Cloacal repair - long common channel with a short urethra - the urogenital separation technique

A cloaca is the rarest type of all of the anorectal malformations

A cloaca presents with a single perineal orifice and no anal opening

It is important to distinguish cloaca from a patient with a urogenital sinus with a normal anus, as such a patient would need an endocrinologic workup

A small single orifice lying just under the clitoris is more typical of a long common channel cloaca, while a larger orifice is more typical of a lower confluence

Patients with cloaca may develop hydrocolpos with urine entering the vagina

The vagina can fill and compress the distal ureters, leading to hydronephrosis

The Mullerian system is duplicated about half of the time in cloacal patients

Cloacal care requires a collaborative approach including partners such as radiology, anesthesia, neurosurgery, nephrology, and nursing

The traditional description of cloaca divided patients into two types: those with a short common channel (less than 3 centimeters) and those with a long common channel (3 centimeters or greater)

A more modern approach is to note both the length of the common channel as well as the length of the urethra

The measurements of common channel and urethral length predict what operation is required

If the common channel is less than 3 centimeters and the urethra at least 1.5 centimeters in length, a total urogenital mobilization can be done

If the common channel is 3 centimeters or longer and the urethra short, a urogenital separation is needed

The urogenital separation technique requires repair where the vagina connected to the common channel

The sphincter complex is delineated using bipolar cautery

Epinephrine soaked gauze is a helpful maneuver to manage spongy osome tissue bleeding

The coccyx is excised to improve exposure

The common channel is intentionally left intact and opened high at the vagina because the common channel will become the neourethra added to the native urethra

An ischiocrectal fat pad is mobilized to cover the repair of the common channel

Rectal mobilization is performed by staying intimately close to the rectal wall, preserving the intramural blood supply provided by the IMA and its branches onto the wall of the rectum

The rectum reaching to 4 centimeters below the superior aspect of the pubic bone means it will comfortably reach the anoplasty at the perineum

The ureters come in laterally and need to be checked for during vaginal mobilization