Anesthetic management of a patient with limb-girdle muscular dystrophy 2B:CARE-compliant case report and literature review

Abstract

Background Limb-girdle muscular dystrophies (LGMDs) belong to few neuromuscular disorders mainly involving pelvic and shoulder girdle muscles. Also, cardiac or pulmonary complications, increased rhabdomyolysis risk when exposed to volatile anesthetics and succinylcholine may increase anesthesia related risks. However, current reports about the anesthesia management of these patients are limited. Case presentation We described our anesthetic management of a 36 years old woman with LGMD 2B receiving arthroscopic knee surgery. In consideration of the high risk of rhabdomyolysis, total intravenous anesthesia (TIVA) was selected for her surgery. Considering the unpredictable respiratory depression, opioid based patient-controlled intravenous analgesia was replaced with an intra-articular cocktail therapy consisting of 20 ml of 0.2% ropivacaine. Also, we reviewed the literatures on anesthetic management of LGMD through searching PubMed, in order to provide a comprehensive and safe guidance for the surgery. Conclusions Carefully conducted general anesthesia with TIVA technique is a good choice for LGMD patients. Neuraxial anesthesia may be used if general anesthesia needs to be avoided. To warrant safe anesthesia for surgery, any decision must be well thought out during perioperative period.

Combined spinal-epidural anesthesia for abdominoplasty and liposuction in Limb-Girdle Muscular Dystrophy: case report

“…In a review of the literature, few reports of elective surgeries were found, the majority of the operations being performed under total intravenous anesthesia (TIVA), and few cases with neuraxial anesthesia supplemented with an intrathecal opioid, already included in other studies. 2 , 3 Two operations were performed under neuraxial anesthesia, included in the review by Cao et al, 2 one laparoscopic cholecystectomy with spinal anesthesia technique and one cesarean section with the CSE technique, which included an intrathecal opioid. They performed neuraxial anesthesia to avoid respiratory complications and the need for intubation, invasive ventilation, and neuromuscular blockade, reducing the risks inherent to patients with myopathies, mainly the potential difficulty of weaning from ventilation.…”

“…They performed neuraxial anesthesia to avoid respiratory complications and the need for intubation, invasive ventilation, and neuromuscular blockade, reducing the risks inherent to patients with myopathies, mainly the potential difficulty of weaning from ventilation. 2 In these cases, when neuraxial anesthesia was performed, a local anesthetic (bupivacaine) and intrathecal opioid (fentanyl) were also used, and adequate monitoring in the perioperative period was maintained 3 and in only one case a patient-controlled analgesia device was used in the postoperative period. 2 …”

“… 1 It often occurs with high serum creatine kinase (CK) levels, but normal levels may be present. 1 , 2 The progression of the disease varies widely among individuals, who may have severe impairment of mobility, cardiac and respiratory function leading to decreased life expectancy, 1 , 2 due to this and the variation in the age range of onset of symptoms, the diagnosis can be difficult and may require genetic testing. 2 …”

“…The benefit of diagnosis arises when these patients require surgical interventions, usually orthopedic procedures, since the anesthetic management in these individuals presents some particularities due to the higher risk of cardiac complications from malign arrhythmias, respiratory complications, malignant hyperthermia (HM), and rhabdomyolysis caused by drugs used in anesthesia. 2 , 3 …”

“…Few cases of operations performed in patients with LGMD were reported or included in reviews, 2 , 3 and there is no standardization of the anesthetic technique in these patients, only general recommendations, being necessary to individualize the perioperative care according to the degree of involvement to reduce risks and adverse pharmacological effects related to neuromuscular diseases.…”