LB44.6

ICD-11 MMS 2026-01

Uterovaginal malformation due to diethylstilbestrol syndrome

Description

Fetal diethylstilbestrol syndrome is characterised by a group of symptoms likely to occur in children and grandchildren of a woman who was treated while pregnant with diethylstilbestrol (DES). The drug is a synthetic nonsteroidal oestrogen, used in the US until 1971 and in Europe until 1978 to try and prevent miscarriage, premature delivery, and other pregnancy complications. It has been estimated that 25% of female fetuses exposed to DES in utero during the first trimester have subsequently developed genital tract anomalies including vaginal adenosis, cervical malformations, vaginal septae, uterine cavity anomalies, or fallopian tube anomalies causing subsequent fertility problems.

Index terms

  • Uterovaginal malformation due to diethylstilbestrol syndrome

Exclusions from above levels

  • LD2A – Disorders of sex development leading to sexual ambiguity
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LB44.0 – Agenesis or aplasia of uterine body
  • LB44.1 – Hypoplasia of uterus
  • LB44.2 – Unicornuate uterus
  • LB44.3 – Bicornuate uterus
  • LB44.4 – Septate uterus
  • LB44.5 – Congenital fistulae between uterus and digestive and urinary tracts
  • LB44.Y – Other specified structural developmental anomalies of uterus, except cervix
  • LB44.Z – Structural developmental anomalies of uterus, except cervix, unspecified

Foundation entity

Uterovaginal malformation due to diethylstilbestrol syndrome – http://id.who.int/icd/entity/1134098724

Part of: LB44: Structural developmental anomalies of uterus, except cervix › 20: Developmental anomalies