What is a Cloacal Malformation?

Females usually have three openings for the genital, intestinal and urinary tracts. In developing fetuses, these openings are connected in the cloaca sac.

Cloacal malformation, also referred to as persistent cloaca, occurs in female children where the rectum, vagina and urethra are fused, forming one common channel. In normal anatomy, these openings are separate.

What are the types of cloacal malformations?

Cloacal anomalies can differ in their presentation, but all have only one opening rather than three. This opening leads to a common area within which all three tracts converge, such as rectum, urethra, and vagina. Physicians refer to this as the common channel. The length of the common channel determines the degree of severity and correspondingly, what treatment the child will require. Common channels may be between 1 cm and 10 cm in length.

If tracts join at a low point, the baby has a short common channel. That point where three tracts join must be close to where one of them is leaving the body. So they can easily let waste leave their body. Prognostic for such children is often much better.

In more complicated cases, the three tracts unite at a higher point from the perineum. This means the common channel is long; hence, it becomes even more challenging for the waste products to pass out of the body. In such a case, the connecting channel between the rectum, vagina, and urethra is long, and the point where the three units meet is further away from the opening.

What are the symptoms of cloacal malformation?

In cloacal malformation, there is only one pathway for drainage within the perineum and not divided into a rectum and urethra and vaginally. Often, when first presenting, an initial suspicion is for an anorectal malformation because of its absent anus presentation. An initial concern in females presenting with small genitalia is that of disorders of sexual development due to an apparently oversized clitoris. At closer inspection, a single orifice will be observed in the perineum.

Other abnormalities related to cloacal malformation include:

Oesophageal atresia and tracheooesophageal fistula, which is where the lower end of the oesophagus is not formed or is joined to the trachea.
Structural abnormalities with the lower portion of the spine.
Structural abnormalities with the heart.
Kidney problems, for example, hydronephrosis, vesico-ureteric reflux, duplex kidney or ectopic ureters.

What causes a cloacal malformation?

The cause of this condition in children is not known, and it appears to happen spontaneously at birth. Researchers believe it is a random condition.

How is cloacal malformation diagnosed?

Suspicion of cloacal malformation may be present antenatally with routine ultrasound studies showing bilateral hydronephrosis; these are swollen kidneys filled with urine that can't be drained down properly. The bladder does not outline very well and there may also be a cystic swelling within the abdomen. Once this has been suspected, follow-up ultrasound studies are then repeated during the rest of pregnancy to follow up the abnormality.

Cloacal malformation may be suspected soon after birth if meconium, dark faeces passed in the first few days of life are not passed or come from a different place. Physical examination will confirm the diagnosis. Imaging scans, such as ultrasound and echocardiogram, will be used to identify any associated problems.

How is cloacal malformation treated?

When a baby is diagnosed with cloacal malformation, surgeons review the internal anatomy and identify any defects associated with the condition. Doctors begin procedures to let a baby's body function appropriately within the first 24 to 48 hours after birth. Some of these procedures may involve the following:

A colostomy so that poop can pass
Sometimes, the fluid built up in the vagina needs to be drained in a procedure called hydroscopes.
After six months and when the child is stabilized and can, from her body, pass urine and stool, the family will see the surgical team again for discussion on treatment strategy.
Anatomy can vary widely in cloacal malformation, so the complexity of the posterior sagittal anorectal vaginal urethral plasty varies.

Can cloacal malformation be prevented?

No, there is nothing you could have done to prevent a cloacal malformation and nothing you did to cause it. It's a pretty rare occurrence and happens randomly.

What can I expect if my child has a cloacal malformation?

These outcomes are usually good. Babies with milder cases, where the common channel was short, have about a 90% chance of having completely normal urinary and rectal function with good control. This means they don't leak pee or poop.

Babies with more complex malformations or larger common channels have about 70% chance of bowel and urinary control. Eventually, most babies can manage normal bowel movements. Less common but still a problematic issue is urinary incontinence (pee leakage). Depending on the degree of incompetence, some children will be required to have a catheter tube to assist drainage of the bladder several times a day.

Most individuals with this condition can lead an active sexual life at the time of maturity.

What questions should I ask my child's provider?

Getting a diagnosis that your child has a malformation might raise many questions in your mind. Some of the questions you may ask the health care provider of your child include:

How do you conduct the surgeries to correct the malformation? When are the surgeries performed?
Is my child going to be hurt? Are they being hurt now?
What should I expect as my child heals?
Will my child need additional surgeries as they mature?
Will my child experience issues with their anus, urethra or vagina as they grow?
Will my child be able to get pregnant and have a baby safely?

Why Choose Tender Palm Super Speciality Hospital for Cloacal Malformation treatment in Lucknow, India?

Tender Palm Super-Speciality Hospital has the most trusted team of Pediatric Urologists with advanced diagnostic equipment and child-friendly care for Cloacal Malformation treatment in Lucknow, India. Our Pediatric Urology department follows international safety standards and has years of experience in successfully managing children's Urological diseases.