腹腔镜手术诊治卵睾型性发育异常13例分析
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摘要
目的总结卵睾型性发育异常的腹腔镜手术诊疗经验。方法回顾性分析2015年1月～2018年10月我院收治并确诊卵睾型性发育异常13例资料,年龄6个月～3岁9个月(中位年龄1岁8个月)。社会性别7例为男性,6例为女性。完善检查后行腹腔镜性腺探查,对双侧性腺包括盆腔、腹股沟、阴囊内性腺均予活检。结果 13例腹腔镜性腺探查活检病理结果:双侧卵睾2例,一侧卵巢、一侧睾丸6例,一侧卵睾、一侧睾丸2例,一侧卵巢、一侧卵睾3例。再次手术行性腺切除及外生殖器整形手术。13例随访3～24个月,平均14. 5月。外生殖器发育基本正常,排尿良好,无社会性别与生长发育不协调。结论卵睾型性发育异常患儿的腹腔镜手术诊疗包括性腺探查活检,性腺切除与固定等,手术方案应依据外生殖器表型、性腺优势、社会性别等综合评估,谨慎决定。
Objective To summarize the experience of laparoscopic therapy for ovotesticular disorders of sex development( DSD). Methods From January 2015 to October 2018,13 children with ovotesticular DSD admitted in our hospital underwent laparoscopic treatment. The age for first surgery ranged from 6 months to 3 years 9 months old( median,1 year 8 months). For 7 patients the social gender was male,and for the rest 6 patients the social gender was female. To determine the gonadal tissue components,biopsy of all gonads in the abdominal cavity,intra-scrotal and intra-groin was required after laparoscopic exploration.Results The pathological results were bilateral ovariotestis in 2 cases,unilateral ovary and testis in 6 cases,unilateral ovariotestis and testis in 2 cases,and unilateral ovariotestis and ovary in 3 cases. Based on diagnostic laparoscopic findings,the gonads and/or ductal structures incompatible with the social gender were removed by laparoscopy,and plastic reconstructions of external genitalia were performed. The patients recovered well during a 3-24 months of follow-up( average,14. 5 months) with good morphologic and cosmetic outcomes. No malignant inclination of the gonad or significant retraction of the testis was found. Conclusions Laparoscopic procedures for ovotesticular DSD include gonadal exploration and biopsy,gonadectomy and orchiopexy. The operation schemes are depended on external genitalia phenotype,gonads evaluation,social psychology,and so on.
Objective To summarize the experience of laparoscopic therapy for ovotesticular disorders of sex development( DSD). Methods From January 2015 to October 2018,13 children with ovotesticular DSD admitted in our hospital underwent laparoscopic treatment. The age for first surgery ranged from 6 months to 3 years 9 months old( median,1 year 8 months). For 7 patients the social gender was male,and for the rest 6 patients the social gender was female. To determine the gonadal tissue components,biopsy of all gonads in the abdominal cavity,intra-scrotal and intra-groin was required after laparoscopic exploration.Results The pathological results were bilateral ovariotestis in 2 cases,unilateral ovary and testis in 6 cases,unilateral ovariotestis and testis in 2 cases,and unilateral ovariotestis and ovary in 3 cases. Based on diagnostic laparoscopic findings,the gonads and/or ductal structures incompatible with the social gender were removed by laparoscopy,and plastic reconstructions of external genitalia were performed. The patients recovered well during a 3-24 months of follow-up( average,14. 5 months) with good morphologic and cosmetic outcomes. No malignant inclination of the gonad or significant retraction of the testis was found. Conclusions Laparoscopic procedures for ovotesticular DSD include gonadal exploration and biopsy,gonadectomy and orchiopexy. The operation schemes are depended on external genitalia phenotype,gonads evaluation,social psychology,and so on.
引文
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3马丹,叶明珠,邓新粮,等.腔镜在两性畸形诊治中的应用(附33例报告).中国微创外科杂志,2016,16(10):913-916.
4 Mouriquand PD,Gorduza DB,Gay CL,et al.Surgery in disorders of sex development(DSD)with a gender issue:if(why),when,and how?J Pediatr Urol,2016,12(3):139-149.
5 Kremen J,Chan YM.Genetic evaluation of disorders of sex development:current practice and novel gene discovery.Curr Opin Endocrinol Diabetes Obes,2019,26(1):54-59.
6 Ocal G,Berberoglu M,Siklar Z,et al.Clinical review of 95 patients with 46xx disorders of sex development based on the new Chicago classification.J Pediatr Adolesc Gynecol,2015,28(1):6-11.
7 Ochi T,Ishiyama A,Yazaki Y,et al.Surgical management of hypospadias in cases with concomitant disorders of sex development.Pediatr Surg Int,2019,35(5):611-617.
8 Kutney K,Konczal L,Kaminski B,et al.Challenges in the diagnosis and management of disorders of sex development.Birth Defects Res C Embryo Today,2016,108(4):293-308.
9梁海燕,张潍平,孙宁,等.74例46,XX性发育异常患儿性腺探查结果及分析.中华小儿外科杂志,2016,37(7):517-521.
10 Krob G,Braun A,Kuhnle U.True hermaphroditism:geographical distribution,clinical findings,chromosomes and gonadalhistology.Eur J Pediatr,1994,153(1):2-10.
11 Chowdhury TK,Kabir M,Chowdhury MZ,et al.The challenges in diagnosis and gender assignment in disorders of sex development presenting to a pediatric surgical unit in a developing country:the role of laparoscopy and simple tests for gender identity.J Pediatr Urol,2014,10(6):1255-1260.
12 Binet A,Lardy H,Geslin D,et al.Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype?J Pediatr Surg,2016,51(3):465-468.
13 Callens N,van der Zwan YG,Drop SL,et al.Do surgical interventions influence psychosexual and cosmetic outcomes in women with disorders of sex development?ISRN Endocrinol,2012,2012:276742.
14 Tafazzoli K,Wünsch L,Bouteleux M,et al.Endoscopy and laparoscopy in disorders of sex development.Sex Dev,2018,12(1-3):100-105.
15王小林,张文,周学锋,等.腹腔镜在儿童真两性畸形诊疗中的应用:附5例报告.中国微创外科杂志,2008,8(2):162-163.
16 Wünsch L,Buchholz M.Imaging,endoscopy and diagnostic surgery.Endocr Dev,2014,27:76-86.
17 Steven M,O’Toole S,Lam JP,et al.Laparoscopy versus ultrasonography for the evaluation of Mullerian structures in children with complex disorders of sex development.Pediatr Surg Int,2012,28(12):1161-1164.
18 Mizuno K,Kojima Y,Nishio H,et al.Transumbilical laparoendoscopic single-site gonadectomy for Turner’s syndrome with Y-chromosome mosaicism.J Pediatr Urol,2012,8(4):e39-42.
2 Hughes IA.Disorders of sex development:a new definition and classification.Best Pract Res Clin Endocrinol Metab,2008,22(1):119-134.
3马丹,叶明珠,邓新粮,等.腔镜在两性畸形诊治中的应用(附33例报告).中国微创外科杂志,2016,16(10):913-916.
4 Mouriquand PD,Gorduza DB,Gay CL,et al.Surgery in disorders of sex development(DSD)with a gender issue:if(why),when,and how?J Pediatr Urol,2016,12(3):139-149.
5 Kremen J,Chan YM.Genetic evaluation of disorders of sex development:current practice and novel gene discovery.Curr Opin Endocrinol Diabetes Obes,2019,26(1):54-59.
6 Ocal G,Berberoglu M,Siklar Z,et al.Clinical review of 95 patients with 46xx disorders of sex development based on the new Chicago classification.J Pediatr Adolesc Gynecol,2015,28(1):6-11.
7 Ochi T,Ishiyama A,Yazaki Y,et al.Surgical management of hypospadias in cases with concomitant disorders of sex development.Pediatr Surg Int,2019,35(5):611-617.
8 Kutney K,Konczal L,Kaminski B,et al.Challenges in the diagnosis and management of disorders of sex development.Birth Defects Res C Embryo Today,2016,108(4):293-308.
9梁海燕,张潍平,孙宁,等.74例46,XX性发育异常患儿性腺探查结果及分析.中华小儿外科杂志,2016,37(7):517-521.
10 Krob G,Braun A,Kuhnle U.True hermaphroditism:geographical distribution,clinical findings,chromosomes and gonadalhistology.Eur J Pediatr,1994,153(1):2-10.
11 Chowdhury TK,Kabir M,Chowdhury MZ,et al.The challenges in diagnosis and gender assignment in disorders of sex development presenting to a pediatric surgical unit in a developing country:the role of laparoscopy and simple tests for gender identity.J Pediatr Urol,2014,10(6):1255-1260.
12 Binet A,Lardy H,Geslin D,et al.Should we question early feminizing genitoplasty for patients with congenital adrenal hyperplasia and XX karyotype?J Pediatr Surg,2016,51(3):465-468.
13 Callens N,van der Zwan YG,Drop SL,et al.Do surgical interventions influence psychosexual and cosmetic outcomes in women with disorders of sex development?ISRN Endocrinol,2012,2012:276742.
14 Tafazzoli K,Wünsch L,Bouteleux M,et al.Endoscopy and laparoscopy in disorders of sex development.Sex Dev,2018,12(1-3):100-105.
15王小林,张文,周学锋,等.腹腔镜在儿童真两性畸形诊疗中的应用:附5例报告.中国微创外科杂志,2008,8(2):162-163.
16 Wünsch L,Buchholz M.Imaging,endoscopy and diagnostic surgery.Endocr Dev,2014,27:76-86.
17 Steven M,O’Toole S,Lam JP,et al.Laparoscopy versus ultrasonography for the evaluation of Mullerian structures in children with complex disorders of sex development.Pediatr Surg Int,2012,28(12):1161-1164.
18 Mizuno K,Kojima Y,Nishio H,et al.Transumbilical laparoendoscopic single-site gonadectomy for Turner’s syndrome with Y-chromosome mosaicism.J Pediatr Urol,2012,8(4):e39-42.
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