otospondylomegaepiphyseal dysplasia

ICD-10: Q77.7ORPHA: 94068

Overview

osteochondrodysplasia that results from mutations in the COL11A2 gene which results in enlargement of the located in epiphysis in located in hand and located in foot, distinct facial features, platyspondyly and hearing loss

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 otospondylomegaepiphyseal dysplasia, sourced from HPO and Orphanet clinical annotations.

Growth delayScoliosisLumbar hyperlordosisDisproportionate short-trunk short statureDysplasia of the femoral headAplasia/hypoplasia involving bones of the extremitiesCoarse facial featuresShort neckPlatyspondylyOsteoporosisKyphosisGenu valgumFlat acetabular roofUpper limb undergrowthFlat faceHip painIncreased head circumferenceAbnormally ossified vertebraeGlossoptosisCleft palateHypertelorismMicrognathiaHearing impairmentRetinal detachmentMyopiaMotor delayBarrel-shaped chestAbnormal foot morphologySpinal cord compressionWaddling gaitAbnormal respiratory system physiologyLimited elbow movementShort long boneShort femurSpinal rigidityBack painReduced bone mineral densityCervical instabilityLaryngotracheomalaciaSmall epiphysesKnee painShort femoral neck

Classification & Codes

ICD-10 Code

Q77.7

Orphanet Code

ORPHA:94068
otospondylomegaepiphyseal dysplasia
ICD-10Q77.7
OrphanetORPHA:94068
Treatments0 drug(s)
Symptoms on record42 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
otospondylomegaepiphyseal dysplasia | OrphanDrug