429 - Dental Management in a 5-Year-Old With Goldenhar Syndrome

Introduction: Goldenhar syndrome, also known as: oculoauriculovertebral spectrum is a congenital condition that is caused by developmental defects in the first and second brachial arches. It is characterized by a classic triad: unilateral facial asymmetry, ear and/or eye malformations and vertebral anomalies but can also present with renal, cardiac, GI, and central nervous system defects. Dental manifestations include malocclusion, congenitally missing teeth, supernumerary teeth, cleft lip and/or palate, micrognathia, enamel and dentin hypoplasia and gingival hypertrophy. More severe dental presentations include complete absence of the mandibular ramus and TMJ.

Case Report: This presentation discusses a 5-year-old female who presented to Children’s Wisconsin Dental clinic by referral from Plastics.  Patient reported inconsistent pain on lower left side of her face. Patient’s medical history is significant for Goldenhar syndrome with left hemifacial microsomia/microtia/hypoplasia of left mandible.
Patient has history of left mandibular graft necrosis which was partially removed. No known drug allergies or daily medications. Clinical and radiographic examination revealed multiple carious lesions of varying extent, left side tongue fibroma, dental hypoplasia, congenitally missing teeth, and remaining hardwire present in left side soft tissue. Treatment was completed under general anesthesia in coordination with oral surgery. This report will include clinical, radiographic, histological findings and treatment rendered.

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