The gonads of 111 South African patients with ovotesticular disorder of sex differentiation

Abstract

Purpose The aims of this study were to describe the gonadal tissue found in the Southern African true hermaphrodite and establish if there was a correlation between the clinical and histopathologic findings and if these findings were similar to patients with this condition elsewhere. Materials and Methods A retrospective study at the University of KwaZulu-Natal, Durban, South Africa, looked at all patients diagnosed with true hermaphroditism seen between 1984 and 2006. For this 23-year period, 111 consecutive true hermaphrodite patients were diagnosed on clinical findings, internal genital assessments, and the histologic examination of 217 gonadal biopsy specimens. All gonadal tissue taken from these patients was sent for histopathologic evaluation. The results were correlated to the clinical and internal genital evaluations of the patients. Results Five patients only had a single gonad. Analysis of the gonadal biopsy specimens showed that there were 118 (54%) ovotestes together with 59 ovaries and 40 testes. The ovotestes were divisible on gross appearance into 11% bipolar and 89% mixed types. Histologically, the mixed-type ovotestes have an outer mantle consisting of ovarian tissue, which encapsulated an inner core of 2 distinct types. The first is an admixed ovotestis (constituting 44% of the mixed ovotestes), the central core consisted of gonadal stroma, with scattered foci of separate ovarian and testicular tissue. The second type was the compartmentalized ovotestis (constituting 56% of the mixed ovotestes); here, the outer mantle was thickened in the upper pole and encapsulated a large core of testicular tissue in the lower pole of the gonad. The bipolar ovotestis had a strictly polar distribution of ovarian and testicular tissue, which had an irregularly interdigitating junction between the 2 types of tissue. Statistical analysis showed that no correlation could be found between the type of gonadal tissue and any of the clinical or genital features. Conclusion Three distinct ovotesticular types are identified in the Southern African true hermaphrodite, which have not been described previously. The structure of these gonads has bearing on the type of biopsy done and the subsequent management of the ovotestes.