ABSTRACT
Our case report is regarding a patient who is a k/c/o citrullinemia type 1 which is a defect in urea cycle posted for facture patella reduction surgery (tension band wiring). He was intellectual disabled because of persistent hyperammonaemia before diagnosis that was controlled with benzoate and L- arginine. Patient preoperative fasting was kept to the minimum and taken as 1st case in the operation theatre. Drugs taken orally on routine basis were continued along with serum ammonia monitoring. Clonidine with midazolam along with propofol infusion to decrease protein hypercatabolism due to stress and to get co-operation of the patient for femoral sciatic block. Postoperative ammonia levels were normal. Sedation is considered useful for preventing hyper ammonemia. Preoperative endocrinology consultation, perioperative serum ammonia level monitoring and coordination between various health departments (nephrologist and endocrinologist) for appropriate care in case of hyper ammonemia and hyperglycaemia perioperatively.