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Donnai-Barrow syndrome

Disease definition

Donnai-Barrow syndrome (DBS) is a rare, often severe, multiple congenital malformation syndrome with typical facial dysmorphism, ocular findings, hearing loss, agenesis of the corpus callosum, and variable intellectual disability. Congenital diaphragmatic hernia (CDH) and/or omphalocele are common.


  • Synonym(s):
    • DBS/FOAR syndrome
    • Diaphragmatic hernia-exomphalos-hypertelorism syndrome
    • Diaphragmatic hernia-hypertelorism-myopia-deafness syndrome
    • FOAR syndrome
    • Facio-oculo-acoustico-renal syndrome
    • Holmes-Schepens syndrome
    • Syndrome of ocular and facial anomalies, telecanthus and deafness
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Neonatal, Antenatal
  • ICD-10: Q87.8
  • OMIM: 222448
  • UMLS: C1857277
  • MeSH: C536390
  • GARD: 1899
  • MedDRA: -

Detailed information


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