Adolescents and endometriosis: Pearls for management

Diagnosis & Therapy

Article 7 of 10: Adolescents and endometriosis:
Pearls for management

Adolescents and endometriosis: Pearls for management

Early diagnosis, prompt initiation of therapy, and long-term management form the cornerstone for minimizing pain and disease progression and preserving future fertility in this patient population.


Case: An adolescent with persistent pelvic pain

Your 15-year-old patient presents 2 days following an emergency department visit for worsening pelvic pain, where she was diagnosed with a 6-cm ovarian cyst. The patient’s history is positive for chronic pelvic pain (6 months’ duration) that is exacerbated by her periods, leading to school absenteeism. You prescribe an oral contraceptive pill (OCP) for dysmenorrhea and ovarian cyst prevention.

Four weeks later the patient returns reporting persistent pain. Ultrasonography is performed; its results are normal with the exception of fluid in the posterior cul-de-sac.


Perspectives on Endometriosis Management

Because the patient experienced persistence of pain on cyclic OCPs, you offer her operative laparoscopy for diagnosis and surgical management of suspected endometriosis.

For many years, endometriosis was considered a disease that primarily affected women of reproductive age. As researchers began to unravel its complexities, however, increasing evidence of its onset in adolescence was uncovered. It is now understood that endometriosis frequently begins during adolescence; in fact, its prevalence in adolescence is believed to mirror that seen in adults, with approximately 10% of young women affected, although some reports have shown a higher incidence.1,2

The symptoms of adolescent endometriosis are often severe and can lead to significant impairments in quality of life, inability to participate in normal activities, and school absenteeism. Without effective diagnosis and treatment, the disease can progress, which may result in pelvic pain, pelvic masses, and possible infertility.3,4

A number of clinical challenges may confound the diagnosis of endometriosis in adolescents. These include variable presentation of symptoms and the absence of predictive signs and symptoms, such as infertility; an unwillingness on the part of adolescents to report symptoms to their health care providers; and the mistaken belief held by many patients and their families that painful periods are normal.

It also should be noted that atypical noncyclic chronic pain, as experienced by the case patient, is more frequently seen in this patient population than in adults, as are atypical endometriosis lesions (red, clear, or vesicular lesions), which have been shown to be more painful than other endometriosis lesions (FIGURE 1).1,4,5 Unfortunately, delayed diagnosis is the norm, with most patients experiencing symptoms for about a decade before a diagnosis is made.

Overcoming the challenges
Marc R. Laufer, MD, Chief of Gynecology at Boston Children’s Hospital, Center for Infertility and Reproductive Surgery at Brigham & Women’s Hospital, Professor of Obstetrics, Gynecology & Reproductive Biology at Harvard Medical School, and Director of the Boston Center for Endometriosis in Boston, Massachusetts, spoke about the opportunities that exist to improve diagnosis and earlier access to care. Recognizing that endometriosis does occur in younger teens—and sometimes in even younger children—is important for facilitating a plan for diagnosis and treatment, he said. “The youngest patient that I have diagnosed is 7 years of age,” he noted. “We have found endometriosis in teenagers with the onset of breast development, before their first menstrual period. We know that endometriosis is an estrogen-dependent disease, so it makes sense that endometriosis lesions could grow with estrogen stimulation, which is marked by the onset of breast development.”

Patient education is another crucial component. “Young women need to know that if they are having pain, it is not normal,” said Dr. Laufer.

“The challenge is to help them find what normal is, because a lot of people think that pain is normal.”

As a general rule, Dr. Laufer recommends further assessment when a young woman experiences pain on nonsteroidal anti-inflammatory drugs (NSAIDs) and estrogen/progestin or progestin after 3 cycles of pills, particularly when the pain adversely impacts her quality of life.4 Asking patients to keep a pain diary may be a useful tool for assessing the response to medical therapy (FIGURE 2).

“The way that I usually like to address levels of pain with young women is to ask them if they feel equal to men or to other women when they are having their period,” he said. “If they are at all disadvantaged from a pain standpoint, that is something to be taken seriously, because they don’t need to be suffering or to have an adverse effect to their quality of life just because they are having a menstrual cycle and pain that proceeds from endometriosis.”

Jennifer E. Dietrich, MD, MSc, Associate Professor in the Departments of Obstetrics and Gynecology and Pediatrics at Baylor College of Medicine and Chief of Pediatric and Adolescent Gynecology at Texas Children's Hospital in Houston, Texas, and President of the North American Society for Pediatric and Adolescent Gynecology (NASPAG), noted that adolescents may be reluctant to disclose information about their symptoms to their health care providers. “Sometimes, young women are very private, particularly when you are dealing with reproductive issues,” she said. “Not every adolescent is comfortable discussing these things.”

Establishing good rapport with adolescent patients is essential to overcome this reluctance, said Dr. Dietrich. One way to help patients relax and open up is to engage them in conversations about subjects that are not as scary.

“Maybe talk about activities they like to do outside of school, ask if they have a good group of friends they trust and feel safe around, or ask about their career goals—sometimes, talking about those things will allow adolescent patients to see that you are approachable and can really help them to open up,” she said.

Thoroughly assessing patients

When assessing adolescent patients with pelvic pain, the importance of taking a thorough history cannot be overstated. “We know that with adolescents, endometriosis doesn’t only have to manifest as pain with the period—there could be pain between the periods and there could also be gastrointestinal and genitourinary symptoms, such as diarrhea, constipation, frequent urination, or discomfort with urination,” said Dr. Laufer. Inquiring about the location, duration, and characteristics of a patient’s pain and associated symptoms is important (TABLE).1

“The other thing that is very important is to ask about a family history relating to gynecology--if there is a history of endometriosis or infertility either on the mom’s side or dad’s side,” said Dr. Laufer. Having that history might raise the index of suspicion in adolescents at increased risk, leading to earlier diagnosis and treatment; however, too often that history is not taken.

“It is not uncommon that a young teenager will be in for an evaluation and we find out that she has been suffering for years with pain despite the fact that her mom was known to have extensive endometriosis and infertility.

“It is sad that she suffered for years before an evaluation was undertaken.”

Whether or not imaging studies are needed should be determined on a case-by-case basis. In some cases, imaging may aid in determining a management plan and is needed to rule out a reproductive tract anomaly. However, it is important to note that the vast majority of adolescents with endometriosis will present with stage 1 or stage 2 disease, which are not detectable via ultrasonography or magnetic resonance imaging. Endometriomas and deeply infiltrating disease are believed to be rare in adolescents. 4

When to offer laparoscopy
The gold standard for a definitive diagnosis of endometriosis in adolescents remains an operative laparoscopy. However, the decision to offer laparoscopy should not be taken lightly. “We try to avoid unnecessary invasive procedures,” said Dr. Dietrich. “We don’t want these adolescents to undergo too many surgeries. As we all know, surgery comes with the risk of scar tissue formation just like endometriosis does, so certainly for the young patient who may be struggling with this, we want to be judicious about offering some medical management. If there is a need to proceed with surgical management, we can make a diagnosis at that time and then immediately follow up with medical management.”

“We tend to follow an algorithmic fashion of trying to make a diagnosis mainly because when one is confronted with a teenager who is having pain we need to first assess the young woman,” said Dr. Laufer (FIGURE 3). This algorithmic approach, which was included in the American College of Obstetricians and Gynecologists Committee Opinion on endometriosis in adolescents, involves the immediate destruction of visible lesions at the time of the diagnostic surgery. 6,7

Aggressive, long-term management for optimal outcomes
Following the laparoscopic excision and/or ablation of endometriosis lesions in adolescents, or in patients who are not candidates for surgical management, medical therapy should be initiated since there is no surgical cure for endometriosis. This may consist of continuous estrogen/progestin therapy, progestins alone, or gonadotropin-releasing hormone (GnRH) agonists. It is recommended that GnRH agonists be avoided in younger adolescents whenever possible because of concerns surrounding its possible effect on bone mineral density in teenagers.

Because the risk of recurrence is known to be high in adolescents, medical therapy is essential for disease suppression even in women who have undergone surgical management.

This generally consists of continuously dosed combined OCPs in the absence of contraindications. Alternatively, a long-cycling regimen in which the patient refrains from taking the combined OCPs for 1 week every 3 to 4 months may be considered.1

“In our practice, we remove or destroy all visible lesions of endometriosis and then use medical and suppressive therapy,” said Dr. Laufer. “In a follow-up study we performed, we looked at women who had had a surgical procedure and had a second operation. The majority of women have one operation, but those with recurrent pain not responding to medical therapy had a second operation 2 to 10 years after the first one. In these women we found that the disease had not progressed. So at least with the techniques we are using, we know that we are not causing adhesion formation and the disease, with surgical management and then menstrual suppression, is kept from progressing to a more advanced stage.”

In addition to long-term medical therapy, multidisciplinary management is also important, said Dr. Dietrich--“particularly for those patients with severe endometriosis, because they’ve struggled with pelvic pain all their lives,” she said. “It may make them sad, or they may feel fatigued because they feel sad.”

“It is not uncommon that depressed mood can occur.” In such cases, referral to a counselor or psychologist may be warranted.

Additionally, musculoskeletal pain can be a secondary component of endometriosis. “Patients’ posture can be different,” noted Dr. Dietrich. “They’re trying to clench their abdomen because they have so much pain sometimes, and that can lead to musculoskeletal pain.” In the presence of such pain, timely referral to physical therapy can help to relieve discomfort and improve a patient’s quality of life.

Case: Resolved
At the time of the operative laparoscopy, your patient is found to have stage 1 endometriosis with clear and red lesions in the anterior and posterior cul-de-sac. The lesions are destroyed and menstrual suppression is subsequently prescribed to avoid disease progression, pain, and potential infertility.

Resources for adolescent patients Resources for providers References
  1. Stuparich MA, Donnellan NM, Sanfillipo JS. Endometriosis in the adolescent patient. Semin Reprod. 2017;35(1):102–109.
  2. Laufer MR, Goitein L, Bush M, Cramer DW, Emans SJ. Prevalence of endometriosis in adolescent girls with chronic pelvic pain not responding to conventional therapy. J Pediatr Adolesc Gynecol. 1997;10(4):199–202.
  3. Doyle JO, Missmer SA, Laufer MR. The effect of combined surgical-medical intervention on the progression of endometriosis in an adolescent and young adult population. J Pediatr Adolesc Gynecol. 2009;22(4):257–263.
  4. Laufer MR. Helping “adult gynecologists” diagnose and treat adolescent endometriosis: reflections on my 20 years of personal experience. J Pediatr Adolesc Gynecol. 2011;24(suppl 5):S13–S17.
  5. Attarone M, Falcone T. Adolescent endometriosis. J Minim Invasiv Gynecol. 2015;22(5):705–706.
  6. American College of Obstetricians and Gynecologists. ACOG Committee Opinion. Number 310, April 2005. Endometriosis in adolescents. Obstet Gynecol. 2005;105(4):921–927.
  7. Laufer MR, Sanfilippo J, Rose G. Adolescent endometriosis: diagnosis and treatment approaches. J Pediatr Adolesc Gynecol. 2003;16(suppl 3):S3–S11.

Drs. Laufer and Dietrich report no financial relationships relevant to this article.

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