Adenomyosis in an 18-Year-Old Adolescent: A Case Report

Adenomyosis is a rare cause of chronic pelvic pain or se- vere dysmenorrhea that presents in the adolescent popula- tion. Here we describe an 18-year-old nulliparous woman who presented with a history of severe and worsening dys- menorrhea with cramps and increased menstrual flow since the menarche occurred 4-years-ago. A magnetic resonance imaging (MRI) described a poorly defined junctional zone of the endometrium, suggestive of adenomyosis, associated with the discrete heterogeneity of the adjacent myometri- um. The patient underwent a course of GnRH agonists for 6 months, followed by continuous combined oral contra- ceptives (COC) pills with pain resolution. In this population, fertility preservation is a goal and therefore initial therapy for focal adenomyosis involves hormonal suppression with COC.

Adenomyosis, Pelvic pain, Dysmenorrhea, Adolescent 1Federal University of Rio Grande do Norte (UFRN), Brazil 2Potiguar University, Brazil 3Department of Gynecology and Obstetrics, Federal University of Rio Grande do Norte (UFRN), Brazil 4Department of Gynecology and Obstetrics, Potiguar University, Brazil CASe RepoRt Check for updates

Adenomyosis is a benign condition of the uterus de- fined by the presence of endometrial glands and stroma > 2.5 mm in depth in the myometrium and a variable degree of adjacent myometrial hyperplasia, causing globular and cystic enlargement of the myometrium, with some cysts filled with extravasated, hemolyzed red blood cells, and siderophages [1,2]. Cystic adenomyosis can be present in both an adult form, which is present in 5-7% of hysterectomy fibroid ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410121 • Page 2 of 4 • Vidal et al. Obstet Gynecol Cases Rev 2018, 5:121 al-myometrial interface, such as that caused by sur- gery or pregnancy may create a predisposition towards endometrial invasion of myometrium, and that local hyperestrogenism or dysperistalsis facilitates the pro- cess. However, evidence of adenomyosis early in repro- ductive life and in the absence of previous surgery or pregnancy suggests that invasion of endometrial tissue is not necessarily mediated mechanically; it may occur from endometrial-myometrial dysfunction [8]. Evidence shows that endometriosis and adenomyosis have in common an endometrial dysfunction involving both eu- topic and heterotopic endometrium. Although anoma- lies are not identical, they share the common feature of leading to increased invasiveness. In both conditions, there is also a reaction of the inner myometrium that, although more pronounced in the case of adenomyosis, is nonetheless also present in endometriosis [6]. The symptoms of adenomyosis are variable and the proportion of women with adenomyosis who have ab- normal uterine bleeding and/or painful symptoms, as was reported by the adolescent patient, is unclear. A consensus on the association between dysmenorrhea and adenomyosis is lacking, with some studies support- ing this relationship, and others finding only a weak as- sociation [8]. Adenomyosis is a rare cause of chronic pelvic pain or severe dysmenorrhea in the adolescent population. For patients who have symptoms of dysmenorrhea or chronic pelvic pain refractory to NSAIDs or COC therapy, additional investigation with imaging may reveal this di- agnosis as happened in this case. MRI is more indicated than TVUS, since it has shown potential superiority over TVUS in some situations, and because defined diagnos - tic criteria (Table 1) have made inter-observer variability bleeding since the menarche occurred 4-years-ago, these were insufficiently relieved by NSAID and COC. She reported having started using COC two-years-ago when her sex life began, and she never underwent sur- gery, had an abortion or suffer any trauma. Her family history is unremarkable. Pelvic examination revealed a normal vagina, vulva and adnexae and a normal-sized anteverted uterus. Transvaginal ultrasound (TVUS) demonstrated a uterus of normal volume and irregular contours, homogeneous myometrium and endometrium and without change of thickness. Ovaries were enlarged at the expense of mul- tiple follicles. A follow-up MRI obtained 1 month later described a poorly defined junctional zone of the endo- metrium, suggestive of myometrial cystic adenomyosis, associated with the discrete heterogeneity of the adja - cent myometrium and, in addition, a small increase in the thickness of the posterior uterine wall (Figure 1). The patient underwent a course of GnRH agonists for 6 months, followed by continuous COCs with pain resolution.

This case demonstrates that adenomyosis is a rare but possible cause of dysmenorrhea and pelvic pain in adolescent patients, despite the disease being typically present in adult women in the third or fourth decade of life. Although endometriosis may be the most common cause of secondary dysmenorrhea in younger patients, adenomyosis should remain in the differential diagnosis [7]. Previous pathogenetic theories for adenomyosis have proposed that traumatization of the endometri- Figure 1: An asymmetric widening of the posterior junctional zone is shown in magnetic resonance imaging (MRI), with ill-de- fined border, presenting an elliptical shape and bright foci, with no mass effect on the endometrial cavity (arrows). ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410121 • Page 3 of 4 • Vidal et al. Obstet Gynecol Cases Rev 2018, 5:121 An example of drugs with higher costs and more trou- blesome side effects are GnRH agonists. They are very effective against adenomyosis related pain, and there - by also contribute to the occurrence of many successful pregnancies and deliveries, however their use is associ- ated with frequent and intolerable hypoestrogenic side effects, including vasomotor syndrome, genital atrophy and mood instability, and the use of these drugs should be for a short period of time among adolescents who do not improve their symptoms with continuous use of COC, which is what happened in our case. GnRH ago- nists also have a negative impact on bone health and a possible negative influence on cardiovascular health [2]. Surgical management is necessary in cases where dysmenorrhea is medication resistant. Some authors argued in favor of the laparoscopic approach. Other surgical approaches have been proposed such as abla - tion after insertion of a radiofrequency needle under ultrasound guidance, the use of a single-incision with monopolar cautery or the use of robotic surgery. In the case of a focal lesion or adenomyotic cyst not involving the endometrial cavity, safe surgical resection is possi - ble [4].

In conclusion, this case draws attention to a little known but not uncommon cause for both primary and secondary dysmenorrhea, which can affect young nul - liparous women. MRI may be useful in establishing a diagnosis even when TVUS is normal. In this population, fertility preservation is the primary goal and, therefore initial therapy for focal adenomyosis involves hormonal suppression with COC. Further research is needed due to limited reports in the literature regarding manage - ment of adenomyosis in the adolescent. Conflicts of Interest The authors declare no potential conflicts of interest and no sources of support.

All authors equal contributed to the design and im- plementation of the research, to the analysis of the case and to the writing of the manuscript.

1. Naftalin J, Hoo H, Nunes N, Holland T, Mavrelos D, et al. (2016) Association between ultrasound features of adeno - myosis and severity of menstrual pain. Ultrasound Obstet Gynecol 47: 779-783. 2. Tsui KH, Lee WL, Chen CY, Sheu BC, Yen MS, et al. (2014) less significant [9]. Although adenomyosis has tradition- ally been confirmed by histopathologic diagnosis from surgery, MRI can allow for accurate diagnosis of adeno- myosis in most cases. Several studies have demonstrat- ed sensitivity rates ranging from 70 to 88% and specific- ity as high as 91%. This is significantly better sensitivity and specificity of MRI compared with TVUS in the diag - nosis of these abnormalities [9,10]. Careful review of the literature reveals very few case reports, case series or small cohorts addressing adeno - myosis or adenomyotic cysts presenting in the female adolescent [3, 4,7,11,12]. A variety of imaging modali - ties were utilized in diagnosis. All cases presented with severe dysmenorrhea or pelvic pain, as reported by the patient in this case. In most cases, as in our patient and in the cases of two adolescents reported by Itam, et al. [7], the diagnosis was only made when patients did not respond to initial empirical treatment including the use of COC and NSAID. Medical treatments for adenomyosis always follow the principles of the management of endometriosis, which are usually aimed at reducing the production of endogenous estrogen or inducing endometrial differen- tiation with progestins. Clinical evidence points to the clear and deleterious effect of uninterrupted ovulato - ry cycles on the development and persistence of ade- nomyosis. The objectives of medical treatment are the inhibition of ovulation, abolition of menstruation, and achievement of a stable steroid hormone milieu [2]. Both medical and surgical treatments offer oppor - tunities for disease stabilization and regression. Initial therapy for focal adenomyosis involves hormonal sup - pression with COC, especially for the female adolescent in whom preserving fertility is paramount. Adolescents may represent a subset of patients with adenomyosis whose lesions are completely treatable with hormonal therapy [10]. For adenomyosis, the goal of therapy is important, and can include symptom relief and possibly increased fertility. The therapeutic goal of medical treatment is not lesion resorption: Lesions survive any drug, at any dose, for any period of use, and come back after treatment dis- continuation. Therefore, treatment should be tailored to the specific symptom or the special request of the in- dividual patient. The results from systematic literature reviews have consistently demonstrated that, if amenor- rhea is obtained, there are no statistical differences be- tween the various available drugs in terms of pain relief, but tolerability, side effects, and costs vary widely [2]. Table 1: Diagnostic criteria for adenomyosis in magnetic resonance imaging (MRI). 1- Thickening of the junctional zone, with a thickness exceeding 12 mm 2- Large, rand asymmetric uterus, with a maximum junctional zone thickness of at least 12 mm and punctate high-intensity myo- metrial foci 3- Bright foci on T2-weighted images, which represent foci of heterotopic endometrial tissue, cystic dilatation of endometrial glands or hemorrhagic foci ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410121 • Page 4 of 4 • Vidal et al. Obstet Gynecol Cases Rev 2018, 5:121 8. Pinzauti S, Lazzeri L, Tosti C, Centini G, Orlandini C, et al. (2015) Transvaginal sonographic features of diffuse ade- nomyosis in 18-30-year-old nulligravid women without en- dometriosis: Association with symptons. Ultrasound Obstet Gynecol 46: 730-736. 9. Agostinho L, Cruz R, Osório F, Alves J, Setúbal A, et al. (2017) MRI for adenomyosis: A pictorial review. Insights Imaging 8: 549-556. 10. Dietrich JE (2010) An update on adenomyosis in the ado- lescent. Curr Opin Obstet Gynecol 22: 388-392. 11. Mansouri R, Santos XM, Bercaw-Pratt JL, Dietrich JE (2015) Regression of Adenomyosis on Magnetic Reso- nance Imaging after a Course of Hormonal Suppression in Adolescents: A Case Series. J Pediatr Adolesc Gynecol 28: 437-440. 12. Manta L, Suciu N, Constantin A, Toader O, Popa F (2016) Focal adenomyosis (intramural endometriotic cyst) in a very young patient - differential diagnosis with uterine fibro- matosis. J Med Life 9: 180-182. Medical treatment for adenomyosis and/or adenomyoma. Taiwan J Obstet Gynecol 53: 459-465. 3. Ball E, Ganji M, Janik G, Koh C (2009) Laparoscopic re- section of cystic adenomyosis in a teenager with arcurate uterus. Gynecol Surg 6: 367-370. 4. Brosens I, Gordts S, Habiba M, Benagiano G (2015) Uter- ine Cystic Adenomyosis: A disease of younger women. J Pediatr Adolesc Gynecol 28: 420-426. 5. Iacovides S, Avidon I, Baker FC (2015) What we know about primary dysmenorrhea today: A critical review. Hum Reprod Update 21: 762-778. 6. Benagiano G, Brosens I, Habiba M (2015) Adenomyosis: A life-cycle approach. Reprod Biomed Online 30: 220-232. 7. Itam SP, Ayensu-Coker L, Sanchez J, Zurawin R, Dietrich JE (2009) Adenomyosis in adolescente population: A case report and review of the literature. J Pediatr Adolesc Gyne- col 22: e146-e147.