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. .

Total urogenital mobilization by posterior sagittal approach and ileal neovaginoplasty in cloacal repair by a common channel of 4 cm.

OBJECTIVE: The total urogenital mobilization (TUM) technique avoids dissection of the vagina from the urinary tract. However, in the case of a long common channel, bowel interposition for vaginal replacement may be necessary. We present a video of the TUM technique in cloacal repair with ileal neovaginoplasty. METHODS: An 8-month-old female patient with cloaca, presenting a 4-cm common channel, was operated on by the TUM technique, through a perineal sagittal with additional abdominal approach. She had undergone decompressing colostomy at birth. Total urogenital sinus mobilization was performed with ileal neovaginoplasty. RESULTS: The patient had a good postoperative outcome, an endoscopy of all structures reconstructed was performed at PO day 30, and home dilatation of colon and vagina by parents was initiated. CONCLUSION: TUM with neovaginoplasty bowel interposition is an effective technique for repairing long common channels.

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.