axial spondylometaphyseal dysplasia

MeSH: C535795ORPHA: 168549

Available Treatments (0)

No treatments linked yet

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Clinical Presentation

Signs and symptoms associated with axial spondylometaphyseal dysplasia, sourced from HPO and Orphanet clinical annotations.

Rod-cone dystrophyRetinal dystrophyShort ribsNarrow chestDeformed rib cageCupped ribsGrowth delayAbnormal ilium morphologyShort statureThoracic hypoplasiaAbnormal proximal femoral metaphysis morphologyReduced visual acuityIliac crest serrationUpper limb undergrowthAbnormality of the thoracic cavityPlatyspondylyNeonatal respiratory distressCoxa varaProximal femoral metaphyseal irregularityAbnormal metacarpal morphologyFlaring of lower rib cageFlared, irregular rib endsAplasia/Hypoplasia of the ribsAplasia/Hypoplasia of the vertebraeFlattened femoral headRespiratory tract infectionIncreased upper to lower segment ratioPtosisCataractPhotophobiaNystagmusAmblyopiaOptic atrophyOsteopeniaDelayed ossification of carpal bonesMild postnatal growth retardationScoliosisHypoplastic iliac wingThoracic scoliosisAcromesomeliaFlat acetabular roofNarrow greater sciatic notchDisproportionate short-trunk short statureDyschromatopsiaPeripheral retinal degenerationPosterior wedging of vertebral bodiesShort femoral neck

Classification & Codes

MeSH Code

C535795

Orphanet Code

ORPHA:168549
axial spondylometaphyseal dysplasia
MeSHC535795
OrphanetORPHA:168549
Treatments0 drug(s)
Symptoms on record47 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO