[Good results with Passerini's technique in severely masculinised female pseudohermaphroditism]

Grupo de Urología Pediatrica, Hospital Vall d'Hebron, Barcelona.

In female pseudohermaphroditism due to congenital adrenal hyperplasia (CAH), some cases develope a very important masculinization degree with a high outlet of the vagina above the outern urethral sphincter. There are several surgical techniques to solve this problem. Our group uses Passerini's thecnique since 1990. 9 Girls with CAH with extreme masculitation (IV and V Prader degree) have been operated with this technique. The age at intervention varies between 9 months and 3 years, with a follow-up between 1 and 12 years. All these patients had hormonal studies, demostrating a deficiency of 21-hydroxilase in 8 cases and a deficiency of 11-B-hydroxilase in one case. To determine the vaginal outlet a genitography was performed in all cases, vaginoscopy in 7 cases and MNR in 2 cases. Passerini's technique consists of two phases: the first step has the objective of forming the distal 2/3 of the vagina using the skin and the urethral mucosa of the phallus. At the second time transtrigonal way is performed to access the vagina which is desinserted from its urethral outlet and anastomosed with the distal neovagina. There were no significative complications in immediate time. The esthetic result is acceptable in all cases, although one case had to be surgically repaired because of a prominent vulvar flap. We use vaginoscopy to prove vaginal permeability in all cases although one of them developed adherences around the anastomosis. This severe genital malformation, very uncommon, has a difficult surgical solution. Passerini's technique allows, with less complications, to create an acceptable outer genitals at early age and only in one surgical time.

PMID: 15503946 [PubMed - indexed for MEDLINE]