Colorectal Quiz Episode 17: Cloaca Part 1
With Dr. Mark Leavitt & Dr. Richard Wood · hosted by Dr. Amanda Jensen & Dr. Kira Ahmad · Marc Levitt
Cued at 5:41 · stops at 6:26 · press play
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
A single perineal orifice in a newborn should clue you into a cloaca, where the vagina, urethra, and rectum are fused together inside creating a single common channel
Hydrocolpos is the distension of the vagina caused by the accumulation of fluid
A cloaca or anorectal malformation is associated with VACTERL and needs to be worked up as such
VACTERL association consists of vertebral anomalies, imperforate anus, cardiovascular anomalies, tracheoesophageal fistulas, esophageal atresia, renal or radial anomalies, and limb defects; three or more anomalies are needed for the association
A hydrocolpos on prenatal ultrasound should alert to the possibility of a cloaca
The diagnostic yield for cloacal malformations on prenatal ultrasound is still much lower than desired
Abnormal kidneys, a single kidney, or a two-vessel cord on prenatal ultrasound can be associated with cloaca
In a large majority of patients with cloaca, the diagnosis is made at birth
Children with cloacas are female and do not need workup for karyotyping or disorders of sexual differentiation, despite potentially prominent clitoral hood and labial tissue
Initial workup for cloaca should consist of NG tube and chest x-ray, cardiac echo, and pelvic and renal ultrasound to assess for TEF, cardiac anomalies, hydrocolpos, and hydronephrosis
If bilateral hydronephrosis and hydrocolpos are present, the hydrocolpos needs to be managed as part of initial treatment
Modern practice has moved away from routine vaginostomy toward clean intermittent catheterization through the common channel for hydrocolpos drainage
To perform CIC for hydrocolpos, pass a tube through the common channel, drain fluid, confirm tube placement in the hydrocolpos with ultrasound, then perform recurrent catheterization
When catheterizing the common channel, you are more likely to get into the vagina than the bladder due to the anatomy of the urethral takeoff
Initial catheterization should be performed with bedside ultrasound confirmation that the tube is in the hydrocolpos and is decompressing it
When the hydrocolpos is drained, the bladder fills, demonstrating that the hydrocolpos was obstructing the ureters
A vesicostomy is the wrong move in almost every cloaca; the hydrocolpos needs to be drained instead
Initial catheterization frequency is three times daily, which can be reduced to twice daily when families begin performing it at home
Serial ultrasounds should be performed every 2-3 days initially, then stretched to weekly, to confirm adequate kidney decompression
After discharge, monthly ultrasounds should be performed to ensure continued kidney decompression, which can be stretched to every six weeks if doing well
Even with a vaginostomy tube, you must continue checking that the kidneys are decompressed, as the tube may not be doing its job
Colostomy should be performed as proximally as possible, at the descending-sigmoid junction, to ensure enough length for distal work
Laparoscopy for newborn colostomy formation provides good anatomical views of the pelvis, allows precise colostomy site selection, and avoids a wound between the two stomas
If a vaginostomy is needed in a patient with a vaginal septum, open the anterior wall of the hydrocolpos and remove a small portion of the septum to drain both sides through one hole
Vaginostomy tubes can become encrusted and colonized, so there is some advantage to tubeless vaginostomy
For massive hydrocolpos requiring open vaginostomy, use a lower midline incision to get above the hydrocolpos, which is very adherent to the anterior abdominal wall and inflamed
A single perineal orifice with no anal opening is a cloaca and does not need an endocrine workup, whereas a perineal opening with a normal anus is a urogenital sinus and does need an endocrine workup
Urogenital sinus patients can also have hydrocolpos and hydronephrosis, requiring similar management but without colostomy
After initial management, follow patients closely to ensure kidneys are well decompressed, the baby is growing and thriving, and parents are managing the stoma
With urine and stool effectively drained, patients should be thriving unless other underlying issues are present