Congenital adrenal hyperplasia presenting as total virilism in a 4 year old child 4.5 year old patient presented with pubic hair, phallic enlargement, accelerated growth and no palpable gonads within the scrotum neither the inguinal area. Nuclear chromatin was positive. Bone age was 7 years. In two separate 24 h urine collections, 17 cetosteroids and pregnanetriol were elevated. Serum 17 hydroxiprogesterone, A 4 androstenedione, dehidroepiandrosterone and testosterone were also elevated, while deoxycorticosterone, corticosterone, follicle stimulating hormone and luteinizing stimulating hormone were normal. Plasma cortisol did not increase after synacthen-rapid administration, Urinary adrenal steroid were supressed by dexamethasone. Pelvic ecothomography showed normal internal female genitalia. Non salt losing congenital adrenal hyperplasia was thus diagnosed. Histerectomy and oophorectomy were performed based on her psychological adaptation to male gender. (Key words: adrenal hyperplasia, congenital, non salt losing, virilism, puberty, precocius.) La hiperplasia suprarrenai congenita (HSC) comprende una serie de alteraciones hereditarias de la esteroidogenesis, siendo el deficit de la 21 hidroxilasa (21-OH) la forma mas frecuente de presentation y la causa mas comun de genitales ambiguos en la mujer.El grado de virilizacion de los genitales externos se relaciona con la cuantfa de la sobreproduccion de androgenos y el peri'odo del desarrollo fetal en que esta se inicia.En las nifias mas severamente masculinizadas se pueden encontrar, excepcionalmente, estructuras prostaticas, fusion completa de labios y formation de uretra peneana, correspondiendo al grado V de la clasificacion de Prader l~4 . Esta forma de presentacion es rara: nemos encontrado 14 casos publicados en la literatura 4^6 , por lo que parece de interes presentar un paciente con dichas caracterfsticas.

Genital reconstruction in congenital adrenal hyperplasiaSurgical results of genital reconstruction in 15 patients with congenital adrenal hyperplasia which were seen in a metropolitan general hospital at Santiago, Chile, along the last 20 years are described. Blood 17 hydroxiprogesterone and urinary pregnanetriol together with sexual positive cromatin were the most useful tests to confirm the clinical diagnosis. Urinary 17 Ketosteroids were not diagnostic in two cases. Preoperative evaluation to define anatomy was done with pelvic ultrasound, genitography and cystoscopy. They were all useful, specially cystoscopy if it was done by experienced persons. Surgical reconstruction of female external genitals was done in 14 girls at childhood with good aesthetic and functional results on the late controls. There was no introital estenosisat followup of cases that reached adulthood, except for one case of small bowel vaginoplasty, which will require further surgery. Male genitalia were asigned to one patient, because his problem was diagnosed late, at age five years, and female sex organs were then extirpated. The patient has good social sex identity but retains many feminine phenothypica! elements.(Key words: Congenital adrenal hyperplasia, genitoplasty.)