儿童苗勒管永存综合征的手术治疗  

Surgical management of persistent Müllerian duct syndrome in children

作　　者：孟庆明[1] 罗添华[1] 张旭辉[1] 王彩红[1] 陈柏峰[1] 王玮 王东 孟称心[1] Meng Qingming;Luo Tianhua;Zhang Xuhui;Wang Caihong;Chen Baifeng;Wang Wei;Wang Dong;Meng Chenxin(Department of Urology,Shanxi Province Children's Hospital,Taiyuan 030001,China)

机构地区：[1]山西省儿童医院泌尿外科,太原030001

出　　处：《中华实用儿科临床杂志》2025年第3期214-217,共4页Chinese Journal of Applied Clinical Pediatrics

基　　金：山西省基础研究计划资助项目(202203021211064)。

摘　　要：目的探索临床治疗儿童苗勒管永存综合征(PMDS)可行的手术方案。方法回顾性病例总结。分析2011年9月至2020年11月山西省儿童医院诊治的8例PMDS的病例资料, 其中6例为散发病例, 2例为双胞胎(单绒毛膜), 年龄1~12岁。病例均有正常男性外生殖器, 染色体核型均为46, XY。术中根据临床分型、输精管结构及始基子宫发育情况选择手术方案, 术后通过门诊彩超随访患儿腹腔内苗勒管结构及睾丸发育情况。结果 4例为腹股沟斜疝型, 3例为双侧隐睾型, 1例为睾丸横过异位型。苗勒管抑制激素(MIH):5例明显降低, 1例正常, 余2例未行MIH检测。双侧性腺活检均为睾丸组织, 但3例可见未分化性腺组织。4例先行性腺活检, 再行根治性手术, 其中3例行一次性手术治疗, 1例先行斜疝修补, 再行根治性手术。3例输精管结构清楚, 行始基子宫切除, 双侧睾丸固定术;3例输精管结构模糊行保留始基子宫的睾丸固定术;1例行始基子宫切除, 一侧睾丸固定术, 另一侧睾丸切除术;1例行Fowler-Stephens分期双侧睾丸固定术。患儿均获得术后随访, 随访时间2~12年, 彩超显示下降睾丸发育良好, 未见隐睾回缩和疝复发, 腹腔内保留始基子宫者未见肿瘤生长。结论 PMDS儿童期的手术治疗应根据其临床分型选择合适的治疗方案, 性腺活检是必不可少的, 是否切除苗勒管结构应根据输精管结构和始基子宫的发育情况综合决定。Objective:To explore feasible surgical options for management of persistent Müllerian duct syndrome(PMDS)in children.Methods:A retrospective case summary was made.The clinical data of 8 patients who were diagnosed with PMDS and treated at Shanxi Children′s Hospital from September 2011 to November 2020 were retrospectively reviewed.Of the 8 PMDS patients,6 were sporadic cases,2 were twins(monochorionic),with an age range from 1 year to 12 years.All cases had normal male external genital organs and a chromosomal karyotype of 46,XY.During the operation,the surgical plan was selected according to clinical classification,vas structure and the development of the initial uterus.After the operation,the Müllerian structure in the abdominal cavity and the testicular development of the children were followed up through color ultrasound at the outpatient department.Results:Four patients had oblique inguinal hernia,3 had bilateral cryptorchidism,and 1 had transverse testicular ectopia.Müllerian inhibitory hormone(MIH)levels were significantly decreased in 5 cases and normal in 1 case.The other 2 cases were not tested for MIH.All bilateral gonadal biopsies were testicular tissue,but undifferentiated gonadal tissue was detected in 3 cases.Four cases underwent radical surgery after gonadal biopsy.Among these 4 children,3 received one-stage radical operation,and 1 received repair of oblique hernia before the radical surgery.Three patients had a well-defined vas structure and underwent hysterectomy and bilateral orchidopexy;three patients had a vague vas structure and received orchidopexy with the uterus preserved;one case underwent hysterectomy,orchidopexy on one side and orchiectomy on the other side;one case had bilateral orchidectomy in Fowler-Stephens stage.All the 8 children were followed up for 2 to 12 years.Ultrasound showed that the descending testis was well developed,with no cryptorchidism retraction or hernia recurrence.No tumor was observed in the preserved uterus in the abdominal cavity.Conclusions:The plan for

关 键 词：儿童 苗勒管永存综合征 手术治疗 

分 类 号：R73[医药卫生—肿瘤]