Anaesthesia and airway management of occulo auricular vertebral dysplasia: A rare case report

Abstract

A 12 year old girl child from the department of Ophthalmology posted for right eye limbal dermoid excision. Opthlamic examination and history revealed bilateral restricted eye movements, limbal dermoids, and decreased vision since birth. Fundus examination showed pathological myopia. There were multiple preauricular appendages in front of both ears. The lateral X-ray of cervical spine showed fused second and third cervical vertebra with loss of intervertebral disc space. Chest X-ray showed scoliosis of Cervico-Thorasic vertebra with convexity to right. Clinical examination of the airway revealed restricted head and neck movements with Mallampatti class II which indicates difficult intubation. The history, clinical examination and radiological findings confirmed her to be a case of Goldenhar syndrome. Difficult airway continues to be a major cause of anaesthesia related complications. Therefore fibre optic intubation under regional nerve block and sedation was planned for the surgery. Lack of associated cardiac and neurological anomalies encouraged us to present the advantage of fibre optic intubation. The larynx was adequately relaxed because of regional nerve block which helped us by decreasing the number of attempt of scopy and vocal cords injury. The intraoperative and postoperative period was uneventful.

Keywords

• Fibre optic intubation
• goldenhar syndrome
• occulo-auricular-vertebral dysplasia