[size=20px]A former 38-week gestational age infant girl with a cloacal malformation underwent a divided sigmoid colostomy in the newborn period. Now eight months of age she presents for possible future surgical intervention. A previous cystoscopy and vaginoscopy showed a common channel with a length of one centimeter.

 

The next operation for this patient with cloaca is

 

A no further operation should be recommended.

 

B closure of sigmoid colostomy alone.

 

C laparoscopic urogenital mobilization.

 

D perineal total urogenital mobilization and anorectovaginourethroplasty.

 

E abdominoperineal mobilization of the vaginal, urinary and anal opening.[/size]

(Untitled)

 

D

D

correct

 

A cloacal anomaly is the persistence of a single confluent channel whereby the rectum, vagina and urethra terminate. It occurs exclusively in girls with an incidence of one in 20,000 live births. In dealing with babies with a cloacal malformation, physicians have to consider preservation of bowel, urinary and sexual functionality.

 

Those with a common channel less than three centimeters can be managed with total urogenital mobilization from a perineal approach while longer lesions may also require an combined abdominoperineal approach. In this case, laparoscopic mobilization would not be needed. The complexity of surgical correction and the functional results of the three systems involved have been related to the length of the common channel; those with common channel greater than three cm have a more complex operation and a worse outcome. In Pena’s series, voluntary bowel movements were achievable in 66% of patients with a channel less than three cm, and voluntary urinary control was seen in 73% of patients. In patients with common channels greater than three cm, urinary control was seen in only 26% and voluntary bowel function was achieved in only 34% of patients. The diverting stoma should be closed after the perineal repair has healed and serial dilations of the neoanus have achieved an appropriate size orifice.

 

Gynecologic function is less well studied. In a longitudinal follow-up of 41 postpubertal patients (mean age 16.8 years), 68% had uterine function, 32% were menstruating normally and 36% required surgery (hysterectomy, partial hysterectomy with vaginoplasty and vaginoplasty) for an obstructed uterus. In this series, there had been no pregnancies to date. These patients should be assessed at puberty with ultrasound or magnetic resonance imaging and vaginoscopy as they start menstruation and sexual activity.