Endometriosis in Adolescence

Endometriosis in Children

“Adult” conditions don’t always adhere to age restrictions. We have previously discussed several conditions more commonly considered in adults that still can affect children (ex, Pulmonary Embolism, Cholelithiasis, Cholecystitis, Nephrolithiasis). Unfortunately, these conditions are more challenging to diagnosis in children because they are less common and also because they can present differently than their adult counterparts. Abdominal pain, we know all too well, is a very common complaint in the ED and has numerous causes. Let us consider another “Adult” condition that may lead to abdominal pain during adolescence – Endometriosis.

Endometriosis: Basics

  • Endometriosis = ectopic endometrial glands and stroma located outside of the endometrial cavity [Sieberg, 2020; ACOG, 2018]
    • It is a surgical and pathologic diagnosis determined by laparoscopy.
    • Laparoscopy visualizes concerning lesions, allows for biopsy for pathologic confirmation, and can also offer means to treat lesions (via ablation, coagulation, or resection).
  • Condition is considered to be an inflammatory-mediated estrogen-dependent disorder. [Sieberg, 2020; ACOG, 2018]
    • Estrogen promotes endometrial tissues to increase prostaglandin production.
      • Can be seen even before menarche.
        • Thelarche is the onset of estrogen production! [Batt, 2003]
      • Prostaglandin pathways lead to inflammation and pain.
    • Can lead to chronic inflammation and fibrosis with subsequent:
      • Infertility
      • Dyspareunia
      • Reduced quality of life
  • Endometriosis is the most common cause of Secondary Dysmenorrhea. [Sieberg, 2020; ACOG, 2018]
    • Primary dysmenorrhea
      • Most common type of dysmenorrhea.
      • Absence of pelvic pathology
      • Often managed successfully with nonsteroidal anti-inflammatory drugs (NSAIDs) and/or hormonal suppression
    • Secondary dysmenorrhea
      • Painful menses related to recognized pathology
      • Persistent symptoms despite appropriate, empiric therapy, should raise concern for possible Secondary Dysmenorrhea.
      • Considerations:
        • Endometriosis
        • Mullerian malformations
        • Cervical stenosis
        • Ovarian cysts
        • Uterine polyps
        • Uterine leiomyomata
        • Adenomyosis
        • Pelvic Inflammatory Disease
        • Pelvic Adhesions
    • Chronic pelvic pain [ACOG, 2018]
      • Pelvic pain that lasts 6 months or more
      • Can be cyclic, acyclic, intermittent, or constant.
  • At least two thirds of adolescent females with “chronic pelvic pain” or “dysmenorrhea unresponsive to therapy” will be diagnosed with endometriosis. [Sieberg, 2020; ACOG, 2018]
    • Endometriosis affects 1 out of 10 women during reproductive age.
      • The true prevalence of endometriosis in adolescence is not known.
      • Likely underestimated (ex, many cases diagnosed in adulthood have had symptoms since adolescence).
    • Most adolescents are diagnosed with early-stage endometriosis, but this does not necessarily correlate with severity of symptoms.
  • Endometriosis in adolescence is different than that diagnosed in adults. [ACOG, 2018; Zannoni, 2016; Batt, 2003]
    • The locations of the lesions are different.
      • Adults more often have the lesion in pelvic peritoneum, ovaries, fallopian tubes, bladder, uterus, and bowel.
      • Adolescents more often have lesions between the ovaries and the peritoneum, in the pouch of Douglas, in the uterosacral ligaments, and on the rectovaginal septum.
    • The appearance of the lesions is different.
      • Typically clear or red and may be vesicle-like.
      • Can be actually obscured by the induction of pneumoperitoneum during laparoscopy.
      • May not be recognized by physicians unfamiliar with endometriosis in adolescence.
    • The lesions may be more metabolical active in adolescents.
      • Produce more prostaglandin.
      • Lead to more pain and inflammation.
    • The presentation is different.
      • Young women are more likely to have acyclic pelvic pain (in up to 90% of cases).
      • GI symptoms (ex, acute abdominal pain, constipation, diarrhea, and nausea) are also commonly reported in adolescents with endometriosis.

Endometriosis: Evaluation

  • Don’t dismiss another person’s pain. [Zannoni, 2016]
  • Keep Ddx broad and look for clues of Endometriosis. [ACOG, 2018; Zannoni, 2016]
    • Pain for 3 – 6 months (while not super exciting to the Emergency Medicine aspect of our brains) raises concern for secondary dysmenorrhea.
    • Prolonged intake of NSAIDS and/or hormonal therapy => think secondary dysmenorrhea.
    • Early menarche at or before age 11 increases risk. [Batt, 2003]
    • Short menstrual cycles (27 days or less) increases risk. [Batt, 2003]
    • Family history of endometriosis increases risk (genetics are always important).
    • Frequent absenteeism due to pelvic pains also increases risk.
  • Ultrasound is the preferred initial image for Secondary Dysmenorrhea. [ACOG, 2018]
    • Primary goal is to evaluate for structural anomalies which may be the source of pain.
    • U/S may also show findings concerning for endometriosis.
    • A normal U/S is useful, but does not rule out endometriosis.
  • Early referral. [Batt, 2003]
    • The goal of earlier detection and intervention requires early consideration and consultation/referral to gynecologists familiar with endometriosis in adolescence. [ACOG, 2018]
    • Can discuss options of advancing medical therapies while also considering laparoscopy.
  • Therapy is multimodal. [ACOG, 2018; Zannoni, 2016]
    • Laparoscopy is both diagnostic and therapeutic.
    • NSAIDs and hormonal therapy (progestin-only agents) are typically used as suppressive therapy even after laparoscopy.
    • Gonadotropin-releasing hormone (GnRH) agonist (Leuprolide) therapy may also be employed.
    • Risks/benefits of all medications must be weighed carefully as this often required chronic use of the therapies so side-effects become important.
    • Unfortunately, endometriosis is not typically “curable.”
    • Other therapies may be advantageous as well:
      • Biofeedback
      • Pain Management Teams
      • Acupuncture
      • Close follow-up

Moral of the Morsel

  • Age matters, but doesn’t define conditions! Endometriosis is associated with estrogen production… and that starts at thelarche… which is not specific to a single age (so assess the Tanner stage!).
  • Chronic problems are not acute, but they still hurt and cause significant consequences. As emergency medicine providers, we must condition ourselves to think outside of the acute time frame.
  • Prolonged pelvic/abdominal pain requiring multiple ED visits with “negative” work-up and no help with NSAIDs? Hmmmm… put endometriosis on your Ddx.

References

Sieberg CB1, Lunde CE2, Borsook D3. Endometriosis and pain in the adolescent- striking early to limit suffering: A narrative review. Neurosci Biobehav Rev. 2020 Jan;108:866-876. PMID: 31862211. [PubMed] [Read by QxMD]
[No authors listed] ACOG Committee Opinion No. 760: Dysmenorrhea and Endometriosis in the Adolescent. Obstet Gynecol. 2018 Dec;132(6):e249-e258. PMID: 30461694. [PubMed] [Read by QxMD]
Stuparich MA1, Donnellan NM1, Sanfilippo JS1. Endometriosis in the Adolescent Patient. Semin Reprod Med. 2017 Jan;35(1):102-109. PMID: 27992932. [PubMed] [Read by QxMD]
Taylor HS1, Giudice LC1, Lessey BA1, Abrao MS1, Kotarski J1, Archer DF1, Diamond MP1, Surrey E1, Johnson NP1, Watts NB1, Gallagher JC1, Simon JA1, Carr BR1, Dmowski WP1, Leyland N1, Rowan JP1, Duan WR1, Ng J1, Schwefel B1, Thomas JW1, Jain RI1, Chwalisz K1. Treatment of Endometriosis-Associated Pain with Elagolix, an Oral GnRH Antagonist. N Engl J Med. 2017 Jul 6;377(1):28-40. PMID: 28525302. [PubMed] [Read by QxMD]
Zannoni L, Forno SD, Paradisi R, Seracchioli R. Endometriosis in Adolescence: Practical Rules for an Earlier Diagnosis. Pediatr Ann. 2016 Sep 1;45(9):e332-5. PMID: 27622918. [PubMed] [Read by QxMD]
Janssen EB1, Rijkers AC, Hoppenbrouwers K, Meuleman C, D’Hooghe TM. Prevalence of endometriosis diagnosed by laparoscopy in adolescents with dysmenorrhea or chronic pelvic pain: a systematic review. Hum Reprod Update. 2013 Sep-Oct;19(5):570-82. PMID: 23727940. [PubMed] [Read by QxMD]
Batt RE1, Mitwally MF. Endometriosis from thelarche to midteens: pathogenesis and prognosis, prevention and pedagogy. J Pediatr Adolesc Gynecol. 2003 Dec;16(6):337-47. PMID: 14642954. [PubMed] [Read by QxMD]

Sean M. Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renowned educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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