Anesthetic Considerations in a Patient with Myotonic Dystrophy for Hip Labral Repair

Abstract

Myotonic Dystrophy (DM) affects multiple organ systems. Disorders such as hyperthyroidism, progressive musculoskeletal weakness, cardiac dysrhythmias, hypoventilation, and cognitive-behavioral disorders may be present in these patients. Thorough preoperative assessment and anesthetic planning are required to minimize the risk of anesthetic complications. Patients with DM can exhibit exquisite sensitivity to sedatives, neuromuscular blocking agents, and volatile anesthetics, resulting in potential postoperative complications. There is limited literature available on successful anesthetic techniques for the DM patient. We present this case report to add to our current fund of knowledge.

General Anaesthesia in the Management of Myotonic Dystrophy Patient Posted for Tympanoplasty

“…Patients may present with different symptomatology as it affects multiple organ system (table 1). 2,4,5 Hence multidisciplinary approach and assessment of the patient is critical to successful perioperative management. In our case the boy was 17-year-old with positive history of congenital cataract and generalized mild muscle impairment scale 2 ( Table 2).…”

“…Perioperati ve monitoring is very essential for the smooth recovery of the patient. 2 Continues 12 lead ECG, SpO2, NIBP and temperature monitoring done. These patients are prone for atrioventricular arrythmias and common cause of sudden deaths of 47% while respiratory complications lead to 29.2% of deaths.…”

“…Myotonia may be precipitated by hypothermia, shivering so temperature monitoring is vital intraoperatively and it is achieved with help of warm blanket, warm IV fluids throughout the procedures. 2,8 Postoperati ve respiratory and cardiovascular complications are high due to prolonged sedative effect of opioids, neuromuscular blockade drugs hence observation for 24 hour is necessary in intensive care unit. 8 For post OP analgesia inj.…”

“…2, Patients with DM can exhibit exquisite sensitivity to sedatives, neuromuscular blocking agents and volatile anaesthetics resulting in potential perioperati ve complications. 2,3 Thus meticulous preoperative assessment and proper anaestheti c planning are prerequisite for successful anaesthetic management. We report a case of myotonica dystrophy type 1 (DM1) with CSOM posted for tympanoplasty under general anaesthesia.…”

Successful Anesthetic Management of Myotonic Dystrophy Type I Using Sugammadex: A Case Report

“…1명이며, 전 세계 인구 10만 명당 2-14명 정도로 알려져 있다[1,7].근이영양증은 제1형과 제2형의 두가지 타입으로 나뉘며, 제1형 근 이영양증(Steinert disease)은 신생아부터 성인까지 모든 연령대에 서 발생할 수 있고, 반면 제2형 근이영양증(proximal myotonic myopathy)은 성인에게서만 발생하며, 증상의 중증도는 제1형에서 심한 것으로 알려져 있다[1,3]. 제1형 근이영양증은 다시 그 발병시기에 따라 선천성(congenital), 청소년기 발현성(childhood-onset), 성인기 발현성(adult-onset)으로 나뉜다.…”