女性假两性畸形临床诊断及手术治疗经验

Clinical Diagnosis and Surgical Treatment Experience for Female Pseudohermaphroditism in 10 Cases

目的探讨女性假两性畸形的临床诊断及手术治疗的经验。方法 2000年3月～2011年10月收治女性假两性畸形10例,其中成人3例,年龄19～25岁,平均年龄21岁,儿童7例,年龄3～7岁,平均年龄4岁。10例患者均表现为外阴畸形,阴蒂不同程度肥大,长约1.9～4.5cm,平均3.2cm,直径0.4～1.1cm,平均0.6cm。阴唇融合,形似男性阴囊,但未触及睾丸、附睾组织。染色体核分析:46,XX,女性核型。彩超检查提示子宫、卵巢等女性内生殖器存在,且未见明显异常;未见明显睾丸、附睾等组织。10例患者均顺利行外阴整形术治疗,即仔细游离海绵体背侧神经血管束,使之与海绵体完全分离,并保存其完整性;部分切除肥大的阴蒂并保留阴蒂头,固定于耻骨阴阜下;若阴蒂过于肥大,可采用阴蒂楔形切口部分切除后缝合的方式缩小阴蒂;矫治大、小阴唇融合畸形,显露尿道外口和阴道外口。结果 10例手术均获得成功。手术时间100～120min,术中出血50～80ml。尿管拔除时间5～7天,术后7～10天出院。1月后复查,切口对合好,Ⅱ/甲愈合,整形后外阴与正常女性类似。结论外阴整形术是治疗女性假两性畸形阴蒂肥大的理想手术方法之一。术后能重建女性外生殖器,使患者外生殖器类似正常女性,提高患者的生活质量。

Objective To explore the experience of diagnosis and surgical treatment for female pseudohermaphroditism. Methods This series included 10 cases of female pseudohermaphroditism（3 adults,whose age ranged from 19 to 25 years with a mean of 21 years; and 7 young girls,whose age ranged from 3 to 7 years with a mean of 4 years）. All the 10 patients presented with abnormal development of vulva and large clitorises, and its length ranged from 1.9 to 4.5 em with a mean of 3.2era and diameter ranged from 0.4 to 1.1 cm with a mean of O. 6 cm. The laboratory examinations showed that the adrenocorticotropin and testosterone increased and the sex chromosomes karyotype of 46 XX. The B - type ultrasound showed that the uterus and ovary were found, and while the testis was not. We used surgery treatment to beauty 10 patient＇s vulvae by makeing the external genitalia being similar to the normal. Results All the 10 operations were completed successfully. Operation time ranged from 100 to 120 min and surgical blood loss ranged from 40 to 80 ml. The time of remove the urethral catheter ranged from 5 to 7 days. The postoperative appearance of external genitalia was similar to the normal after 1 month. Conclusion The surgery is a satisfactory therapy for those patients. Patient＇s genitals were similar to normal female, and also the quality of life improved.