Cloacal Exstrophy: a complex disease

Introduction Cloacal exstrophy is a rare occurrence with an incidence of 1:200,000 to 1:400,000 live births. It represents one of the most challenging reconstructive endeavors faced by pediatric surgeons and urologists. Aside from the genitourinary defects, there are other associated anomalies of the gastrointestinal, musculoskeletal and neurological systems that require a multidisciplinary approach when counseling anxious parents. Material and Methods We present a video of a patient with cloacal exstrophy treated with 21 days of life. Surgery consisted of separation and tubularization of the cecal plate from the exstrophied bladder halves and colostomy construction. The bladder was closed primarily and umbilical scar reconstructed and used for ureteral and cistostomy drainage. A urethral catheter was used to guide bladder neck tubularization. A final epispadic penis was obtained and planned for further repair in a second step. Results The patient had an initial uneventful postoperative course and immediate outcome was excellent. The bladder healed nicely but patient presented with abdominal distension in the 5th day of postoperative setting requiring parenteral nutrition. The distal colon persisted with lower diameter although non obstructive, but causing difficulty for fecal progression. Continuous colostomy dilatation and irrigation were required. Conclusions Approximating the bladder halves in the midline at birth and primary bladder closure is a viable option, intestinal transit may be a issue of concern in the early postoperative follow-up. .

Abordaje sagital posterior, una forma de manejo en descensos urogenitales / Later sagittal approach: a technical option for urogenital descent

El abordaje sagital posterior ha sido ampliamente descrito en patología Ano-Rectal; en la última década se ha extendido su utilidad en patologías urológicas complejas. En el presente trabajo se muestra la experiencia de este abordaje en pacientes con Seno urogenital (SUG). Materiales y método: Se realizó en 9 pacientes, 5 por SUG exclusivo (debido a hiperplasia suprarrenal virilizante) y 4 portadoras de cloaca. Resultados: La edad promedio de la cirugía fue de 6 años con un rango de 1 a 16 años. El largo promedio del SUG fue de 4 centímetros (2 a 6 cm.). En una paciente sólo se realizó descenso del canal común dejándolo como uretra pues no permitió el descenso vaginal, esta paciente es portadora de doble vagina y ha evolucionado con hidrocolpos a repetición por lo que ha debido ser drenado en 2 ocasiones. De las 9 pacientes en 2 no se puede evaluar continencia urinaria (1 con vesicostomía y la otra aún no adquiere continencia por edad); de las 7 restantes 2 mantienen un régimen de Cateterismo intermitente limpio, 1 paciente evolucionó con incontinencia de orina de esfuerzo que no ha sido tratado aun pues se está esperando mayor tiempo de postoperatorio (3 meses actualmente), las 4 restantes (todas portadoras de SUG) tienen continencia urinaria normal. 7 pacientes tienen continencia fecal, 2 pacientes no pueden ser evaluadas por estar aún colostomizadas. En cuanto a estenosis vaginal no se ha reoperado a ninguna paciente, requiriendo dilataciones bajo anestesia en 3 de ellas. El seguimiento promedio es de 2 años (3 meses a 5 años). Conclusión: Preferimos el abordaje sagital posterior en SUG de más de 2 cm. por que da un excelente campo quirúrgico, conserva la continencia fecal, disminuye la incidencia de estenosis vaginal y mejora los resultados estéticos. Evita además el uso de colgajos perineales y preservala continencia urinaria cuando no está asociado a cloaca.

The posterior sagittal approach has been broadly described in anorectal pathology. We report our experience with this technique for the management of patients with urogenital sinus (UGS). Materials and methods: It was carried out in 9 patients, 5 for exclusive UGS (due to congenital adrenal hyperplasia) and 4 with cloaca. Results: Mean age was 6 years (range 1 to 16 years). Mean UGS was 4cm (range 2 to 6 cm). In one patient common channel descent was performed leaving it as a urethra since vaginal descent was not feasible, this patient had a double vagina and evolved with hidrocolpos requiring drainage in 2 occasions. Of the 9 patients, 2 could not be evaluated for incontinence (1 pediatric and 1with vesicostomy). Of the remaining 7, 2 are under clean intermittent catheterization, 1 evolved stress urinary incontinence, 4 (all with of UGS) are continent. Seven patients have fecal continence, 2 patients cannot be evaluated because of colostomys. Three patients required dilation for vaginal stricture. Mean follow-up is 2 years (range 3 months to 5 years). Conclusion: We prefer the posterior sagittal approach in UGS of more than 2 cm because it provides an excellent surgical field, it conserves the fecal continence, it diminishes the incidence of vaginal strictures and it improves cosmetics. It also avoids the use of perineal flaps while preserving continence when it is not associated to cloaca.

Congenital pouch colon with imperforate anus; a report of 3 cases

Congenital pouch colon [CPC] is an unusal condition in imperforate anus [IA] usually of high variety this malfotmation is mostly reported from India, where it comprises about 7% of all anorectal malformations. In this anomaly the normal colon is very short or absent and is replaced by a pouch like dilatation that usually communicates through a fistula with genitourinary tract or persistent cloaca. We are reporting on three cases of newborns [2 females, and 1 male] with this anomaly diagnosed during September 2001 to September 2004 These three cases comprise less than 3% of all cases of IA during this time period. The two girls had anorectal agenesia, short segment of colon and a pouch with vaginal fistula and persistent cloaca [Type II]. Colon in the third case was replaced by a dilated pouch that communicated with a wide fistula to the bladder [Type I]. This is very important for pediatric surgeons to be aware of the features of this condition to allow for proper diagnosis and surgical management

Superior vesical fissure, a curious variant of exstrophy: an experience of three cases in children hospital, Pims

To present experience of superior vesical fissure. Study of management of patients with superior vesical fissure: a variant of bladder exstrophy. The Children's Hospital, PIMS, Islamabad. Patients, cases of superior vesical fissure. Superior vesical fissure is a rare variant of bladder exstrophy. There is a wide spectrum of presentation of these patients. Pubic diastasis if present is minimal. Careful assessment of the extent of the superior vesical fissure is important. Treatment is tailored for the individual patient. Operative management is much easier and satisfying as compared to bladder exstrophy in terms of continence and function