Renal agenesis

HOUSTON -- Developmental abnormalities of the vulva and vagina are often easy to correct, but also easy to misdiagnose, Robert K. Zurawin, M.D., warned at a conference on vulvovaginal diseases sponsored by Baylor College of Medicine.

Many physicians have not been trained to recognize these rare disorders and, as a result, run the risk of doing excessive or inappropriate surgery, according to Dr. Zurawin, chief of the section of pediatric and adolescent gynecology at Baylor and of the gynecology service at Texas Children's Hospital in Houston.

"You need to be familiar with the syndromes before you treat. Many people are confronted with these conditions, and they don't know what they really are," he said. "With the obstructions, for example, they may just think it is an imperforate hymen and are not even aware that there is even an entity called obstructed hemivagina."

Clitoral hypertrophy is the only developmental abnormality of the clitoris, according to Dr. Zurawin. It used to be treated by clitoridectomy with "very unsatisfactory results," he said, describing more conservative procedures in use today. "This is mainly a cosmetic problem for patients, and the surgical management is resection of the enlarged clitoris," he said.

Abnormalities of the vulva include congenital labial fusion, which he said could be corrected with a simple flap procedure. Surgery is rarely used, however, for acquired labial agglutination. "This is one of most common referrals from pediatricians, because they don't know what to do with it and they are afraid," Dr. Zurawin said.

He attributed most cases to diaper rash, bubble baths, and detergents that can inflame fair skin. A common treatment is application of estrogen cream daily for several weeks until the labia are separated, he said. After that, parents are told to use oil or cream to keep the labia moist and apart.

Surgery would be used only if the opening were so small that a child was retaining urine, Dr. Zurawin said.

Hypertrophy of the labia minora and majora is primarily a concern of 11- and 12-year-old girls who wear tight jeans, according to Dr. Zurawin. When there is unilateral enlargement of the labia, they complain that the condition is unsightly and uncomfortable. Though labial hypertrophy can be corrected with a simple resection, he said, "Many times I tell them to wait--the other side will catch up."

Prolapse of the urethral mucosa presents with vaginal bleeding in early childhood and can look frightening, but is fairly common, Dr. Zurawin said. "It responds beautifully to estrogen," he said, adding that resection is necessary in rare cases and should not be too deep.

For hemangioma of the vulva, he recommended sending the patient to a dermatologist who would use laser therapy.

Among developmental abnormalities of the vagina, imperforate hymen is usually asymptomatic until a child reaches menarche, he said. It can be repaired with a simple incision, but without a digital examination it can easily be mistaken for another vaginal abnormality: transverse vaginal septum. The latter requires surgery and should be resected as much as possible.

Vaginal duplications are often asymptomatic and can be easily resected.

He characterized obstructed hemivagina, however, as "one of the most misdiagnosed anomalies in gynecology." Children will often have regular periods for a few months until the occlusion interferes. Often surgeons or gynecologists will do a major operation, he said, when all that is necessary is surgery to remove the septum.

Magnetic resonance imaging is not sufficient in these cases, however, as these children might also have undetected renal abnormalities, Dr. Zurawin warned. "I am very adamant about doing simultaneous laparoscopy," he said. "I want to make sure there are no other associated anomalies."

Vaginal agenesis is a fairly common anomaly for which there are a variety of approaches to creating a new vagina. With a new combined vaginal and laparoscopic approach, the child could be released from the hospital after an overnight stay and would heal very quickly, he said.

"If you have experience, [developmental abnormalities] are really not that difficult to treat ... and we are really developing minimally invasive operations for what used to be large operations," Dr. Zurawin said.

He recommended most gynecologists not try to correct these conditions, however. "They should really refer them to someone who had been trained.... They shouldn't attempt them by themselves," he said.

RELATED ARTICLE: Surgery to Correct Genitalia May Pose Legal Risk

Gynecologists should exercise caution when asked to correct ambiguous genitalia in young children or testify to child abuse, according to Dr. Zurawin.

Sexual assignment operations can produce "a legal medical nightmare," he warned.

Some children who underwent these procedures have grown up and formed organizations in opposition to them, calling attention to the transgender movement, said Dr. Zurawin.

"The paradox there is the best time to do the surgery is when [children] are 3 years old, before they are old enough to establish their sexual identity and before the surgery is traumatic," he said. "But on the other hand, psychologically speaking, you have cases of kids coming back later and saying, 'Why did you do this to me? I wanted to be whatever I was.'"

If parents want to go ahead with the surgery, he recommended legal counseling for the parents and the physician, and suggested that they consult transgender societies for their advice as well. "Parents don't have carte blanche in determining the surgical outcomes of their children," he said.

Child abuse cases are also risky for gynecologists who are not experienced in developmental anomalies, according to Dr. Zurawin.

"You have to be clinically precise," he said. "It's especially important to be experienced and competent when being called to evaluate for sexual abuse. You can't just assume a hymen is a hymen."

BY JANE SALODOF MACNEIL

Contributing Writer

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