Treatment of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser syndrome in Denmark: a nationwide comparative study of anatomical outcome and complications

Fertil Steril. 2018 Sep;110(4):746-753. doi: 10.1016/j.fertnstert.2018.05.015.

Abstract

Objective: To compare the long-term anatomical outcome and complications in treatments of vaginal agenesis in Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.

Design: A historical comparative follow-up study using medical chart reviews.

Setting: Public hospitals.

Patient(s): A nationwide cohort of patients diagnosed with MRKH syndrome (n = 168).

Intervention(s): McIndoe vaginoplasty (n = 54), self-dilation (n = 60), coital dilation (n = 20), Baldwin vaginoplasty (n = 4), Williams vaginoplasty (n = 3), Davydov vaginoplasty (n = 2), or no treatment (n = 29).

Main outcome measures(s): Mean vaginal depth at follow-up, anatomical treatment success rates at levels of ≥6 cm, ≥7 cm, and ≥8 cm, complications, and resurgery.

Result(s): Mean vaginal depths were 7.4 cm (95% confidence interval [CI] 6.8-8.1 cm), 7.3 cm (95% CI 6.7-7.9 cm), and 8.7 cm (95% CI 7.9-9.5 cm) at follow-up in patients treated by McIndoe vaginoplasty, self-dilation, and coital dilation, respectively. Overall complication rates in the three groups were 35/54 (65%), 21/52 (35%), and 1/20 (5%), respectively. Eighteen (33%) of the patients who underwent McIndoe vaginoplasty needed resurgery.

Conclusion(s): Our findings support the current recommendations of dilation therapy as the first-line treatment of vaginal agenesis and emphasize the relevance of coital dilation in patients able to regularly engage in coital activity. However, further studies of functional outcome and patient satisfaction are needed.

Keywords: Dilation; Mayer-Rokitansky-Küster-Hauser syndrome; müllerian aplasia; vaginal agenesis; vaginoplasty.

Publication types

  • Comparative Study

MeSH terms

  • 46, XX Disorders of Sex Development / diagnostic imaging
  • 46, XX Disorders of Sex Development / epidemiology*
  • 46, XX Disorders of Sex Development / therapy*
  • Adolescent
  • Cohort Studies
  • Congenital Abnormalities / diagnostic imaging
  • Congenital Abnormalities / epidemiology*
  • Congenital Abnormalities / therapy*
  • Denmark / epidemiology
  • Dilatation / adverse effects
  • Dilatation / methods
  • Female
  • Follow-Up Studies
  • Humans
  • Mullerian Ducts / abnormalities*
  • Mullerian Ducts / diagnostic imaging
  • Postoperative Complications / diagnosis
  • Postoperative Complications / epidemiology*
  • Prospective Studies
  • Registries
  • Treatment Outcome
  • Vagina / abnormalities*
  • Vagina / diagnostic imaging
  • Young Adult

Supplementary concepts

  • Mullerian aplasia
  • Vagina, absence of