LD21.0

ICD-11 MMS 2026-01

Syndromes with microphthalmia as a major feature

Description

Syndromes in which abnormally small eyes form an important component.

Index terms

  • Syndromes with microphthalmia as a major feature
  • Microphthalmos associated with syndromes
  • Microphthalmos associated with MIDAS syndrome
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  • Microphthalmos associated with Fryns syndrome
  • Microphthalmos associated with Branchio-oculo-facial syndrome
  • Microphthalmos associated with Delleman syndrome
  • Microphthalmos associated with Lenz microphthalmia
  • Microphthalmos associated with growth retardation, ocular abnormalities, microcephaly, brachydactyly and oligophrenia
  • Microphthalmos associated with GOMBO
  • Microphthalmos associated with other syndromes
  • Syndromic nanophthalmos with Kenny-Caffey syndrome
  • Syndromic nanophthalmos with autosomal dominant vitreoretinochoroidopathy
  • Syndromic nanophthalmos with ADVIRC - [autosomal dominant vitreoretinochoroidopathy]
  • Microphthalmia, Lenz type
  • Syndromic microphthalmia type 1
  • Oculo-facio-cardio-dental syndrome
  • Syndromic microphthalmia type 2
  • Anophthalmia or microphthalmia - oesophageal atresia
  • Syndromic microphthalmia type 3
  • Microphthalmia - ankyloblepharon - intellectual deficit
  • Syndromic microphthalmia type 4
  • Syndromic microphthalmia due to OTX2 mutation
  • Syndromic microphthalmia type 5
  • Bakrania-Ragge syndrome
  • Syndromic microphthalmia type 6
  • Microphthalmia, dermal aplasia, and sclerocornea syndrome
  • MIDAS syndrome
  • Microphthalmia, dermal aplasia, and sclerocornea
  • Syndromic microphthalmia type 7
  • Microcephaly, microphthalmia, ectrodactyly of lower limbs, prognathism syndrome
  • MCOPS8
  • MMEP syndrome
  • Microcephaly - microphthalmia - ectrodactyly of lower limbs - prognathism
  • Syndromic microphthalmia type 8
  • Viljoen-Smart syndrome
  • Anophthalmia - pulmonary hypoplasia
  • MCOPS9 - [Syndromic microphthalmia type 9]
  • Matthew-Wood syndrome
  • Syndromic microphthalmia type 9
  • Microphthalmia - brain atrophy
  • Syndromic microphthalmia type 10
  • Ophthalmoacromelic syndrome
  • Microphthalmia with limb anomalies

Exclusions from above levels

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  • LD2A.Y – WAGR syndrome
  • LD41.P – Cat-eye syndrome
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LD21.Y – Other specified syndromes with eye anomalies as a major feature
  • LD21.Z – Syndromes with eye anomalies as a major feature, unspecified

Foundation entity

Syndromes with microphthalmia as a major feature – http://id.who.int/icd/entity/609020523

Allowed postcoordination axes

Part of: LD21: Syndromes with eye anomalies as a major feature › 20: Developmental anomalies