真两性畸形10例报告

摘要

From 1976 to 1991,10 patients with true hermaphroditism were treated by surgical intervention.The clinieal features include:perineal hypospadias with a single opening of urogenital sinus,bifid scrotum,a palpable gonad at the groin or hemi - scrotum ( 8 cases)and a moderate phallic development or a hypertrophic clitoris.The definitive diagnosis was established by physical findings,laboratory tests such as chromosomal pattern and so on,laparotomy with exposure of both gonads either in the inguinal region or in the abdomen and biopsies of gonadal tissue for frozen section.In one side the testis was present in all patients and in the opposite side it was found to be an ovary in 6,an ovotestis in 3,overy and a testis in l.Besids,a uterus was found in 7,the fallopian tube development was seen in 9 and breast enlargement occurred in 4 teenage cases.The karyotype of chromosome was as follows:46XX in 4,46XY/46XX ia 1,46XY/45XO in 1.Based on their initial sex,9 patients underwent masculinizstion from hermaphrodite.%10例真性两性畸形均表现为会阴型尿道下裂,尿生殖窦型单一开口,阴囊分裂,阴茎或阴蒂有一定程度的发育.8例患儿一侧腹股沟或分裂的阴囊中可扪及性腺块.染色体型以46,XX为主.10例均经性腺探查及病理活检确诊.10例均经手术治疗,按其原来社会性别,9例改为男性.会阴型尿道下裂伴隐睾者应疑及本病,若同时染色体核型为46,XX,则应作性腺探查以明确诊断.