Case Presentations Part II: Cloaca and Complex ARMs 2015

In high common channel cloacas, the common walls between the rectum, vagina, and bladder are not long and are easier to separate through the abdomen than posterior sagittally.

Saline perturbation is used intraoperatively to assess the patency of the reproductive tract in cloaca repairs.

When assessing bilateral Müllerian structures, if one side is well developed and the other very atretic, it may be advantageous to retain the well-developed side and remove the atretic side to avoid menstrual complications and preserve childbearing potential.

Colon used for vaginal interposition grafts is placed anti-peristaltic due to blood supply considerations and the way it reaches the perineum.

Using the rectum for vaginal replacement may change a patient who is potentially fecally continent into one who is not, because removing the rectal reservoir impairs continence.

In patients with good functional prognosis (good sacrum, no tethered cord, good malformation), preservation of the rectum is extremely important for bowel control.

Doctor Pena found that patients with vestibular fistulas and absent vagina who had their rectum used as a vagina (with colon brought down as rectum) had less than optimal bowel control in long-term follow-up.

The rectum is a natural reservoir, and removing it in patients with anorectal malformation makes the patient likely to be incontinent because they cannot tolerate constant stool passage when colon is connected directly to the perineum.

Descending colon is increasingly used for vaginal replacement because the arcades are favorable for preserving blood supply, compared to small bowel or rectum.

In approximately 65 cases of cloacas that came to Doctor Pena's center after initial colostomy elsewhere, babies with phallic-appearing structures were diagnosed as intersex and extensively studied, only to be found chromosomally normal females with cloacas.

Doctor Pena has never seen a patient with both intersex and cloaca.

A patient with a pseudophallus and single perineal orifice can be differentiated from adrenal hyperplasia by palpation: in true adrenal hyperplasia, real corpora (penis) will be palpable, whereas in cloacas the phallic structure is just folded skin.

The folded skin of the phallic structure in cloacas can be used to facilitate vaginal reconstruction.

Families of patients with cloacas misdiagnosed as intersex agonize during the 2-3 weeks doctors spend trying to make the intersex diagnosis.