Abstract

Introduction: Spinal Muscular Atrophy (SMA) is a genetic disorder impacting approximately 1 in 10,000 individuals. It manifests across varying degrees of severity. Generally, the disease's onset at an earlier age corresponds to more pronounced symptoms, such as difficulty with swallowing, breathing, etc. A present case of a two-year-old male child diagnosed with SMA type 2 was planned for feeding gastrostomy under general anesthesia. This case report highlights how awareness of this rare ailment can facilitate early identification, empower parents to seek genetic counseling and help prevent potential complications. Case Report: A 2-year and 10-month-old male child was brought with cough, cold and shortness of breath persisting for three days. Medical history reveals that he had SMA type 2 and underwent Zolgensma. Provisional diagnosis of SMA type2/ bronchopneumonia with respiratory distress was made. His treatment commenced with oxygen support via nasal prongs, IV antibiotics Inj. Collistin 75K/kg/day TID, Inj. Fluconazole 100 mg iv OD, Nebu Asthalin, Nebu Budecort nebulization. Following day, i/v/o respiratory distress, he was connected to high-flow nasal cannula support. The antibiotics regimen was adjusted to include increased doses of PIPTAZ syrup azithromycin and oseltamivir. He was premedicated with Inj. Glycopyrrolate, Inj. Midazolam and Inj. Fentanyl and pre-oxygenation with 100% oxygen and intubated with 4mm uncuffed endotracheal tube and shifted to the OT. Anesthesia was induced with sevoflurane and maintained with a combination of nitrous oxide and oxygen. Skeletal muscle relaxation atracurium was given. The pediatric surgeon performed gastrostomy by inserting MIC-KEY Tube of 12FR through the anterior abdominal wall into the stomach. Throughout the procedure, his hemodynamic parameters remained stable and was transferred to the Pediatric ICU with Ambubag and ET tube ventilation and then transitioned to mechanical ventilator support. Postoperative recovery was uneventful. Conclusion: Individuals with SMA can effectively be administered with general anesthesia, using muscle relaxants and inhalation anesthetics. Addressing specific concerns and ensuring access to advanced airway and imaging equipment are pivotal for safely managing anesthesia in this specialized patient cohort.

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