Cloacal Deformities | Treatments

What are the treatment options for cloacal deformities?

Cloacal deformities require surgical repair. The treatment plan devised for your child will depend on the type and extent of the abnormality.

Stabilizing your newborn

Before forming a treatment plan to correct a cloacal deformity, your doctor’s immediate concern will be to stabilize your baby’s condition. This may involve the creation of a diverting colostomy to allow the passage of stool. In a colostomy, the large intestine is divided into two sections and the ends of the intestine are brought through surgically created openings (stomas) in the abdomen. The upper section allows stool to pass into a collection bag, while the lower section allows for drainage of mucus produced by the intestine.

The urinary bladder may also need to be decompressed to relieve obstruction of urine flow from the bladder and, at times, the kidney. Some children will be able to void urine on their own, but for others, intermittent catheterization may be needed to help eliminate urine. The vagina, if it develops an accumulation of fluid called hydrocolpos, sometimes also needs to be decompressed with a drain.

Surgical correction

After stabilization, and when your baby has had a chance to grow, our doctors will confirm the anatomic features of the deformity and make a plan to correct it. Treatment typically involves the surgical creation of a urethra and vagina. In some children with less severe deformity, the urethra and vagina are intact and do not need to be created, but simply have their openings brought to the skin surface. Finally, a reconstruction of the bowel, through a procedure called a "pull-through" of the colon, must be completed. For this procedure, the surgeon may have to open the abdomen to complete the connection of the colon to the rectum. This procedure is sometimes called a posterior sagittal anorectovaginourethroplasty (PSARVUP).

Follow-up and further surgery

Once your child has healed, a third operation will be performed to close the colostomy and reestablish normal bowel movements through the rectum. Further urinary or genital tract surgery may also be needed. At the time of colostomy closure, the team will also examine the urethra, vagina and rectum for adequate healing.

Sometimes, depending on the malformation, more urologic reconstruction will be needed in the future. Your child’s urologist will decide if this is necessary.

What is the long-term outlook for a child with a cloacal deformity?

The outlook for a child with a cloacal deformity depends on a number of factors, including the extent of the problem, associated anomalies, whether the sacrum is affected and procedure used to correct the problem. The clinicians at Boston Children's have a great deal of experience treating children with all kinds of cloacal deformities.

Our team will discuss your child’s potential for potty training for urine and stool. If your child is unable to potty train for stool, she can do our bowel management program. If your child has difficulty with potty training for urine, the urologist will help with different medical therapies to keep her dry and in normal underwear. Children with cloacal deformities will require routine follow-up as they approach puberty. Our gynecologist is experienced in treating girls with cloacal deformities.