INTRODUCTION: Treatment of the cryptorchid testicle is justified due to the increased risk of infertility and malignancy as well as the risk of testicular trauma and psychological stigma on patients and their parents. Approximately 20% of cryptorchid testicles are nonpalpable. In these cases, the videolaparoscopic technique is a useful alternative method for diagnosis and treatment. MATERIALS AND METHODS: We present data concerning 90 patients submitted to diagnostic laparoscopy for impalpable testicles. Forty-six patients (51.1%) had intra-abdominal gonads. In 25 testicles of 19 patients, we performed a two stage laparoscopic Fowler-Stephens orchiopexy. The other 27 patients underwent primary laparoscopic orchiopexy, in a total of 29 testicles. RESULTS: We obtained an overall 88% success rate with the 2 stage Fowler-Stephens approach and only 33% rate success using one stage Fowler-Stephens surgery with primary vascular ligature. There was no intraoperative complication in our group of patients. In the laparoscopic procedures, the cosmetic aspect is remarkably more favorable as compared to open surgeries. Hospital stay and convalescence were brief. CONCLUSIONS: In pediatric age group, the laparoscopic approach is safe and feasible. Furthermore, the laparoscopic orchiopexy presents excellent results in terms of diagnosis and therapy of the impalpable testis, which is why this technique has been routinely incorporated in our Department.

OBJECTIVE: Klippel-Trenaunay-Weber syndrome (KTWS) is a congenital condition characterized by vascular malformations of the capillary, venous and lymphatic systems associated to soft tissue and bone hypertrophy in the affected areas. This syndrome may involve bladder, kidney, urethra, ureter and genitals. We report the treatment of 7 KTWS patients with urogenital involvement. MATERIALS AND METHODS: From 1995 to 2005, 7 patients with KTWS were evaluated and the charts of these patients were reviewed. RESULTS: Patients’ median age was 19-years (range 4 to 46-years) and only 1 was female. The clinical presentation included genital deformities in 3 cases, hematuria in 2 and urethrorragia in 2, one of which associated with cryptorchidism and phimosis. Three patients had an association of pelvic and genital malformations, including 2 patients with hematuria due to vesical lesions and 1 patient with left ureterohydronephrosis due to a pelvic mass. Two patients had urethral lesions. Treatment included endoscopic laser coagulation for 1 patient with recurrent hematuria and 1 patient with urethrorrhagia, pelvic radiotherapy for 1 patient with hematuria and circumcision in 2 patients with genital deformities. One patient required placement of a double-J catheter to relieve obstruction. Hematuria and urethrorragia were safely and effectively controlled with laser applications. Circumcision was also effective. The patient treated with radiotherapy developed a contracted bladder and required a continent urinary diversion. CONCLUSIONS: Urogenital involvement in patients with KTWS is not rare and must be suspected in the presence of hematuria or significant cutaneous deformity of the external genitalia. Surgical treatment may be warranted in selected cases.

O tratamento da hiperplasia supra-renal congênita por deficiência da 21-hidroxilase (HSRC-21OH) tem como objetivos repor glico e mineralocorticóides, evitar a virilização dos genitais externos, prevenir a desidratação por perda de sal, controlar o hiperandrogenismo sem afetar a velocidade de crescimento, preservar a função gonadal, fertilidade e estatura final. Relatamos a nossa experiência no acompanhamento de 96 pacientes com HSRC-21OH. Nas crianças utilizamos como glicocorticóide o acetato de cortisona (18-20 mg/m²/dia) e nos adultos a dexametasona (0,25-0,75 mg/dia). Quando necessário, a reposição do mineralocorticóide foi feita com 9alfa-fluor-hidrocortisona 50-250 µg/dia dependendo da faixa etária. Apesar da substituição adequada com glico e/ou mineralocorticóides o resultado final do tratamento da HSRC-21OH ainda deixa a desejar, principalmente em relação ao crescimento, já que a estatura final na maior parte das casuísticas está entre -1 e -2 DP em relação a estatura alvo. Novas terapêuticas da HSRC-21OH, como a associação de hidrocortisona, fludrocortisona, flutamida e testolactona ou a suprarrenalectomia cirúrgica ainda estão em fase experimental e a evolução a longo prazo é necessária para avaliar seu real efeito.

The purpose of the treatment for congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH-21OH) is to replace gluco and mineralocorticoids, prevent virilization of the external genitalia, salt loss and hyperandrogenism and preserve gonadal function, fertility and final height. Our experience in the treatment of 96 patients with CAH-21OH is presented. Children have been treated with cortisone acetate (18-20 mg/m2/day) and adults with dexamethasone (0.25-0.75 mg/day). If necessary, mineralocorticoid replacement was accomplished with 9alpha-fludrocortisone, 50-250 µg/day, according to chronological age. Despite therapy with gluco and mineralocorticoids the end results for CAH-21OH is still inadequate, especially when growth is concerned; in most reports final height reaches between 1 to 2 SD bellow target height. New therapeutic approaches for CAH-21OH, such as the association of flutamide and testolactone to hydrocortisone and fludrocortisone, and even surgical or chemical adrenalectomy, are still experimental and long-term follow-up is necessary to evaluate their validity.