Abstract: Objective To discuss the perioperative anesthesia management strategy for the surgical resection of rectal cancer in a patient with a rare neuromuscular disease-Facioscapulohumeral Muscular Dystrophy (FSHD) complicated by restrictive ventilatory dysfunction. Methods Clinical data of a patient with FSHD complicated by moderate-to-severe restrictive ventilatory dysfunction undergoing rectal cancer surgery were retrospectively collected; the clinical manifestations, complication, preoperative evaluation, intraoperative anesthesia management and postoperative pain treatment were analyzed and summarized. Results FSHD is a rare genetic disorder, and preopera-tive multidisciplinary evaluation is critical. In this case, total intravenous anesthesia was employed, with invasive arterial pressure monitoring, blood gas analysis, body temperature, sufficient analgesia, close respiratory monitoring and lung protective ventilation strategy after preoperative multidisciplinary evaluation. After thorough sputum suction, lung expansion, and complete recovery of muscle strength, the patient was successfully extubated; ensuring respiratory monitoring after surgery, sufficient analgesia was administered, and transferred to the ICU for monitoring, and ultimately discharged with satisfactory treatment results. Conclusions For patients with rare neuromuscular diseases such as FSHD, thorough preoperative evaluation and optimization are essential. Clinicians should be aware of related complications, such as restrictive ventilatory dysfunction, and develop individualized anesthesia plans. Intraoperative monitoring, particularly of the respiratory and hemodynamic systems, should aim to prevent hypoxia and carbon dioxide retention, thereby reducing the risk of complications.

Key words: muscular dystrophy, restrictive ventilatory dysfunction, surgical treatment, perioperative anesthesia management