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Vascular Ehlers-Danlos syndrome — DFSRH MCQ

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EasyEhlers-Danlos SyndromeVascular Ehlers-Danlos syndromeDFSRH

A 28-year-old nulliparous woman with genetically confirmed vascular Ehlers–Danlos syndrome due to a pathogenic COL3A1 variant requests removal of her etonogestrel implant because she wishes to conceive. The implant remains in date and is causing no adverse effects. She has no history of arterial dissection or bowel rupture and assumes that pregnancy risk is therefore similar to that associated with hypermobile Ehlers–Danlos syndrome. She has not received preconception counselling from maternal medicine, clinical genetics or her vascular EDS team. She wishes any pregnancy to be planned and would accept a short delay in implant removal if this materially improves safety. Which is the most appropriate management plan?

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

Reveal the answer and explanation

Correct answer: C — Retain the implant and arrange urgent multidisciplinary preconception review before planned removal

Explanation lettering: E = shown as A · A = shown as B · B = shown as D · D = shown as E

Vascular EDS must be distinguished from hypermobile EDS: COL3A1-related tissue fragility confers substantial risks of arterial, gastrointestinal and uterine rupture during pregnancy, with the greatest risk around the peripartum period. A previous uncomplicated history does not remove this genotype-associated risk. UK maternal medicine protocols therefore classify vascular EDS as requiring maternal medicine centre involvement. The implant should, with her agreement, remain in place while urgent multidisciplinary preconception counselling is arranged. This should involve maternal medicine and her vascular/genetics or EDS team, enabling individualised discussion of maternal risk, inheritance, surveillance and alternatives before fertility resumes. Etonogestrel concentrations fall rapidly after removal, and pregnancies have occurred within 14 days, so removing it before review creates avoidable risk of an unplanned conception. A is insufficient because routine primary-care review does not match the complexity or severity of vascular EDS. B offers specialist review but substitutes user-dependent condoms for highly effective contraception during a high-risk interval. D delays specialist input until pregnancy is established, when preventive reproductive planning is no longer possible. E is coercive and overstates the position: pregnancy is exceptionally high risk and may be medically discouraged, but counselling must support informed, individualised decision-making rather than impose permanent reproductive prohibition.

Reference: FSRH Guideline: Progestogen-only Implant (February 2021; amended July 2023) — https://www.fsrh.org/Common/Uploaded%20files/documents/fsrh-guideline-progestogen-only-implants.pdf Working together to optimise outcomes for Women with Medical Problems in Pregnancy (September 2024) — https://cdn.healthiertogether.nhs.uk/docs/Wessex-Maternal-Medicine-Network-Guideline-V2.pdf Vascular Ehlers-Danlos syndrome and pregnancy: A systematic review (2024) — https://pubmed.ncbi.nlm.nih.gov/38926786/