Cloacal malformations
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 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 pediatric colorectal surgeons, urologists and gynecologists use 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?
Immediate care for a newborn with cloaca will involve draining urine and performing a colostomy to allow stool to pass from the body. Once the infant is stable, a thorough diagnosis will be conducted. The medical team may recommend an MRI to capture detailed images of the spine and pelvis. An endoscopic examination, typically done after the neonatal period, may also be suggested to give the team a clearer anatomical view of the malformation. Based on the findings from these tests and assessments, a personalized treatment plan will be developed, tailored to your baby’s specific condition, and will include surgical reconstruction.
How will my child recover after surgery for cloaca?
Recovery following reconstructive surgery for cloacal malformation usually requires several days or more in the hospital. Your child will need to be carefully monitored to ensure that the urethra, vagina, and rectum are healing properly.
As with all anorectal malformations, surgery will restore some function, but the nerves and muscles controlling bowel movements may remain weak. Children who undergo surgery for anorectal malformations might experience fecal incontinence or constipation, which can be effectively managed through our bowel management program. Some patients may need to catheterize the urinary system to ensure proper bladder emptying. Your medical team will explain what the recovery process will involve for your child.
For those who require it, the bowel management program can optimize bowel function, combined with a fiber-rich diet to combat constipation. COCOE offers a bowel management program that includes a week-long outpatient program, followed by continued appointments as needed until the child can stay clean and dry. This program is customized based on the patient’s diagnosis, age, symptoms, medical history, and objectives. It is available 8-10 times a year.
What long-term care will my child need after recovery from cloaca?
Children born with cloacal malformations require ongoing, long-term follow-up care, as this condition can heighten the risk of certain urological, gynecological, and colorectal issues. For instance, some patients may face challenges like constipation, urinary tract infections, and fecal or urinary incontinence. If not promptly addressed, these issues can lead to more severe health problems. Any established treatment plan should be regularly reviewed and adjusted as necessary. Some patients might also require additional surgeries later in life. Assessing patients at puberty is essential to ensure that the gynecologic anatomy supports normal menstruation.