SHEN Jian,BI Yun-li.Laparoscopic management of persistent müllerian duct combined with cystoscope[J].Journal of Clinical Pediatric Surgery,2013,12(02):107-109.[doi:10.3969/j.issn.1671— 6353.2013.02.009]
腹腔镜结合膀胱镜手术治疗苗勒管永存综合征
- Title:
- Laparoscopic management of persistent müllerian duct combined with cystoscope
- Keywords:
- Laparoscopy; Cystoscopy; Pseudohermaphroditism/SU; Cryptorchidism/SU
- 摘要:
- 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(Persistent müllerian duct syndrome,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 — 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 — 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。
- Abstract:
- Objetive Persistent müllerian duct syndrome(PMDS)is a rare malformation, characterized by the presence of müllerian structures in a virilized male, frequently presents as undescended testis, either 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 cases were reviewed for the ages at operation, the chief complaint, the symptoms and signs presenting, and the finding and management in the operations. Results 11 patients with PMDS were treated by laparoscopic procedure 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 hypospadias, 2 were hospitalized as cryptorchidism, and 5 were hospitalized as abdominal pain and epididymo-orchitis. 6 paitients had cryptorchidism( 4 left side including one transverse testicular ectopia, 2 bilateral including one right side transverse testicular ectopia). 6 patients had hypospadias. 4 patients had both hypospadias and cryptorchidism. 3 patients had absolutely normal appearance of genitalia and presented as abdominal pain with epididymo-orchitis( two cases)or abdominal mass with infection( one case). In 6 cases the vasa deferentia were cut off because of their entrance into the müllerian cyst. Cystoscope was used to assist the laparoscopic procedure as a guide to identify the edge of the müllerian 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 müllerian structures are encountered. Excision of the müllerian structures is recommended in symptomatic cases such as that with repetatus infection or abdominal pain. Laparoscope combined with cystoscope is an effective minimally invasive technique to the management of these patients.
参考文献/References:
1 Nathalie di Clemente, Anti-Müllerian hormone receptor defect [J]. Best Practice & Research Clinical Endocrinology & Metabolism, 2006, 20(4): 59 9— 610. 2Josso N, Cate RL, Picard JY, et al. Anti-Müllerian hormone, the Jost factor. In Bardin CW(ed.). Recent Progress in Hormone Research[J]. San Diego: Academic Press, 1993. pp. 1 — 59. 3Rey R, Lukas-Croisier C, Lasala C, et al. AMH/MIS: what we know already about the gene, the protein and its regulation[J]. Molecular and Cellular Endocrinology 2003; 211: 21 — 31. 4Wuerstle M, Lesser T, Hurwitz R, et al. Persistent müllerian duct syndrome and transverse testicular ectopia [J]. Journal of Pediatric Surgery,2007, 42: 211 6— 2119. 5Vandersteen DR, Chaumeton AK, Ireland K, et al. Surgical management of persistent müllerian duct syndrome[J]. Urology,1997, 49: 941 — 945. 6Lima M, Aquino A, Dòmini M, et al. Laparoscopic removal of Müllerian duct remnants in boys [J]. J Urol, 2004, 171(1): 36 4— 368.
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