RESUMEN
PURPOSE: We reviewed cosmetic and functional outcome of masculinizing genitoplasty (MPG) in intersex patients assigned as a male performed in our service for more than 12 years. MATERIALS AND METHODS: A total of 57 patients underwent MPG in our department during a 20-year period. Of these cases MPG was performed using modern 1-stage surgical techniques from 1991 to 2003 in 39 (68%). Of the 39 children 8 (21%) had 17beta-hydroxysteroid dehydrogenase deficiency, 2 (5%) had 3beta-hydroxysteroid dehydrogenase deficiency, 8 (21%) had 5alpha-reductase deficiency, 2 (5%) had mixed gonadal dysgenesis, 1 (2.6%) was a true hermaphrodite, 1 (2.6%) had Klinefelter's syndrome, 1 (2.6%) had partially androgen insensitivity syndrome and 16 (41%) had idiopathic male pseudohermaphroditism. The presenting disease was severe proximal penoscrotal hypospadias in 30 cases (77%), perineal hypospadias in 9 (23%) and müllerian duct opening in the perineum along with the urethral meatus in 16 (28%). Median patient age at surgery was 1.8 years. MPG was performed with a transverse pedicled preputial island flap as an onlay in 29 cases (74%). The remaining 10 patients (26%) underwent tubularization of the mucosa in the perineal area and end-to-end anastomosis to a tube made from the pedicled prepuce. Scrotal transposition as well as orchiopexy was performed in some patients as an independent operation so as not to jeopardize the perineal and preputial flaps. RESULTS: In 23 (59%) of the 39 children 1 operation achieved satisfactory cosmetic and functional results in terms of good urinary stream and straight phallus during erection. Three (7%) children presented with various degrees of breakdown of the urethroplasty and required a repeat operation. In 5 patients (12.8%) a small urethral fistula developed and closure was performed. CONCLUSIONS: A 1-stage male genitoplasty for male pseudohermaphroditism is accompanied by a reasonable incidence of major complications. It should be performed in early childhood to avoid psychological and social anxiety by the child and parents.
Asunto(s)
Trastornos del Desarrollo Sexual/cirugía , Genitales Masculinos/cirugía , Procedimientos Quirúrgicos Urogenitales/métodos , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Colgajos Quirúrgicos , Resultado del TratamientoRESUMEN
PURPOSE: We compared long-term morbidity associated with left in situ nonfunctioning or poorly functioning renal moiety of a duplex system in children with prenatal vs postnatal diagnosis of ureterocele who underwent endoscopic puncture. MATERIALS AND METHODS: A total of 48 children underwent primary endoscopic puncture of duplex system ureterocele. Of the cases 35 (73%) were diagnosed prenatally (group 1) and 13 (27%) postnatally (group 2). Median age at time of puncture was 4 months in group 1 and 3.5 years in group 2. A total of 20 patients in group 1 (57%) and 8 in group 2 (62%) presented with intravesical ureterocele, while 15 in group 1 (43%) and 5 in group 2 (38%) had ectopic ureterocele. A total of 20 children in group 1 (57%) and 7 in group 2 (54%) had a nonfunctioning renal moiety, and 15 in group 1 (43%) and 6 in group 2 (46%) had a poorly functioning ureterocele moiety. Vesicoureteral reflux (VUR) was present in 23 children in group 1 (66%) comprising 30 renal refluxing units (RRUs), and in 12 in group 2 (92%) comprising 14 RRUs. Median followup was 9 years (range 1 to 15) for both groups. RESULTS: Preoperative urinary tract infection (UTI) was common in group 2 (92%) vs group 1 (20%). No patient in group 1 had development of UTI after puncture, while 23% of the children in group 2 presented with UTI. Four children (2 from each group) with ectopic ureterocele required secondary puncture resulting in satisfactory drainage. A total of 14 RRUs (47%) showed spontaneous resolution of VUR in group 1 compared to 3 (21%) in group 2. Four RRUs (13%) required endoscopic correction due to high grade VUR in group 1. Two RRUs (17%) were treated with endoscopic correction and 2 (17%) with ureteral reimplantation due to UTI in group 2. Only 1 patient in group 1 underwent nephrectomy due to nonfunctioning kidney, while 2 patients in group 2 required partial nephrectomy due to UTI. CONCLUSIONS: Our data reveal that prenatal diagnosis of duplex system ureterocele is associated with fewer UTIs, and early endoscopic management may decrease UTI and the need for additional surgery. Nonfunctioning or poorly functioning renal moieties left in situ following successful endoscopic decompression of ureterocele are not associated with additional morbidity and do not require partial nephrectomy in the majority of the cases.
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Enfermedades Fetales/diagnóstico , Enfermedades Renales/etiología , Diagnóstico Prenatal , Punciones/métodos , Ureterocele/cirugía , Ureteroscopía/métodos , Factores de Edad , Preescolar , Coristoma/diagnóstico , Femenino , Estudios de Seguimiento , Humanos , Lactante , Nefrectomía , Embarazo , Estudios Retrospectivos , Resultado del Tratamiento , Uréter/anomalías , Uréter/cirugía , Ureterocele/clasificación , Ureterocele/diagnóstico , Infecciones Urinarias/etiología , Reflujo Vesicoureteral/etiologíaRESUMEN
OBJECTIVE: To evaluate the long-term efficacy of a tunica albuginea dorsal plication technique for treating congenital and acquired penile curvature. PATIENTS AND METHODS: We retrospectively evaluated 83 patients (median age 1.8 years) who had their penile curvature corrected surgically using dorsal tunica albuginea plication between 1992 and 2002. The results were evaluated objectively using a pharmacological erection test or subsequently based either on the parents' reports or patients' self-assessment. The median (range) follow-up was 6 (0.7-10) years. RESULTS: Seventy (84%) patients had penile plication as an integral part of hypospadias repair, while the remaining 13 (16%) with a normal urethra had dorsal plication only. Twenty-eight (34%) of the 83 patients had an erection test during a repeat hypospadias repair or closure of a urethrocutaneous fistula; 22 of these had a straight penis, while the remaining six required additional plication for a satisfactory cosmetic outcome. Parents of 45 (54%) children reported that their child had a normal erection with no chordee during the follow-up. Ten (12%) adult patients reported straight erections enabling satisfactory penetration and sensation during sexual intercourse. None of the patients reported penile shortening or erectile dysfunction after surgery, and none had recurrent curvature during the follow-up. There was no difference in the results between patients with congenital or acquired penile curvature. CONCLUSIONS: Dorsal plication of the tunica albuginea is a simple and effective method in the long term for correcting congenital and acquired penile curvature.
Asunto(s)
Induración Peniana/cirugía , Pene/cirugía , Adolescente , Adulto , Niño , Preescolar , Humanos , Hipospadias/cirugía , Lactante , Masculino , Persona de Mediana Edad , Erección Peniana , Induración Peniana/congénito , Induración Peniana/fisiopatología , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
In the last decades there is a shifting paradigm in the understanding of intersex conditions with regards to the gender assignment and the choice of the surgical technique for genitoplasty. The cosmetic and functional results of masculinizing genitoplasty are rarely assessed in the literature. We reviewed the medical literature regarding primary diagnosis, gender assignment, preoperative evaluation, goals of surgical correction, timing of surgery and surgical technique. We critically reviewed our technique of masculinizing genitoplasty in 55 patients. In conclusion, we believe that this masculinizing genitoplasty is a feasible procedure in most of the patients with ambiguous genitalia raised as males with satisfactory functional and cosmetic results.