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goldenhar_syndrome [2015/07/04 02:34] – created admingoldenhar_syndrome [2025/07/14 01:30] (current) – [Eye Findings] Scott Larson
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 +====== Goldenhar Syndrome ======
 +a.k.a oculo-auriculo-vetebral spectrum
  
 +====Main Features====
 +  * Anomalies of structures derived from the first and second bronchial arches
 +  * Hemifacial microsomia (facial asymmetry due to underdevelopment of the mandible and associated tissues)
 +  * Auricular anomalies
 +    * microtia
 +    * preauricular skin tags
 +    * atresia of external auditory canal
 +  * Conductive hearing loss
 +  * Vertebral defects 
 +    * hemivertebrae
 +    * fused vertebrae 
 +
 +====Eye Findings====
 +  * Solid limbal (epibulbar) dermoids
 +  * Micropthalmia
 +  * [[duane_syndrome|Duane Syndrome]]
 +  * Lid colobomas or clefts
 +====Other Findings====
 +  * Facial nerve palsy ipsilateral to microtia
 +  * Cardiac defects
 +  * Kidney anomalies
 +  * One case of neuroblastoma
 +====Etiology====
 +  * Disruption of neural crest cell migration
 +  * Mostly sporadic, most do not have known genetic defects
 +  * rarely 7q21.11 deletion
 +  * occasional single gene-defects
 +====Resources====
 +  - [[https://pubmed.ncbi.nlm.nih.gov/35110414|Tingaud-Sequeira et al., "Oculo-Auriculo-Vertebral Spectrum: New Genes and Literature Review on a Complex Disease" (Journal of Medical Genetics. 2022;59(5):417-427]]
 +  - [[https://pubmed.ncbi.nlm.nih.gov/26068384/ |Puvabanditsin S. et al. "7q21.11 Microdeletion in a Neonate With Goldenhar Syndrome: Case Report and a Literature Review" Cleft Palate Cranifac J. 2016;53(2):249-52]]
 +  - [[https://jamanetwork.com/journals/jamaotolaryngology/fullarticle/509077?utm_source=openevidence&utm_medium=referral |Carvalho GJ et al. "Auditory and Facial Nerve Dysfunction in Patients With Hemifacial Microsomia."Archives of Otolaryngology--Head & Neck Surgery. 1999;125(2):209-12]]
 +  - [[https://pubmed.ncbi.nlm.nih.gov/14632345/|Michel-Adde C. et al. "Goldenhar syndrome and neuroblastoma: a chance association?" Acta Paediatr 2003;92(10):1223-5]]
 +
 +{{tag>syndrome}}