Oculocerebrocutaneous syndrome

MeSH: C538088ORPHA: 1647

Overview

Oculocerebrocutaneous syndrome (OCCS) is a rare congenital disorder associated with an intellectual disability and is typically characterized by the triad of eye, central nervous system and skin malformations

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Oculocerebrocutaneous syndrome, sourced from HPO and Orphanet clinical annotations.

Preauricular skin tagPtosisIntellectual disabilitySeizureCerebellar hypoplasiaPolymicrogyriaAbnormal cerebellar vermis morphologyAplasia/Hypoplasia of the skinSkin tagsCryptorchidismHydrocephalusEyelid colobomaAbnormal rib morphologyHypopigmented skin patchesCalvarial skull defectAlopeciaVentriculomegalyHemiplegia/hemiparesisAplasia/Hypoplasia of the corpus callosumWide mouthOrofacial cleftHypertelorismHearing impairmentIris colobomaNystagmusCongenital diaphragmatic herniaMissing ribsHand polydactylyAbnormal fingernail morphologyDysarthriaDandy-Walker malformationCongenital hip dislocationTalipesTessier cleftFinger syndactylyCorneal opacityShort distal phalanx of fingerAplasia/Hypoplasia of the distal phalanges of the toesExostosesAbnormal pinna morphology

Classification & Codes

MeSH Code

C538088

Orphanet Code

ORPHA:1647
Oculocerebrocutaneous syndrome
MeSHC538088
OrphanetORPHA:1647
Treatments0 drug(s)
Symptoms on record40 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO