Autosomal recessive spondylocostal…

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Autosomal recessive spondylocostal dysostosis

ORPHA:2311Malformation syndrome

Also called Jarcho-Levin syndrome

What it is

A rare genetic axial skeletal dysostosis characterized by multiple segmentation defects of the vertebrae in combination with abnormalities of the ribs (malalignment, intercostal fusion and possible reduction in rib number), leading to short trunk in proportion to height, short neck and non-progressive scoliosis that is typically mild.

Key facts

Age of onset
Antenatal, Neonatal
Inheritance
Autosomal recessive
Classified as
Malformation syndrome

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

DLL3Disease-causing germline mutation(s)
HES7Disease-causing germline mutation(s)
LFNGDisease-causing germline mutation(s)
MESP2Disease-causing germline mutation(s)
RIPPLY2Disease-causing germline mutation(s)
TBX6Disease-causing germline mutation(s)

ICD-10 codes

Q76.8filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Benefit programs to look at

Programs whose eligibility touches the same ICD-10 categories. Eligibility is decided by the administering agency, never by this page.

Cross-references

GARD 6798MESH C537565MONDO 0010180OMIM 122600OMIM 277300OMIM 608681OMIM 609813OMIM 613686OMIM 616566UMLS C0265343

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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