Estrategia terapéutica en la extrofia de cloaca femenina / Therapeutic strategy in cloacal exstrophy

La extrofia de cloaca femenina enfrenta al cirujano pediá- trico con un reto en el que está implicada la reconstrucción del tracto genitourinario y digestivo así como su manejo funcional. El objetivo de nuestro trabajo es presentar nuestra experiencia en el manejo de las malformaciones genitales en niñas con extrofia de cloaca así como de la continencia urinaria y fecal. Revisamos las historias clínicas de las pacientes tratadas en nuestro centro en las que se ha llegado a una corrección definitiva del espectro malformativo de la extrofia de cloaca. En las tres pacientes se ha realizado un cierre del cuello vesical y un estoma cateterizable continente a través del cual llevan a cabo un programa de autocateterismo vesical intermitente, manteniéndose secas. Con respecto a la integración psicosexual, las tres pacientes están satisfechas con el resultado cosmético obtenido, aunque sólo una de ellas mantiene relaciones sexuales. El manejo de la extrofia de cloaca femenina debe tener en cuenta tanto los factores anatómicos como funcionales. La reconstrucción genital en niñas con extrofia de cloaca requiere un plan ajustado en cada paciente y en ocasiones realizar varias intervenciones hasta que la paciente esté satisfecha con el resultado tanto cosmético como funciona (AU)

Cloacal exstrophy is a complex multisistemic anomaly thatinvolves gastrointestinal and genitourinary issues. The aim of our paper is to report our experience in dealing with genital reconstruction and faecal and urinary continence in patients with female cloacal anomalies. We reviewed the clinical records from the three patients we have achieved a final reconstruction. We recorded the surgical management and both functional and anatomic results. The three of them required a bladder neck closure associated with a continent stoma, they are dry with intermittent catheterization and free of upper urinary complications. Genital reconstruction required a unique plan for each one, according to their anatomy and their cosmetic desires. One of the patients reported satisfactory sexual intercourse. Management of patients with cloacal exstrophy has major concerns about urinary and fecal continence and about genital reconstruction and function. Knowing the long-term results may help to develop management strategies and improve counselling for patients who have undergone reconstruction (AU)

[Therapeutic strategy in cloacal exstrophy]. / Estrategia terapéutica en la extrofia de cloaca femenina.

Cloacal exstrophy is a complex multisistemic anomaly that involves gastrointestinal and genitourinary issues. The aim of our paper is to report our experience in dealing with genital reconstruction and faecal and urinary continence in patients with female cloacal anomalies. We reviewed the clinical records from the three patients we have achieved a final reconstruction. We recorded the surgical management and both functional and anatomic results. The three of them required a bladder neck closure associated with a continent stoma, they are dry with intermittent catheterization and free of upper urinary complications. Genital reconstruction required a unique plan for each one, according to their anatomy and their cosmetic desires. One of the patients reported satisfactory sexual intercourse. Management of patients with cloacal exstrophy has major concerns about urinary and fecal continence and about genital reconstruction and function. Knowing the long-term results may help to develop management strategies and improve counselling for patients who have under-gone reconstruction.