Case Report Open Access

Anaesthetic Management of Two Patients of Steinert Syndrome (Myotonic Dystrophy-Type 1) for Emergency Surgery

Abstract

Anaesthesia for Myotonic dystrophy type 1 (DM1), also known as Steinert's disease, is a genetic disorder that mainly affects muscle function and is characterized by progressive muscle weakness and loss of muscle mass. The involved muscles often goes into sustained contraction and fail to relax (myotonic crisis) under physical, chemical, thermal or electrical stimulations. Patients with DM1 are hypersensitive to too many anesthetic drugs, especially muscle relaxants and opioids. Anesthesia for DM1 patients often results in cardiovascular, skeletal and pulmonary complications. Postoperative respiratory complications, such as hypoxia, atelectasis, aspiration and respiratory failure are very high in patients with DM-1. Thorough patient evaluation and proper anaesthetic planning and active postoperative care are very important in managing such patients. We encountered two patients with Steinert myotonic dystrophy syndrome for emergency surgeries, one for exploratory laparotomy for perforated appendix and another of acute rectal prolapse for manual reduction and rectopexy. Both cases were successfully managed without using any opioid and muscle relaxant.

Mohapatra S, Aneez A, Upadhyay SP* and Rajamohan N

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