Dyggve–Melchior–Clausen syndrome

MeSH: C535726ORPHA: 239

Overview

rare skeletal disorder belonging to the group of spondyloepimetaphyseal dysplasias

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 Dyggve–Melchior–Clausen syndrome, sourced from HPO and Orphanet clinical annotations.

Intellectual disabilityFailure to thriveSpondyloepimetaphyseal dysplasiaDisproportionate short-trunk short statureMicrocephalyCoarse facial featuresShort neckPectus carinatumBroad ribsAbnormality of the vertebral columnPlatyspondylyHypoplastic iliaMotor delaySkeletal dysplasiaEpiphyseal dysplasiaShort long boneHypoplasia of the capital femoral epiphysisWide pubic symphysisAbnormal hip bone morphologyHypoplastic acetabulaeAbnormal femoral head morphologySevere short statureLimb muscle weaknessBeaking of vertebral bodiesAbnormality of the vertebral endplatesBroad femoral neckIliac crest serrationRhizomeliaShort thoraxSevere intellectual disabilityHorizontal inferior border of scapulaAbnormal pelvis bone morphologyMetaphyseal dysplasiaDelayed speech and language developmentHyperactivityMild intellectual disabilitySpastic tetraparesisHyperreflexiaLimited elbow extensionProtuberant abdomenFrequent fallsPoor speechInability to walkRespiratory insufficiency due to muscle weaknessRecurrent upper respiratory tract infectionsCoxa varaGenu valgumAbnormality of the ankleLimited knee extensionHypoplasia of the odontoid processAtlantoaxial instabilityDifficulty standingBroad carpal bonesGlenoid fossa hypoplasiaDiminished ability to concentrateGait disturbance

Classification & Codes

MeSH Code

C535726

Orphanet Code

ORPHA:239
Dyggve–Melchior–Clausen syndrome
MeSHC535726
OrphanetORPHA:239
Treatments0 drug(s)
Symptoms on record56 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO