瓣菱形吻合法治疗新生儿环状胰腺的临床研究[J].临床小儿外科杂志,2018,17(07):542.
Tang Huajian,Zheng Jintao,Li Weiliao,et al.Efficacies of modified diamondshaped anastomosis for neonatal annular pancreas.[J].Journal of Clinical Pediatric Surgery,2018,17(01):542.
[5]单禹华,顾松,徐敏,等.小儿肝移植术后肠梗阻病因鉴别及预后分析[J].临床小儿外科杂志,2021,20(06):576.[doi:10.12260/lcxewkzz.2021.06.013]
Shan Yuhua,Gu Song,Xu Min,et al.Differentiation and prognosis of intestinal obstruction after pediatric liver transplantation[J].Journal of Clinical Pediatric Surgery,2021,20(01):576.[doi:10.12260/lcxewkzz.2021.06.013]
备注/Memo
收稿日期:2021-06-19。
基金项目:浙江省医药卫生科技计划项目(2021ZH070、2022ZH019)
通讯作者:张恒,Email:zhangheng2114890@163.com