Neonatal Anaesthetic Management of a Patient with Goldenhar's Syndrome with Hydrocephalus

Goldenhar's syndrome, or oculo-auriculo vertebral dysplasia, is a rare form of hemifacial microsomial-6 in which the facial and vertebral anomalies which are frequently associated with cardiac and pulmonary defects present a difficult technical and physiological challenge for proper anaesthetic management.The approach used in a neonate with Goldenhar's syndrome needing a ventriculoperitoneal shunt is presented.The usefulness of pulse oximetry to assess both oxygenation and cardiovascular tolerance is illustrated.CASE REPORT The 2800 gram infant was born by spontaneous vaginal delivery at 38 weeks to a primigravida healthy mother.Family history and pregnancy were normal.Labour was prolonged and necessitated the application of a vacuum extractor.Birth asphyxia made mask resuscitation necessary and subsequent transient respiratory distress ensued.The patient was referred to us at ten days of age for evaluation of multiple congenital abnormalities including microtia, right preauricular tags, right microphthalmia with epibulbar dermoid, severe hypoplasia of right malar, mandibular and maxillary areas, hypoplasia of third thoracic vertebra, hypoplasia of the right lung and dextrocardia, 'M.