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Contraception and endometrial protection during hormone replacement therapy in mosaic Turner syndrome — DFSRH

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EasyTurner SyndromeContraception and endometrial protection during hormone replacement therapy in mosaic Turner syndromeDFSRH

A 27-year-old woman with 45,X/46,XX mosaic Turner syndrome attends for contraceptive advice. She underwent spontaneous puberty and menstruated intermittently before developing premature ovarian insufficiency. She has an intact uterus and currently uses transdermal estradiol with oral micronised progesterone for 12 days each month. She understands that pregnancy would carry increased cardiovascular risk because she has a bicuspid aortic valve, although recent aortic imaging is stable. She has started a new relationship and wants highly effective reversible contraception. Her current estradiol regimen controls her symptoms well, and she wishes to continue it but would prefer to stop the cyclic oral progesterone. She has no contraindication to intrauterine contraception. Pregnancy can be reasonably excluded, and screening does not indicate a need to delay insertion. Which management plan is most appropriate?

Educational content. Not a substitute for clinical judgement or local policy.

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Correct answer: B — Insert a 52 mg levonorgestrel intrauterine device, continue transdermal estradiol and stop oral progesterone

Explanation lettering: D = shown as B · B = shown as C · C = shown as D

A 52 mg levonorgestrel intrauterine device provides highly effective contraception and can also supply the progestogenic component required to protect the endometrium during systemic oestrogen therapy. FSRH supports any 52 mg LNG-IUD for endometrial protection as part of HRT for 5 years; it should therefore be replaced at 5 years if this indication continues, even though its contraceptive licence may be longer. Turner syndrome-associated premature ovarian insufficiency does not reliably exclude intermittent ovulation, particularly with 45,X/46,XX mosaicism and previous spontaneous menstruation. Moreover, NICE explicitly advises that HRT is not contraceptive. This makes A inappropriate. B could provide both sex-steroid replacement and contraception, but it unnecessarily abandons a well-tolerated estradiol regimen contrary to her stated preference. C is effective contraception, but a copper IUD cannot provide endometrial opposition, so cyclic progesterone would still be required. E is an attractive smaller-device option for contraception, but FSRH does not recommend the 19.5 mg LNG-IUD for endometrial protection during HRT. The 52 mg device uniquely meets both immediate objectives: reliable contraception and replacement of cyclic oral progesterone while transdermal estradiol continues.

Reference: FSRH Clinical Guideline: Intrauterine Contraception (March 2023; amended 21 January 2025) — https://www.fsrh.org/Common/Uploaded%20files/documents/fsrh-clinical-guideline-intrauterine-contraception-mar-23-amended.pdf Menopause: identification and management (NICE guideline NG23) — Recommendations (Published November 2015; guideline updated 15 April 2026) — https://www.nice.org.uk/guidance/ng23/chapter/Recommendations Pregnancies in women with Turner syndrome: a retrospective multicentre UK study (2022) — https://pubmed.ncbi.nlm.nih.gov/34800331/