Pediatric Cloacal Malformations

The Pediatric Colorectal & Pelvic Reconstruction Program team at Children's National Hospital has the world's greatest volume of experience in surgically treating even the most complex cloacal malformations, and specializes in the repair of colorectal, urologic and gynecologic organs.

What are cloacal malformations?

A cloacal malformation is a type of anorectal malformation that occurs in a female fetus when the rectum, vagina and urinary tract form a single channel known as a cloaca, rather than three separate openings. The severity of the condition depends on where the three tracts have joined together. This type of malformation happens when the embryonic anorectal and urogenital channels fail to separate during the sixth and seventh weeks of gestational development. Cloacal malformations are rare; they only occur in approximately 1 in 50,000 live births. 

 

What are the different types of cloacal malformations? 

Cloaca with a short common channel

The most common type of malformation. There is a good length urethra. This can be repaired using a posterior sagittal incision only to reach urethra, vagina and rectum. For the urethra and vagina, a total urogenital mobilization would be employed.

 

 

 

Cloaca with a long common channel

There is a short urethra. Repairing it requires separation of the vagina from the common channel, and the common channel then becomes the urethra. The rectum is also quite high. This type of repair requires a posterior sagittal approach as well as an abdominal approach, using either laparoscopy or a laparotomy.

 

 

 

 

Cloaca with a very high rectum

There is a low confluence and short common channel. In this case, a total urogenital mobilization can be employed, and for the rectum, laparoscopy would be needed.

 

 

 

 

Cloaca with hydrocolpos

This occurs in about one half of all cloacas. The urine, rather than passing through the common channel, goes back into the vagina, dilating it. And the large vagina can compress the ends of the ureters and cause hydronephrosis (stretched/swollen kidneys).

 

 

 

 

Cloaca with two hemivaginas

This occurs in about half of all cloacas – these patients have a duplicated gynecologic (Mullerian) system.

 

 

 

 

What is a cloacal exstrophy?

Cloacal exstrophy is one of the most complicated and severe forms of cloacal malformations. It occurs when a baby is born with several organs that are exposed, such as the bladder or large intestine, or when certain organs protrude from the navel (called an omphalocele).

What are the symptoms and complications of cloaca in children?

Newborn babies with cloacal malformations do not have visible anuses. Urine usually successfully passes, but it is mixed with vaginal mucous and stool.  They also may have symptoms or complications such as:

• Abdominal swelling
• Enlarged kidneys
• Having only one kidney
• Hydrocolpos—a condition causing fluid to back up in the vagina and uterus, which can cause a blockage of the ureters
• Urine that backs up into the kidneys (reflux)
• Problems with function of gynecologic organs that may persist into adulthood

How are cloacal malformations diagnosed in children?

Cloacal malformations can be very complex and require multidisciplinary treatment from several different specialists. Children diagnosed with this condition may also have anomalies in other organ systems. 

In some cases, doctors may be able to diagnosis the malformation in utero. However, in most cases, making an accurate diagnosis requires a physical examination and various diagnostic tests after the baby is born. Our expert pediatric colorectal surgeons, urologists and gynecologists use the latest diagnostic tests and tools to evaluate for all the possible defects that could be related to your child’s condition.

When evaluating a newborn with a suspected cloacal malformation, the following diagnostic tests may be used:

• Abdominal ultrasound: to examine the kidneys, bladder, gynecologic system and pelvic organs
• Spinal ultrasound: to evaluate for spine abnormalities or other neurological problems such as tethered cord or myelomeningocele
• X-rays: to examine the spine and sacrum

Immediate treatment of the newborn will entail drainage of the urine and a colostomy to allow for stool to exit the body. Once the baby has stabilized, a comprehensive diagnosis will be completed. The medical team may order an MRI to obtain high-definition images of the spine and pelvis. An endoscopic examination, taking place after the neonatal period, may also be recommended as it allows the team to attain a more detailed anatomical understanding of the malformation. Based on these test and examination results, the medical team will create an individualized treatment plan unique to your baby’s specific condition that will involve surgical reconstruction. 

How are cloacal malformations treated?

This occurs in about one half of all cloacas. The urine, rather than passing through the common channel, goes back into the vagina, dilating it.  And the large vagina can compress the ends of the ureters and cause hydronephrosis.How will my child recover from surgery for cloaca?

Recovery from cloacal malformation reconstructive surgery will typically require several or more days in the hospital. Your child will need to be closely supervised to make sure the urethra, vagina and rectum have adequately healed. 

As with all anorectal malformations, surgery will restore some function, but the nerves and muscles that control the bowels may still be weak. Children who have had surgery for anorectal malformations may experience fecal incontinence or constipation, but this can be well managed using our bowel management program. Some patients require catheterization of the urinary system to ensure good emptying of the bladder.  Your medical team will discuss what recovery will entail for your child.

For patients who need it, the bowel management program can help facilitate optimal bowel function in combination with following a healthy diet rich in fiber to combat constipation. Children’s National’s bowel management program consists of a week-long outpatient program followed by ongoing appointments (as needed) until the child can remain clean and dry. The program is individualized based on the patient’s diagnosis, age, symptoms, medical history and goals. It’s offered 12 to 15 times per year.

What long-term care will my child need for recovery from cloaca? 

Children born with cloacal malformations require long-term, continued follow-up care as this condition can increase the risk of certain urological, gynecological and colorectal conditions. Some patients, for example, may experience constipation, urinary tract infections and fecal and urinary incontinence. If not treated promptly, these conditions can result in more serious medical problems. Any established treatment regimen should be periodically evaluated and adjusted as necessary. Some patients may also need additional surgeries later in life.  Assessment of patients at the age of puberty is vitally important to ensure that the gynecologic anatomy allows for expected menstruation.

Around the ages of 11 and 12, patients and their families should start planning a transition to an adult colorectal surgeon experienced in treating patients living with anorectal malformations. Taking a proactive approach as children enter adolescence is important as many patients can develop new problems at this stage, which can impact their quality of life. Adolescents with this condition may also develop issues with body self-image and sexual function and may benefit from psychological support. Having a planned transition can empower young adults to take a larger role in managing their own condition, which can result in life-long health benefits.

As Children’s National patients become adults, their needs will evolve and change over time. Some adult patients who were treated for cloacal malformations as children may experience infertility, obstetric challenges, kidney disease or renal failure, among other complications. For these reasons, it’s critical that patients seek and receive ongoing care for their condition throughout their lives.