Nager acrofacial dysostosis

MeSH: C538184ORPHA: 245

Overview

acrofacial dysostosis characterized by underdeveloped cheek bones, very small lower jaw, cleft palate, defects in the middle ear, absent eyelashes, and a notch in the lower eyelids called a coloboma, in children

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 Nager acrofacial dysostosis, sourced from HPO and Orphanet clinical annotations.

Hypoplasia of the maxillaMicrognathiaHearing impairmentDownslanted palpebral fissuresDelayed speech and language developmentSkeletal dysplasiaAplasia/Hypoplasia of the thumbHypoplasia of the zygomatic boneWide mouthAbnormal palate morphologyCleft palateAtresia of the external auditory canalPtosisLower eyelid colobomaJoint stiffnessRespiratory insufficiencyHypoplasia of the radiusAbnormal nasal morphologyAplasia/Hypoplasia of the radiusSparse lower eyelashesMicrotiaAplasia/Hypoplasia of the eyebrowUnilateral renal agenesisTriphalangeal thumbAbnormality of the lower limbPhocomeliaAbnormal cardiovascular system morphologyNon-midline cleft of the upper lipPosteriorly rotated ears

Classification & Codes

MeSH Code

C538184

Orphanet Code

ORPHA:245
Nager acrofacial dysostosis
MeSHC538184
OrphanetORPHA:245
Treatments0 drug(s)
Symptoms on record29 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
Nager acrofacial dysostosis | OrphanDrug