LD20.3

ICD-11 MMS 2026-01

Syndromes with holoprosencephaly as a major feature

Description

Any syndrome caused by failure of the prosencephalon to divide in two during the antenatal period. These syndromes may present with closely spaced eyes, cyclopia, flat nasal bridge, single maxillary central incisor, small head size, and clefts of the lip and palate.

Index terms

  • Syndromes with holoprosencephaly as a major feature
  • XK aprosencephaly
  • Holoprosencephaly - craniosynostosis

Exclusions from above levels

  • LD2F.13 – Meckel syndrome
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Foundation children elsewhere

Sibling codes

  • LD20.0 – Syndromes with cerebellar anomalies as a major feature
  • LD20.1 – Syndromes with lissencephaly as a major feature
  • LD20.2 – Syndromes with microcephaly as a major feature
  • LD20.4 – Syndromes with brain calcifications as a major feature
  • LD20.Y – Other specified syndromes with central nervous system anomalies as a major feature
  • LD20.Z – Syndromes with central nervous system anomalies as a major feature, unspecified

Foundation entity

Syndromes with holoprosencephaly as a major feature – http://id.who.int/icd/entity/1723390898

Part of: LD20: Syndromes with central nervous system anomalies as a major feature › 20: Developmental anomalies