Gender Assignment for Children With Intersex Problems: An Egyptian Perspective

Gender identity is a complex process of differentiation that is affected by numerous variables. Three-hundred and fourteen cases of intersex were diagnosed and surgically managed between 1986 and 2000. Cases of male pseudohermaphroditism (MPH) formed the major group (59 %) ; 27 % had female pseudohermaphroditism (FPH) . Mixed gonadal dysgenesis (MGD) and true hermaphroditism (TH) were the most common variants of the abnormal gonadal differentiation group ( 13 1and 18 cases respectively ) . Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency constituted the major part of FPH (n =76 ) . Fifty-three per cent of patients were raised as males at the time of presentation. Forty-three patients (13 % ) reassigned their sex after diagnosis of their disease : 31 cases (10 % ) changed their sex from females to males, the majority was due to either 5 α-reductase deficiency or partial androgen insensitivity syndrome(PAIS), (16 and 14 cases respectively). Twelve cases (4 % ) reversed their sex from males to females, CAH was the cause in 10 patients . Ninety-eight cases (31 %) were considered as “ sex misassignment” , while 41 cases (13% ) retained a “discordant sex” even after their sex-counseling. Only 16 cases (5% ) were diagnosed in the first three months of life which reflected the major defect in early diagnosis of such conditions in the community; a problem which was also proved by the large number of cases diagnosed at adolescence (n = 9). Familial occurrence was reported in 36 patients. Gonadoblastoma was detected in 2 cases of PAIS and TH. Several social, cultural, and religious factors related to the this local area influencing the gender assignment of such patients were elicited. Several reconstructive surgical procedures were performed after reassignment with excision of all discordant tissues e.g. gonadectomy (n=131) and hysterectomy (n=33).This included a wide variety of either feminizing genitoplasty e.g. clitoroplasty (n=104), vaginoplasty (n =110) and labioplasty (n =84) or male genital reconstruction such as urethroplasty (n=160), scrotoplasty (n=78) ,orchiopexy (n=40), etc…The different factors influencing the decision of rearing in this community should be properly comprehended so as to avoid the problems of mismanagement of intersex conditions with early screening of such cases.