Anaesthetic management of a known case of Werner syndrome for excision of left frontal meningioma

Introduction: Progeria or premature ageing is a rare congenital abnormality in children, with a higher risk of complications during sedation or anaesthesia, due to their challenging airway anatomy and the potential for cardiovascular events. We report the successful anaesthetic management using general anaesthesia of a known case of Werner syndrome. Case Summary: A 34 year old, 37 kg, male presented with a history of numbness and tingling sensations in right upper and lower limb with blurred vision. Investigations revealed a meningioma in the left frontal region and patient was posted for craniotomy for tumor excision. Progeria was diagnosed during the preoperative work-up based on physical appearance and history of diabetes mellitus, hypertension, hypothyroidism, chronic kidney disease and early onset bilateral cataract. His GCS was 15 and vital parameters were normal. Airway assessment was normal. Routine investigation showed FBS: 210 mg/dl, creatinine: 2.7 mg/dl and potassium: 4.5 mEq/L. ECG showed ST segment elevation in V1 - V6 and LVH, baseline echo showed EF = 55% with global hypokinesia. Anticipating a difficult airway, difficult airway trolley was kept ready in OR. Preoxygenation preceded invasive monitoring. Standard intravenous induction with etomidate 0.3 mg/kg, and after confirming adequate mask ventilation, Atracurium 0.5 mg/kg was given and patient was intubated. Anesthesia was maintained with oxygen: Air-50%, sevoflurane (MAC 0.5-0.6) and atracurium infusion. The patient was reversed, extubated and shifted to ICU. Procedure was uneventful. Conclusion: Anaesthetic problems encountered in a case of progeria include difficult airway and myocardial infarction. Prior IDL to rule out any airway abnormality and cardiac work up to rule out myocardial infarction helps one be better prepared. Anticipation of difficult airway and managing the patient like geriatric age group is a key to management of such patients.