Abstract
Goldenhar syndrome, caused by dysplasia of the first and second brachial arches, means the symptom complex consisting of epibulbar dermoids, skin tags on preauricular areas and vertebral anomalies.
We experienced a rare infant case of this syndrome associated with arachnoid cyst in the right temporal area, communicating hydrocephalus, and two dermoids in the right cerebellopontine angle and the anterior portion of the right petrous pyramid. This 4-month-old girl had the asymmetrical head and the right ear of abnormal shape since birth and visited to our department because of bulging of the right fronto-temporal area. On admission, her head circumference was 46 cm and her right anomalous auricle associated with epibulbar dermoids was noted. CT scan of the brain revealed the arachnoid cyst in the right temporal area, communicating hydrocephalus and two dermoids in the right petrous pyramid which were idnentified by the EMI number. We carried out the ventriculo-peritoneal shunt for hydrocephalus and cranioplasty for the bulging of the temporal bone.
Goldenhar syndrome may rarely been associated with intracranial lesion and the study of CT scan for Goldenhar syndrome should be recommended.
