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goldenhar_syndrome [2025/04/18 20:40] – external edit 127.0.0.1goldenhar_syndrome [2025/07/14 01:30] (current) – [Eye Findings] Scott Larson
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 ====== Goldenhar Syndrome ====== ====== Goldenhar Syndrome ======
 +a.k.a oculo-auriculo-vetebral spectrum
  
-  solid limbal dermoids +====Main Features==== 
-  * lid colobomas +  Anomalies of structures derived from the first and second bronchial arches 
-  * pre-auricular skin tags +  * Hemifacial microsomia (facial asymmetry due to underdevelopment of the mandible and associated tissues) 
-  * vertebral abnromalities +  * Auricular anomalies 
-  * Associated with [[duane_syndrome|Duane Syndrome]]+    * 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}} {{tag>syndrome}}