THE PATTERN OF INTERSEX DISORDERS AND GENDER ASSIGNMENT IN THE EASTERN PROVINCE OF SAUDI-ARABIA

Objective: To determine the pattern of intersex disorders manifestable as ambiguous genitalia and the gender assignment of the patients. Des ign: A retrospective study of patients with ambiguous genitalia to det ermine the associated intersex disorders and the gender assignment. Se tting: King Fahd Hospital of King Faisal University, Al-Khobar, Saudi Arabia. Subjects: Over 10 years 25 patients with ambiguous genitalia w ere seen. Results: Male pseudohermaphroditism, gonadal dysgenesis and female pseudohermaphroditism occurred in 56%, 8% and 36%, respectively . There were no true hermaphrodites. At birth, 21% and 79% of male pse udohermaphrodites were assigned a male and a female gender, respective ly. At puberty, most of these females insisted on masculinization oper ations, based on their male gender identity and behaviour and their ma le genotype. The parents of female pseudohermaphrodites, including 22% assigned a male gender at birth, accepted clitoro-vaginoplasty based on the female genotype. Conclusions: Our observation that male pseudoh ermaphroditism formed the highest proportion of intersex disorders is explained, in part, by the high incidence of patients with pseudovagin al perineoscrotal hypospadias. There is, therefore, a possibility of a cluster of male pseudohermaphroditism due to 5 alpha-reductase defici ency in the Eastern Province making determination of dihydrotestostero ne desirable.