Vaginal Anomalies: Cloacal Anomalies

Cloacal anomalies are among the greatest challenges of pediatric surgery and urology. But what are they? And if your newborn has been diagnosed with this combination of defects, what can you expect? The following information should help you talk to your child's doctors.

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What are cloacal anomalies?

Cloacal anomalies encompass a wide array of complicated defects that occur during development of the female fetus during pregnancy. In cloacal anomalies, the reproductive, gastrointestinal and urinary tracts merge to drain out of one common channel instead of three separate openings. But if the point where they come together is low, a child has no visible anus (the duct through which feces exit the body), even though the remaining anatomy appears normal. In these patients, urinary tract obstructions are unusual because the tract typically opens into a wide common channel that drains freely.

However, if the coming together of these structures is high, then the common channel is long and urinary tract infections are common. Moreover, the clitoris looks like a penis, causing gender confusion. Cloacal-related anomalies can also result in multiple vaginas, a malformed anus and other defects of the ureters and kidneys.

Cloacal anomalies are discovered typically at the time of birth. Upon physical examination, the newborn usually has only one opening which is usually a hooded, elongated structure. The infant also commonly has abdominal swelling.

Patients who have a cloacal anomaly undergo many radiologic examinations including X-rays, ultrasounds and MRI.

An abdominal ultrasound is performed to see if there is swelling of the bladder, vagina and rectum. It will also show if there is a condition called hydronephrosis, in which there is a buildup of urine in the kidneys and ureters. This is usually due to compression of the bladder outlet caused by buildup of urine in the bladder.

In addition, a retrograde genitogram will be done where dye is injected into the common channel and an X-ray is taken. The dye helps the doctor understand the anatomy that cannot be seen and it may also reveal any of the associated anatomical defects that are associated with this disorder. In all, this information helps the surgeon decide which procedure is best suited for treatment.

Lastly, an MRI of the pelvis and spine will be done, which can detect if there are any spinal defects present.

Cloacal anomalies require surgical repair. The procedure depends on the type and extent of the abnormality. In any case, the first priority in treating this problem is to stabilize the newborn, which may require a colostomy, which permits the child to pass stools through an opening created on the abdominal wall. This procedure includes dividing the colon into two and sewing the open ends to the abdomen. The upper end allows the passage of stool while the lower end permits the drainage of mucous created by the colon. Because the bladder is typically swollen, it may need to be decompressed by catheterization. The vagina, because of the buildup of substances in response to mother's hormones, may also require decompression.

Once the patient is stabilized and the anatomy is clearly defined, the reconstructive process may begin. Usually, reconstruction occurs after one year of age. If the common channel is smaller than three centimeters, the rectum is detached from the vagina. The common channel is brought down to the surface of the skin. The common channel is then divided and the openings of the vagina and urethra are put in the appropriate place.

Your child's ability to control either the flow of her urine or elimination of her stool depends on the severity of the anomaly. In milder cases, more than 90 percent of patients have good sphincter control and a nearly normal pattern eliminating stools. Between 70 and 80 percent of patients with more severe cloacal anomalies will have good to excellent stool control with a few infrequent episodes of leaking. The other 20 to 30 percent of patients may have long-term problems, depending on the severity of their deformity. They can look forward to periodic medical and surgical interventions.

Yes. Once the cloacal anomaly is corrected, a normal and enjoyable sex life is possible.

No. There are no known risk factors for this deformity.

Reviewed October, 2009

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Glossary Terms

abdomen:
	Also referred to as the belly. It is the part of the body that contains all of the internal structures between the chest and the pelvis.
abnormality:
	A variation from a normal structure or function of the body.
anatomy:
	The physical structure of an internal structure of an organism or any of its parts.
anus:
	Opening at the end of the digestive tract where feces (stool) leave the body. The final two inches of the rectum.
bladder:
	The bladder is a thick muscular balloon-shaped pouch in which urine is stored before being discharged through the urethra.
catheter:
	A thin tube that is inserted through the urethra into the bladder to allow urine to drain or for performance of a procedure or test, such as insertion of a substance during a bladder X-ray.
catheterization:
	Insertion of a narrow tube through the urethra or through the front of the abdominal wall into the bladder to allow urine drainage.
clitoris:
	Sensitive female sex organ, which is visible at the front of the vagina.
colon:
	Large intestine.
colostomy:
	An opening, surgically created in the abdomen, that functions as an anus.
decompression:
	A surgical procedure to reduce pressure in an organ, part of the body or the pressure of tissues on a nerve.
feces:
	The body's solid waste matter, composed of undigested food, bacteria, water and bile pigments, and discharged from the intestines (bowel) through the anus.
fetus:
	An unborn offspring from the end of the eighth week of conception until birth.
gastrointestinal:
	Also referred to as GI. The stomach and the intestines.
genitogram:
	X-ray examination of the internal genital duct system.
hormone:
	A natural chemical produced in one part of the body and released into the blood to trigger or regulate particular functions of the body. Antidiuretic hormone tells the kidneys to slow down urine production.
hydronephrosis:
	Swelling of the top of the ureter, usually because something is blocking the urine from flowing into or out of the bladder.
infection:
	A condition resulting from the presence of bacteria or other microorganisms.
ions:
	Electrically charged atoms.
kidney:
	One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.
kidneys:
	One of two bean-shaped organs that filter wastes from the blood and discharge these waste products in urine. The kidneys are located on either side at the level of the 12th ribs toward the back. The kidneys send urine to the bladder through tubes called ureters.
MRI:
	Also referred to a magnetic resonance imaging. 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.
ostomy:
	A surgical procedure such as a colostomy or ileostomy, in which an artificial opening for excreting waste matter is created.
pelvis:
	The bowl-shaped bone that supports the spine and holds up the digestive, urinary, and reproductive organs. The legs connect to the body at the pelvis.
penis:
	The male organ used for urination and sex.
radiologic:
	X-ray.
rectum:
	The lower part of the large intestine, ending in the anal opening.
retrograde:
	Backwards.
retrograde genitogram:
	Special contrast X-rays to diagnose ambiguous genitalia.
sphincter:
	A round muscle that opens and closes to let fluid or other matter pass into or out of an organ. Sphincter muscles keep the bladder closed until it is time to urinate.
stool:
	Waste material (feces) discharged from the body.
ultrasound:
	Also referred to as a sonogram. A technique that bounces painless sound waves off organs to create an image of their structure to detect abnormalities.
ureter:
	One of two tubes that carry urine from the kidneys to the bladder.
ureters:
	Tubes that carry urine from the kidneys to the bladder.
urethra:
	A tube that carries urine from the bladder to the outside of the body. In males, the urethra serves as the channel through which semen is ejaculated and it extends from the bladder to the tip of the penis. In females, the urethra is much shorter than in males.
urinary tract:
	The system that takes wastes from the blood and carries them out of the body in the form of urine. Passageway from the kidneys to the ureters, bladder and urethra.
urinary tract infection:
	Also referred to as UTI. An illness caused by harmful bacteria, viruses or yeast growing in the urinary tract.
urine:
	Liquid waste product filtered from the blood by the kidneys, stored in the bladder and expelled from the body through the urethra by the act of urinating (voiding). About 96 percent of which is water and the rest waste products.
urology:
	Branch of medicine concerned with the urinary tract in males and females and with the genital tract and reproductive system of males.
vagina:
	The tube in a woman's body that runs beside the urethra and connects the uterus (womb)to the outside of the body. Sometimes called the birth canal. Sexual intercourse, the outflow of blood during menstruation and the birth of a baby all take place through the vagina.

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