CHIME syndrome

MeSH: C536729ORPHA: 3474

Overview

CHIME syndrome is a rare ectodermal dysplasia syndrome characterized by ocular colobomas, cardiac defects, ichthyosiform dermatosis, intellectual disability, conductive hearing loss and epilepsy

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 CHIME syndrome, sourced from HPO and Orphanet clinical annotations.

Tall statureAbnormality of the dentitionBrachycephalyEpicanthusHypertelorismShort philtrumAbnormality of the outer earHearing impairmentDepressed nasal ridgeRetinal colobomaStrabismusPtosisHypodontiaMicrodontiaIntellectual disabilityAbnormal dental morphologyAplasia/Hypoplasia of the nipplesAbnormal dermatoglyphicsIchthyosisErythemaThick vermilion borderCleft palateUpslanted palpebral fissureSeizureTetralogy of FallotTransposition of the great arteriesShort footShort palmAbnormal epiphysis morphologyAplastic clavicleCorneal opacityAplasia/Hypoplasia of the phalanges of the handAplasia/Hypoplasia of the phalanges of the toesPulmonary valve atresiaSupernumerary toothAbnormality of the kidneyHydronephrosisAutismHyperkeratosisVentricular septal defectCerebral cortical atrophyFine hairAcute leukemiaOsteolysisHip dislocationSparse hairClubbing of toesSkin ulcer

Classification & Codes

MeSH Code

C536729

Orphanet Code

ORPHA:3474
CHIME syndrome
MeSHC536729
OrphanetORPHA:3474
Treatments0 drug(s)
Symptoms on record48 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO