Volatile-Free Total Intravenous Anesthesia for Major Gynecologic Surgery in a Manifesting Female Carrier of Becker...

Becker muscular dystrophy (BMD) is a rare X-linked dystrophinopathy caused by dystrophin gene (DMD gene) variants, resulting in reduced or dysfunctional dystrophin. Despite a milder course than Duchenne muscular dystrophy, patients face significant perioperative risk from skeletal muscle degeneration, respiratory weakness, cardiomyopathy, and conduction abnormalities. Succinylcholine and volatile anesthetics have been associated with life-threatening hyperkalemia, rhabdomyolysis, and malignant hyperthermia-like reactions in dystrophinopathies. Clinically manifest BMD in females is exceptionally rare due to skewed X-inactivation, and perioperative data in this population remain scarce. We report a 39-year-old woman with genetically confirmed, clinically manifested BMD who underwent elective left salpingo-oophorectomy with surgical staging for a large adnexal mass. She had severe proximal weakness and was non-ambulatory but had no respiratory, swallowing, or aspiration symptoms. Multidisciplinary preoperative evaluation (neurology, cardiology, hepatology, anesthesiology, gynecologic oncology, intensive care) confirmed preserved cardiac function (ejection fraction >70%), normal conduction, no respiratory compromise evidenced by normal pulmonary function tests, and adequate hepatic reserve. A trigger-free anesthetic strategy was used: the workstation was prepared to eliminate residual volatile agents, and total intravenous anesthesia was maintained with propofol, dexmedetomidine, and midazolam, avoiding succinylcholine and volatile agents. Reduced-dose atracurium provided neuromuscular blockade, and multimodal opioid-sparing analgesia included bilateral rectus sheath blocks and non-opioid analgesics. Surgery was uneventful, with no hemodynamic instability, arrhythmias, hyperkalemia, or rhabdomyolysis. The patient was extubated in the operating room after full neuromuscular recovery, then electively admitted to the ICU for observation given the risk of delayed respiratory compromise. Her postoperative course was uncomplicated, with stable cardiorespiratory function and effective analgesia. Histopathology confirmed an adult granulosa cell tumor of the ovary. This case suggests that major gynecologic surgery can be safely performed in clinically manifest female BMD patients through multidisciplinary planning, trigger-free total intravenous anesthesia, careful neuromuscular blockade, opioid-sparing analgesia, and planned ICU surveillance, offering valuable guidance for this rare, high-risk population.