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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.
What are cloacal anomalies?
Cloacal anomalies encompass a wide array of complicated defects that occur during development of the female fetus during pregnancy. Normally, the reproductive, gastrointestinal and urinary tracts merge to drain out of one common channel. But if this place 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.
Yet, 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 upper urinary tract and kidneys.
How are cloacal anomalies diagnosed?
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 X-ray is done to look for a mass and to see if there are any hard body parts present. 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.
How are cloacal anomalies treated?
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 artificial anus created in 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.
Frequently asked questions:
Will my child be able to control her urine or feces following the procedure?
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.
Will she be able to have a normal sex life?
Yes. Once the cloacal anomaly is corrected, a normal and enjoyable sex life is possible.
Are there any risk factors for cloacal anomalies?
No. There are no known risk factors for this deformity.
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