Hipospadias: del diagnóstico ecográfico prenatal al manejo posnatal / Hypospadias: from prenatal ultrasound diagnosis to postnatal management

El hipospadias es la localización anormal del meato urinario y es la malformación de genitales externos más frecuentemente diagnosticada. El diagnóstico prenatal es posible mediante ecografía sistemática desde la semana 20 de gestación, siendo más fácil su diagnóstico en el tercer trimestre. Las formas leves suelen ser aisladas, familiares o asociadas a disfunción placentaria o restricción de crecimiento intrauterino, mientras que las formas más graves presentan hasta un 30% de asociación a defectos fetales, anomalías cromosómicas/genéticas o anomalías del desarrollo sexual. La tríada para el diagnóstico ecográfico prenatal consiste en curvatura ventral del pene, anomalía del prepucio dorsal y punta del pene roma. La valoración de la uretra durante la micción y el aspecto del chorro miccional son de gran utilidad para clasificar el defecto. Cuando se diagnostica hipospadias peneano o escrotal es aconsejable realizar una amniocentesis para estudio genético fetal y valorar otros signos de adecuada virilización, como el descenso testicular a partir de la semana 27. El seguimiento tras el parto debe ser multidisciplinario, incluyendo urólogo y endocrinólogo infantil. En hipospadias leves el pronóstico es bueno con reparación quirúrgica en el primer año de vida, pero las formas graves pueden presentar un reto mayor para su corrección funcional y estética.

Hypospadias refers to the abnormal location of the meatus; it is the most common genital malformation detected in the fetus and newborn. Prenatal diagnosis is feasible from 20 weeks onwards with routine ultrasound; however, it is easier to diagnose during the third trimester of pregnancy. Mild defects are usually isolated, familiar o related to placental disfunction or intrauterine growth restriction, while the severe hypospadias are associated to other fetal defects, genetic or chromosomal abnormalities or disorders of sex development. In about 30% of cases. The triad of ultrasound findings prenatally is ventral curvature of the penis, redundant dorsal foreskin and blunt distal penis. The identification of the urethra during the micturition and the direction of the urinary stream help in the classification of the defect. When severe hypospadias is detected, the recommendation is to perform genetic amniocentesis and search for other ultrasound findings related to poor virilization in the fetus, as testicular descent after 27 weeks of gestation. Postnatal follow up should be multidisciplinary including infantile urologist and endocrinologist. The prognosis in distal hypospadias is usually good following surgical repair, however in severe cases surgical interventions may be more challenging in order to obtain satisfactory outcome in terms of function and esthetic.

Hipospadias: Enfoque molecular y endocrinológico / Hypospadias: molecular and endocrinological focus

Las hipospadias son el arresta en el desarrollo ventral del pene. Puede ser una malformación que se presenta de forma aislada o asociada a otras alteraciones como lo es la presencia de una curvatura ventral anormal del pene, criptorquidia, hernias inguinales, entre otras. En todos los casos es importante considerar las hipospadias como un grado leve de feminización. Actualmente no existe prevención para el desarrollo de las hipospadias y el único manejo es la corrección quirúrgica de la lesión. Grandes avances se han logrado en el estudio de los procesos moleculares asociados en el desarrollo de las hipospadias. Los receptores androgénicos (RA), alteraciones endocrinas se han postulado como fenómenos influyentes en el desarrollo de ésta patología. El objetivo del presente artículo es determinar los eventos moleculares asociados en el desarrollo de las hipospadias.

Hypospadias repair

The Departments of Urology, Pediatric Surgery and Plastic Surgery, Quaid-e-Azam Medical College, Bahawal Victoria Hospital, Bahawalpur. Jan 1999 to Dec 2004. Prospective. Patients admitted with hypospadias in these departments were included in this study except patients with multiple failed repairs previously. Standard procedures were practiced for every type of defect i.e. MAGPI and Mathieu's repair for coronal hypospadias, Snodgrass urethroplasty for proximal and distal penile hypospadias. The age range observed during this study was 1.5 to 25 years while 64% of patients were less than 10 years of age. The type of defect was coronal in 25%, penile in 60%, penoscrotal and perineal in 15% of the patients. Initial success rate was 78% and overall success rate was 92%. Complications observed were fistula formation 7%, stenosis of anastomotic site 7% and dehiscence of repair 3%. Thorough evaluation of urethral and penile malformation brings best outcome of surgery for hypospadias. Hypospadias repair should be offered to the child before school going age so as to prevent psychological impacts of genital malformations

One stage repair of severe hypospadias: Original versus modified koyanagi technique

Although the original Koyanagi technique seemed applicable for the repair of severe hypospadias at or proximal to the penoscrotal junction, its use has resulted in a high complication rate. The technique was modified to ensure better vascularity of the flaps. The purpose of this study is to report the results of original Koyanagi technique and its modifications in patients with severe hypospadias. The original Koyanagi parameatal prepuital flap technique was performed in 16 boys with severe hypospadias [group I]. In a subsequent 11 patients, the modified Koyanagi technique was used to preserve blood supply to the flaps [group II] The meatus was located at or proximal to the penoscrotal junction in all patients. Moderate to severe degrees of chordee was noted in all of them. The median age of patients was 13 and 20 months at time of repair for group I and II respectively. Each patient was evaluated as regard to site of the new meatus, straightness of the phallus, and stream of urine, development of fistula, urethral or meatal stenosis, any other complication, and the need for another operation. Follow up ranged from 36 to 84 months in group I, and 3 to 36 months in group II. A fistula developed in 8 of the 27 patients [29.6%] The frequency of fistula was more common in group I [6116] than in group II [2/11]. Urethral diverticulum occurred in one patient in group II, and meatal stenosis in 2 in group I. Significant infection resulted in a regressed meatal position in 2 [one in each group]. Good cosmetic results were achieved in all except the latter 2 cases. Secondary operations were needed in 8 patients [6 in group I and 2 in group 2] The indication for secondary surgery were closure of persisted urethrocutaneous fistula in 6 patients [5 in group 1 and 1 in group 2] and regressed meatus in 2 [one in each group]. A single staged repair can be safely and effectively performed even in patients with the most severe penoscrotal hypospadias. The modified Koyanagi technique has relatively lower complication rate than original Koyanagi repair. The complication rate is acceptable considering the severity of these cases

Glanular closure during the tubularized incised plate procedure: a prospective randomized study

Hypospadias is reported in 1:200 live births. The introduction of the tubularized incised plate [TIP] procedure has revolutionized the treatment of hypospadias. The method of glanular closure during the TIP procedure, whether in single or double layers, has not been evaluated in the literature. To evaluate the optimal technique of glanular closure during the TIP repair of the distal hypospadias. Sixty-six infants and children with distal penile hypospadias were treated primarily with the TIP procedure. Age ranged between 9 months and 4 years with a mean age of 20.8 months at time of repair. Patients were randomly subdivided into two equal groups. In group A, the glans was repaired in a single layer, while in group B, the glans was closed in two layers. All cases were subjected to the same protocol of urethral stenting, penile bandage, and catheter removal by the third postoperative day. Both groups were followed up and compared with regard to the results of TIP repair and the incidence of complications. In group A, two cases developed glanular disruption versus none in group B. this was found statistically significant [p<0.05]. No statistical difference was found in between groups with regard to meatal stenosis, fistula formation, or in the overall cosmetic and functional outcomes. Medium-term follow up showed excellent results in 82.9% while good results were obtained in 17.2%. On the mid-term follow up, the TIP procedure continues to prove itself as the gold standard in treatment of the distal hypospadias. The double-layered glanuloplasty seems to hold an improved morbidity profile, with significantly lower glanular disruption rate, when compared to the single layered repair. Large-numbered studies are still needed to provide further evidence for the superiority of the double-layered glanuloplasty.

Resultados post-operatorios de la corrección de hipospadias: Hospital Padre Justo-Rubio años 1986-1996 / Post-operaties results of the hypospadias of correction: Hospital Padre Justo-Rubio years 1986-1996

Se realizó un estudio retrospectivo, analizando los resultados quirúrgicos de 29 niños operados de hipospadias entre 1986-1996 del Hospital Padre Justo de Rubio, se señalan las diferentes técnicas quirúrgicas empleadas, los grupos etareos; tipo anatómico de hipospadias y resultados post-operatorios de la cuerda y neoretar. Nuestro estudio demostró que las hipospadias distales son las más frecuentes; el mayor número de intervenciones se realizó en el grupo etáreo de 1 a 2 años, las técnicas más utilizadas, MAGPI; y movilización con avance uretral para las Hipospadias Distales y Subcoronales. La técnica de Nomomura para las proximales, Duckett y Devine Horton para las mediopeneanas. La complicación post-operatoria más frecuente es la estrechez uretral con un alto porcentaje por la técnica de avance y bajo con la uretroplastia de MAGPI Las fístulas uretrocutáneas ocupan el segundo lugar y su incidencia es alta con técnica de Nomomura. Los resultados post-operatorios de la neouretra fueron excelentes con MAGPI; regular con avance uretral y poco satisfactoria con Nomomura. La corrección de la cuerda peneana excelente con la diferentes técnicas empleadas. Finalmente la mayoría de los casos ameritaron dilataciones uretrales sucesivas y cura de fístula