腹腔镜结合膀胱镜手术治疗苗勒管永存综合征

Laparoscopic management of persistent miillerian duct combined with cystoscope.

目的回顾性分析本院自2003年至2011年经腹腔镜手术治疗的11例苗勒管永存综合征（Persistentmiillerianductsyndrome，PMDS）患儿的临床特点。方法检索我院数据库中腹腔镜手术的PMDS患儿临床资料，包括手术时年龄、临床表现（症状和体征）、术中发现和处理等。结果2003年2月至2011年7月，共有11例患儿行腹腔镜苗勒管切除术。手术时平均年龄（49．82±41．20）个月（5—145个月）。除早期有1例因出血中转开放手术外，其余10例均为全程腹腔镜手术。11例中，5例以尿道下裂收入院，2例以隐睾收入院，5例以腹痛和附睾一睾丸炎入院；6例有隐睾，其中4例左侧（包括1例睾丸横过异位），2例双侧（包括1例右侧睾丸横过异位）；6例有尿道下裂；4例同时有尿道下裂和隐睾；3例外阴完全正常而表现为腹痛伴附睾一睾丸炎（2例），腹部包块伴感染（1例）。6例因为输精管进入苗勒管囊肿中，而不得不将其切断。在最近3例患儿中，我们采用了腹腔镜结合膀胱尿道镜的方法，以确定苗氏管残余的边界和切除范围。结论苗勒管残余结构经常在手术探查时发现。对于有症状的病例，如反复感染、腹痛等，苗勒管结构切除是必需的。腹腔镜结合膀胱尿道镜的手术方法具有创伤小、切除彻底、安全系数高等优势。

Objetive Persistent mtillerian duct syndrome （PMDS） is a rare malformation, character- ized by the presence of mttllerian structures in a virilized male, frequently presents as undescended testis, ei- ther intraabdominal or within a hernial sac. We intend to describe the characters and surgical management of the patients with PMDS by laparoscopic procedure in our hospital from February 2003 to July 2011. Methods We indexed all the cases of PMDS operated by laparoscopic procedure in our hospital＇ s database. These eases were reviewed for the ages at operation, the chief complaint, the symptoms and signs presenting, and the find- ing and management in the operations. Results 11 patients with PMDS were treated by laparoscopic proce- dure since 2003 when such procedure was held in our hospital for the first time. The mean age at operation was 49.82 ±41.20 months（ raging from 5 to 145 months）. In one case open procedure was taken at last because of the difficulty to handle bleeding during our early experience. In all the 11 patients, 5 were hospitalized as hy- pospadias, 2 were hospitalized as cryptorchidism, and 5 were hospitalized as abdominal pain and epididymo-or- chitis. 6 paitients had cryptorchidism（ 4 left side including one transverse testicular ectopia, 2 bilateral incht- ding one right side transverse testicular ectopia）. 6 patients had hypospadias. 4 patients had both hypospadias and cryptorehidism. 3 patients had absolutely normal appearance of genitalia and presented as abdominal pain with epididymo-orehitis （ two cases） or abdominal mass with infection（ one case）. In 6 cases the vasa defer- entia were cut off because of their entrance into the maUerian cyst. Cystoscope was used to assist the laparo- scopic procedure as a guide to identify the edge of the mttUerian duct remnant and its junction to the urethra in the very last 3 cases. Conclusions The diagnosis is almost exclusively obtained during surgical exploration, at which time the often unanticipated mtillerian structures are encountered. Excision of the mtillerian structures is recommended in symptomatic cases such as that with repetatus infection or abdominal pain. Laparoscope com- bined with cystoscope is an effective minimally invasive technique to the management of these patients.