Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents for evaluation of Oculo-Auriculo-Vertebral (OAV) spectrum features. Clinical history significant for unilateral craniofacial microsomia, preauricular appendages, and epibulbar dermoids. Associated vertebral anomalies and cardiac/renal involvement noted. Current focus on staged reconstruction of auricular and mandibular hypoplasia.
Clinical Examination Findings
Physical exam reveals asymmetric facial development with mandibular hypoplasia. Ocular findings: epibulbar dermoids, microphthalmia, or coloboma. Auricular findings: microtia (Grade I-IV), preauricular tags, or fistulae. Skeletal: cervical spine fusion or hemivertebrae. Neurological: intact cranial nerves, no focal deficits.
Treatment Protocol
Multidisciplinary management plan initiated. Surgical intervention: staged autologous rib cartilage or porous polyethylene ear reconstruction (Medpor). Mandibular distraction osteogenesis for facial asymmetry. Orthodontic alignment and orthognathic surgery planning. Referral to ophthalmology for dermoid excision and pediatric cardiology for structural assessment.