Myotonic Dystrophy: An Anaesthetic Dilemma

SUMMARY: Myotonic dystrophy (dystrophia myotonica, DM) is a chronic, slowly progressing, highly variable inherited multisystemic disease that can manifest at any age from birth to old age. We present a 32-year-old female with adenexal mass posted for exploratory laparotomy. She was a known case of dilated cardiomyopathy (DCMP).The ECG suggested incomplete RBBB & LAHB & the ECHO revealed mild mitral regurgitation, tricuspid regurgitation, pulmonary artery hypertension with severe left ventricular dysfunction (ejection fraction of 30-35%). General anaesthesia (GA) with epidural anaesthesia was planned. The patient was haemodynamically stable through out the surgical procedure. The patient was reversed and shifted to post anaesthesia care unit. On the 2nd postoperative day patient developed respiratory distress and hypotension. ABG revealed Type 1 respiratory failure. Since the patient didn't improve with oxygen therapy and nebulisation, she was intubated and shifted to ICU. Patient was tolerating the tube without sedation and relaxants so, consultant anaesthesiologist asked for neurologist referral to rule out myotonic dystrophy. Subsequent muscle biopsy and genetic analysis was suggestive of myotonic dystrophy. Despite all possible efforts we were unable to wean her off the ventilator for 390 days. Patients with myotonic dystrophy are a challenge to the attending anaesthesiologist. These patients can be very well managed with preoperative optimized medical treatment and well-planned perioperative care.