Surgical treatment of girls with congential adrenal hypertrophy
Article published in Urologia Polska 2001/54/4.

authors

Maria Wieczorek-Grohman ¹, Wiesław Urbanowicz ¹, Jerzy Starzyk ²

¹ Klinika Urologii Dziecięcej Katedry Chirurgii Pediatrycznej Uniwersytetu Jagiellońskiego w Krakowie Kierownik kliniki: dr hab. med. Wiesław Urbanowicz
² Klinika Endokrynologii Dzieci i Młodzieży Katedry Pediatrii Uniwersytetu Jagiellońskiego w Krakowie Kierownik kliniki: prof, dr hab. med. Antoni Dziatkowiak

keywords

congenital adrenal hypertrophia, virilism, feminizing genitoplasty

summary

Objective. In children, the most common form of hermaphroditism is female pseudohermaphroditism, mostly manifested as congenital adrenal hypertrophy (CAH). As a consequence of an enzymatic delect, external genital organs become virilized and the patient has to be treated surgically.

Material and methods. Forty-nine girls with CAH were operated on. Group 1 included nine patients subjected to a synthesis of feminization techniques developed by Snyder et al., 14 patients from Group 2 were operated on employing the same technique modified by the authors, while Group 3 included 26 girls in whom the modified procedure was subjected to another modification.

Results. The most satisfactory results were achieved in Group 3, where the technique was modified twice. Very good and good results were obtained in 95.9% of cases.

Conclusions. The reconstruction of urogenital abnormalities in girls with CAH should involve all the components of the defect. The principle underlying the clitoroplasty should be the preservation of the glans with an undamaged dorsal ncuro-vascular bundle while performing a total resection of the cavernous bodies. The modifications introduced by the authors allow for a radical procedure and eliminate risks involved in exposing the dorsal neuro-vascular bundle.

references

correspondence