80. Management of Endometriosis in an 18-year-old with Turner's Syndrome: A Case Report.
In: Journal of Pediatric & Adolescent Gynecology, Jg. 36 (2023-04-01), Heft 2, S. 207-208
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Zugriff:
Although present in 30-50% of young women with chronic pelvic pain, endometriosis is uncommon among patients with primary ovarian insufficiency (POI) and is thought to be extremely rare in patients with Turner's syndrome. To date, fewer than a dozen cases of endometriosis in patients with Turner's syndrome have been described in the literature. This case report adds to the paucity of data and describes management of an 18-year-old female with Turner's syndrome and surgical diagnosis of endometriosis. Our patient has been treated at an academic pediatric hospital since 2017. She has a diagnosis of Turner mosaicism with karyotype 45X (11)/46 X,iXq (10) and required pubertal induction by Endocrinology. At age 13, she was started on hormone replacement therapy (HRT) with estradiol. Progesterone was added one year later. At age 16, a levonogestrel intrauterine device (LNG-IUD) was placed for treatment of dysmenorrhea. The following year, she began to have cyclic pelvic pain and irregular bleeding. Surgical evaluation showed pathology-confirmed endometriosis. Given her POI requiring HRT, she would not benefit from GnRH analogues. Estradiol was switched to norethindrone acetate (NETA) to decrease stimulation of endometriosis while providing partial protection of bone mineral density. Two months later, pain and menses had improved. A few months later, NETA dose was increased due to pelvic pain unrelieved with NSAIDs. Endocrinology recommended restarting estradiol as she had been off this treatment for six months. Unfortunately, her daily pelvic pain worsened despite further increase in NETA dose to 10 mg BID, and she had hypoestrogenic symptoms despite estradiol replacement. Once 18 years old, the patient requested hysterectomy with bilateral salpingo-oophorectomy for long-term management of her endometriosis and pelvic pain. She had expressed for several years that she did not wish to have biological children nor to ever carry a pregnancy. Following extensive counseling, including reproductive repercussions of surgery, patient was scheduled for total laparoscopic hysterectomy with bilateral salpingo-oophorectomy at a short interval. Patients with Turner's syndrome are not often identified to have endometriosis, given the high incidence of associated POI and need for HRT. Optimal treatment has not yet been established. For this unique population with both conditions, the benefits of HRT must be weighed against the risk of endometriosis exacerbation. In our patient, resumption of estradiol replacement may have worsened her pelvic pain. We look forward to re-evaluating symptoms following definitive surgery, with follow-up assessment to be included in the final abstract. Supporting Figures or Tables ▪ [ABSTRACT FROM AUTHOR]
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80. Management of Endometriosis in an 18-year-old with Turner's Syndrome: A Case Report.
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Autor/in / Beteiligte Person: | Tulchinskaya, Viktoriya ; Kanj, Rula V. |
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Zeitschrift: | Journal of Pediatric & Adolescent Gynecology, Jg. 36 (2023-04-01), Heft 2, S. 207-208 |
Veröffentlichung: | 2023 |
Medientyp: | academicJournal |
ISSN: | 1083-3188 (print) |
DOI: | 10.1016/j.jpag.2023.01.223 |
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