Cenani-Lenz syndactyly syndrome

MeSH: C538150ORPHA: 3258

Overview

dysostosis characterized by syndactyly, malformation of the forearm and lower limb bones, renal hypoplasia or aplasia and that has material basis in homozygous or compound heterozygous mutation in the LRP4 gene on chromosome 11p11

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 Cenani-Lenz syndactyly syndrome, sourced from HPO and Orphanet clinical annotations.

Absent fingernailFrontal bossingSynostosis of carpal bonesAbnormal metacarpal morphologyFinger syndactylyOligodactylySynostosis of jointsHypertelorismDownslanted palpebral fissuresToe syndactylyAbsent toenailRadioulnar synostosisHypoplasia of the radiusHypoplasia of the ulnaShort thumbMalar flatteningShort philtrumHearing impairmentProtruding earConvex nasal ridgePtosisCataractProptosisNystagmusEctropionHypodontiaAbnormal dental enamel morphologyAbnormal rib morphologyHypothyroidismLaryngomalaciaFoot oligodactylyScoliosisHigh, narrow palateHip dislocationMicromeliaElbow dislocationShort noseAbnormal form of the vertebral bodiesCrossed fused renal ectopiaAbnormal dermatoglyphicsRenal hypoplasia/aplasia

Classification & Codes

MeSH Code

C538150

Orphanet Code

ORPHA:3258
Cenani-Lenz syndactyly syndrome
MeSHC538150
OrphanetORPHA:3258
Treatments0 drug(s)
Symptoms on record41 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO