autosomal dominant spondylocostal dysostosis

ORPHA: 1797

Overview

Autosomal dominant spondylocostal dysostosis is a very rare and mild form of spondylocostal dysostosis characterized by vertebral and costal segmentation defects, often with a reduction in the number of ribs

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 autosomal dominant spondylocostal dysostosis, sourced from HPO and Orphanet clinical annotations.

Intrauterine growth retardationScoliosisVertebral segmentation defectSevere short statureProminent occiputWide nasal bridgeAnteverted naresShort neckUpslanted palpebral fissureHyperlordosisShort thoraxAbnormal morphology of female internal genitaliaCleft palateMicrocephalyMacrocephalyAbnormal rib morphologyPosterior rib fusionMissing ribsRecurrent respiratory infectionsSpina bifida occultaAbnormal sacrum morphologyAbnormal cardiovascular system morphology

Classification & Codes

Orphanet Code

ORPHA:1797
autosomal dominant spondylocostal dysostosis
OrphanetORPHA:1797
Treatments0 drug(s)
Symptoms on record22 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
autosomal dominant spondylocostal dysostosis | OrphanDrug