Mullerian agenesis

BIG SKY, MONT. -- Endometriosis in adolescents has a very different appearance than in adults, Dr. Joseph S. Sanfilippo said at an ob.gyn. update sponsored by the Geisinger Health System.

"If you don't look carefully you'll miss endometriosis in adolescents," cautioned Dr. Sanfilippo, professor of ob.gyn. at the University of Pittsburgh.

Endometriosis in teens is typically early-stage disease.

Unlike the classic blue, brown, or black lesions that gynecologic surgeons are accustomed to seeing in adults, biopsy-proven endometriosis in adolescents often takes the form of clear vesicles or small red polypoid hemorrhagic or petechial lesions.

"When I have a teenager, I always get a peritoneal biopsy to confirm the diagnosis," he said. "It's simple to do through the laparoscope."

When a parent asks about the prognosis for her 17-year-old daughter with endometriosis, the honest answer is "bad." Symptomatic adolescent-onset endometriosis is most often a lifelong problem that will progress to severe fibrotic disease.

The exception involves adolescents who are found to have an outflow tract obstruction, as has been reported in various series to be the case in 10%-40% of teens with endometriosis.

Outflow tract obstruction of any cause--including a didelphic uterus or other uterine anomalies, vaginal septum, hematometra, renal agenesis, or imperforate hymen--is associated with extensive endometriosis that is typically reversed upon establishing a patent outflow tract.

The theory is that the outflow tract obstruction causes menstrual fluids to flow retrograde where they aggressively induce formation of endometriosis in the pelvic cavity.

"The message here is that when you have an adolescent with chronic pelvic pain, you have to think mullerian defect," the ob.gyn. emphasized.

The prevalence of endometriosis in adolescents appears to be similar to that in adults. At laparoscopy, it is found in 35%-70% of adolescents presenting with chronic pelvic pain.

Endometriosis has a strong genetic component.

A teenager with pelvic pain who has a strong family history of endometriosis is at roughly fivefold increased risk of having the disease, compared with a symptomatic adolescent without a family history.

The Oxford Endometriosis Gene Study (OXEGENE) is an ongoing international collaborative project aimed at identifying the multiple genes that are believed to predispose to endometriosis.

It's worth keeping an eye out for future OXEGENE reports, because it's expected that this study will eventually yield data that alter practice, Dr. Sanfilippo commented.

As he does with his adult patients, Dr. Sanfilippo routinely performs laparoscopy on adolescents whose chronic pelvic pain doesn't respond adequately to a trial of NSAIDs or ovulatory suppression with a high-progestin, low-estrogen OC. He finds endometriosis in adolescents mostly in the pouch of Douglas, on the uterosacral ligaments, and in the ovarian fossa.

'Always flip the ovaries up and look behind them. You'll find endometriosis there," he advised.

After addressing the endometriosis and adhesions surgically, Dr. Sanfilippo routinely prescribes medication aimed at preventing progression of the disease and preserving fertility.

Popular options include 6-9 months of daily medroxyprogesterone acetate, or a progestin-dominant OC administered indefinitely although these measures are based on anecdotal experience rather than well-designed studies.

COPYRIGHT 2002 International Medical News Group
COPYRIGHT 2002 Gale Group