Cloacal Deformities - Children's Hospital Boston

300 Longwood Avenue
Boston, MA 02115
(617) 355-6000

My Child Has:

Cloacal Deformities

Programs that treat this condition

Center for Bladder Exstrophy Care & Support Group

Gender Management Service (GeMS) Clinic

What are cloacal deformities?

Cloacal deformities encompass a wide range of complicated defects that occur during the prenatal development of the lower abdominal structures. (The cloaca is the part of an embryo that develops into these structures) Cloacal deformities typically refer to the most complex variety of imperforate anus (a condition where the anus has not been formed or is perforated, and the colon communicates with the bladder). It can also refer to a single opening in the perineum (pelvic floor) of a girl, into which common channel enters the urethra, the vagina, as well as the rectum.

Variations of cloacal deformities are multiple, but may involve the opening of the bladder/urethra, genital tract, and rectum. There may be multiple variations in the genital tract where there may be vaginal atresia (absence or abnormal closure of) as well as a lack or failure in the development of other female reproductive structures including the uterus and fallopian tubes. There may also be variation in the degree of development of the bladder, abdominal wall, and rectum.

Cloacal deformities are often associated with anamolies of the upper urinary system, renal anomalies, imperforate anus, and anomalies of the sacrum(the part of the spinal column that is directly connected with the pelvis). The most complicated of these deformities is cloacal exstrophy. A child born with cloacal exstrophy is born with many inner-abdominal structures, such as the large intestine and bladder, exposed and an omphalocele will be present as well.

To view normal anatomy of the urinary tract and digestive tract, see Urinary Tract Anatomy and Digestive System.

What causes cloacal deformities?

The cause of cloacal deformities is unknown. It has nothing to do with anything the baby's parent's did during pregnancy. It is very unlikely that this condition can be prevented.

How are cloacal deformities diagnosed?

Cloacal deformities can be diagnosed on prenatal ultrasound. After birth, exstrophy can be determined by phyiscal examination. Other diagnostic tests and procedures can include:

A careful analysis of structures of the upper urinary system and sacrum. Doctors will also look for any esophageal, vertebral, tracheal, radial, and cardiac anomalies that are sometimes associated with cloacal deformities.
A careful analysis of the perineal and pelvic anatomy with the use of some combination of the following imaging studies:

Computerized tomography scan (Also called a CT or CAT scan.) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called "slices"), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat and organs. CT scans are more detailed than general x-rays.

Magnetic Resonance Imaging (MRI) a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.

Endoscopy: a diagnostic procedure in which doctors use a small instrument called an endoscope for visualization of the interior of a hollow organ, such as the rectum, urethra or vagina.

Abdominal ultrasound (Also called sonography.) - a diagnostic imaging technique which uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.

How are cloacal deformities treated?

Cloacal deformities require surgical repair. The treatment plan devised for your child will depend on the type and extent of the abnormality. Before the treatment plan to correct the deformity is put in place, doctors will be concerned with stabilizing your baby. This may involve the creation of a diverting colostomy to allow passage of stool. With a colostomy, the large intestine is divided into two sections and the ends of the intestine are brought through surgically created openings in the abdomen. The upper section allows stool to pass through the opening (called a stoma) and then into a collection bag. The lower section allows mucus that is produced by the intestine to pass.

The bladder must also be decompressed to relieve obstruction of the upper urinary tract. This is done with catheterization to help eliminate urine. The vagina must also be decompressed.

After a period of stabilization, growth and clarification of your baby's anatomy, a definitive correction of the deformity is planned. The treatment typically involves the surgical creation of the urethra and vagina. Finally, a reconstruction of the bowel as a perineal "pull-through" must be completed. For this procedure, the surgeon may have to open the abdomen to complete the connection of the colon to the rectum.

At a third operation once healing is assured, the colostomy will be closed and the fecal stream reestablished. Further urinary or genital tract surgery may also be needed.

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

The future outlook for a child with a cloacal deformity is dependent on a number of factors, including the extent of the problem, presence or absence of associated anomalies (most commonly involving the genitourinary system), the presence of an intact sacrum and the technical exactness and healing of the operative procedure used to correct the problem. More mild cases generally have a favorable long-term outlook. These children usually have excellent sphincter control and a near normal stooling pattern. In more severe cases, 70-80 percent of children will have a very favorable outlook with good to excellent stool control and only infrequent episodes of leakage or incontinence. The other 20-30 percent, depending on the degree of associated problems, will likely need a life-long bowel management program with a variety of medical and surgical adjuncts utilized to optimize their lifestyle.



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