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Pediatric Cloacal Malformations
The team at the Children’s National Colorectal Program has the world's greatest volume of experience in surgically treating even the most complex cloacal deformities, and specializes in the repair of colorectal, urologic and gynecologic organs.
A cloacal malformation is a type of anorectal malformation that occurs when the rectum, vagina and urinary tract form a single channel rather than three separate openings in newborn girls. The severity of the condition depends on where the three tracts have joined together.
Many newborn girls with cloacal malformations experience other complications including defects of the urinary and gynecologic organs.
One of the most complicated and severe cloacal deformities is called a cloacal exstrophy. This occurs when a baby is born with several organs that are exposed, such as the bladder, large intestine or when certain organs protrude from the navel (called an omphalocele).
Newborn babies with cloacal malformations do not have visible anuses. They also may have symptoms or complications such as:
- Abdominal swelling
- Enlarged kidneys
- Having only one kidney
- Hydrocolpos, a condition where fluid has backed 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
Cloacal malformations can be very complex and require multidisciplinary treatment from several different specialists. 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.
These diagnostic tests include:
- Abdominal ultrasound: to examine the kidneys, bladder and pelvic organs
- Spinal ultrasound: to evaluate for spine abnormalities or other neurological problems
- 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 and a comprehensive diagnosis has been made, the medical team will create an individualized treatment plan unique to your baby’s specific condition that will involve surgical reconstruction.
Cloacal malformations will likely require several different types of reconstructive surgeries. Surgery is performed through the perineum and sometimes through the abdomen and depending on the severity of the condition, may involve urinary or vaginal reconstruction or replacement. Bowel reconstruction will involve a technique called posterior sagittal anorectoplasty (PSARP), also known as the “pull through” procedure, that repairs the rectum and anus. A vaginoplasty (repair of the vagina) and urethroplasty (repair of the urethra) are also performed. Your child may require a colostomy during the healing process. A colostomy entails making a small incision in the large intestine that diverts the colon to excrete waste into a small bag located outside of the body. A colostomy is a temporary measure used to allow the intestines time to heal. This is usually created in the newborn period many months before the cloacal repair. Once all reconstructive surgeries have been performed and you child has healed, a colostomy closure may be performed to allow them to pass stools successfully on their own.
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. For several months after surgery, anal dilations may need to be performed to help stretch the anus to allow healthy bowel function.
As with all anorectal malformations, surgery will restore some function, but the nerves and muscles that control the bowels may still be weak. For patients who need it, a bowel management program helps facilitate optimal bowel function in combination with following a healthy diet rich in fiber to combat constipation. Your medical team will discuss what recovery will entail for your child.
Watch the video
Management of Cloacal Malformations – What is New in 2021?
Listen as Marc Levitt, M.D., who serves as Division Chief of Colorectal & Pelvic Reconstruction, discusses the new ways to manage cloacal malformations.
The Division of Pediatric Colorectal & Pelvic Reconstruction offers the latest advancements in diagnosis and treatment for all types of pediatric colorectal disorders.