Journal of Clinical and Diagnostic Research. 2022 Jul, Vol-16(7): UD01-UD03 1 1 DOI: 10.7860/JCDR/2022/56479.16578 Case Report Anaesthesia Section Anaesthetic Management in a Patient of Goldenhar Syndrome Posted for Drainage of Brain Abscess with External Ventricular Drain Insertion CASE REPORT A 6-year-old male child presented with sudden onset fever 103 o F for five days, four episodes of non projectile vomiting, headache for two days, and two episodes of generalised tonic clonic seizure. The child was diagnosed four years back with Tetralogy Of Fallot (TOF) with Ventricular Septal Defect (VSD) and severe pulmonary stenosis diagnosed on 2D Echocardiogram (2D Echo). Blalock Taussig (BT) shunt surgery was done at 4 years of age. He was known to have Goldenhar syndrome and weighed 14 kg at the time of presentation. Thus, he belonged to American society of Anaesthesiologist (ASA) grading class III. Patient was fully immunized and milestones achieved as per age. The child was not on any anticoagulants. On clinical examination, the child had features of microtia, partially deaf (could hear only loud sounds), coloboma of both eyes, high arched palate and grade 3 clubbing [Table/Fig-1]. Cardiac examination revealed grade 3 pan systolic murmur (TOF with VSD). hypertension and ejection fraction of 60%. There was no evidence suggestive of infective endocarditis. X-ray neck Anteroposterior (AP) and lateral view showed no abnormality, while chest radiograph showed a boot-shaped heart [Table/Fig-2]. Magnetic Resonance Imaging (MRI) brain showed cerebral abscess in left temporoparietal region extending into left ventricle with perilesional oedema [Table/Fig-3]. SHILPA SHANKAR 1 , VIVEK CHAKOLE 2 , MANISH SONKUSALE 3 , SHIVANI DALAL 4 , BHAKTHI PATIL 5 Keywords: Anaesthetic challenges, Cardiac, Congenital, Difficult airway, Peroperative, Oculo auricular vertebral dysplasia ABSTRACT Goldenhar syndrome or Oculo-auricular Vertebral Dysplasia (OAVD) is characterized by a wide range of congenital anomalies including ocular, auricular, facial, cranial, vertebral and cardiac abnormalities. Facial and oral abnormalities especially mandibular hypoplasia, micrognathia, high arch palate and limitation of neck movements resulting from vertebral anomalies, the difficult intubation, laryngoscopy and mask ventilation were expected. Hereby, presenting the case report of a 6-year-old male child, known to have goldenhar syndrome, who underwent drainage of left sided brain abscess with external ventricular drain insertion. In view of the anticipated difficult airway and cardiac anomalies, careful preoperative evaluation, preparation and well formulated contingency plans for airway maintenance, endotracheal intubation and intraoperative haemodynamic is required to combat the perioperative anaesthetic challenges in all cases of goldenhar syndrome. [Table/Fig-1]: a): Grade 3 clubbing; b): Microtia in patient. Patient had a glasgow coma scale score of E3V3M4 with normal tone and power of grade 4/5 in all the 4 limbs. Deep tendon reflexes were present with plantar extensor response bilaterally. Fundoscopy showed papilledema. Haemogram and coagulation profile within normal limits. Blood culture showed no growth. Lumbar puncture and Cerebrospinal Fluid (CSF) study did not reveal any abnormalities. The 2D Echo findings revealed continuous flow across the BT shunt, large VSD with overriding of aorta, stenotic pulmonary valve, severe hypoplasia of pulmonary arteries (6 mm), pulmonary artery [Table/Fig-2]: Chest X-ray showing mild cardiomegaly (boot shaped heart). [Table/Fig-3]: Magnetic Resonance Imaging (MRI) brain showing cerebral abscess left temporoparietal region. (Images from left to right). Anaesthetic Management in Operation Theatre Nil by mouth status was confirmed and informed written consent was taken. Antibiotic prophylaxis (inj. ceftriaxone 50 mg/kg intravenously) was given 30 minutes prior to surgery. Multipara monitors were attached and peripheral venous line 22 gauge secured. De-airing of intravenous line was done and ensured that no air bubbles were present in the i.v. line. Baseline values were recorded as pulse rate of 126 bpm, non invasive Blood Pressure (BP) of 100/60 mmHg, SpO 2 was 92% with 6 litre O 2 and end tidal carbon dioxide concentration value of 22 mm Hg. Preoperatively SpO 2 maintained in 80-85% range (off O 2 ). The patient was given inj. glycopyrrolate 0.004 mg/kg i.v., inj. midazolam 0.05 mg/kg i.v. and inj. fentanyl 2 mcg/kg i.v. Preoxygenation was done with 100% oxygen. Induction was achieved with inj. ketamine 2 mg/kg i.v.