Anaesthesia for Spinal Muscular Atrophy

摘要

Spinal Muscular Atrophy (SMA) comprises of a group of neuromuscular disorders due to degeneration of anterior horns cells of spinal cords and brain stem. We report the anaesthetic management of a patient for laparoscopic Nissen Fundoplication due to gastroesophageal reflux disease. We used total intravenous anaesthesia with propofol and fentanyl. No muscle relaxant was used. Anaesthetic Management A 5-years old female patient weighing 14 kg, a known case of Spinal Muscular Atrophy type I with gastroesophageal reflux disease (GERD) was scheduled for elective laparoscopic Nissen Fundoplication and gastrostomy.She was diagnosed as a case of Spinal Muscular Atrophy at the age of 6 month with muscle biopsy. Her two elder sisters died due to the same disease. She had also history of repeated admission to hospital due to recurrent pneumonia.She was receiving pantoprazole 15 mg orally once a day, domperidone 1.2 mg orally four times a day, pulmicort inhaler 500 μg three times a day, injection ceforoxime 470 mg intravenously three times a day and heparin flush intravenously three times a day.On physical examination, she was bed ridden but well oriented in time, space and person. She was unable to hold her neck. She never started walking. Upper limb power was grade 3/5 and lower limbs 1/5. Muscles of upper and lower limbs were flaccid and atrophied. Sensory system was intact. Both knees and hip joints were held in flexion position. There was equinovarus deformity of both feet. Palmar surface of both feet was at 90° facing medially. There was tibial extortion as well. She had khyphoscoliosis of spinal column. Anterior rib cage and sternum were protruding out as shown in the figure 1.