Integrated Advanced Monitoring and Target-Controlled Infusion Anesthesia in a Child With Arthrogryposis Multiplex Congenita

Arthrogryposis multiplex congenita (AMC) is associated with significant anesthetic challenges due to multisystem involvement, craniofacial abnormalities leading to potential difficult airway, thermoregulatory instability, and concerns regarding susceptibility to malignant hyperthermia. Current perioperative practice emphasizes advanced airway planning, quantitative neuromuscular monitoring, tailored hypnotic delivery, and reliable neuromuscular reversal. We describe the perioperative management of a child with AMC using an integrated, technology-guided anesthetic strategy. A 10-year-old, 33.5 kg male with AMC and retrognathia presented for corrective lower-limb orthopedic surgery. He had a previous history of intraoperative hyperthermia. Standard American Society of Anesthesiologists (ASA) monitoring was augmented with entropy-guided depth of anesthesia, quantitative train-of-four (TOF) monitoring, and invasive arterial pressure monitoring. Anesthesia was induced and maintained using target-controlled infusion of propofol and remifentanil. Tracheal intubation was successfully achieved using videolaryngoscopy following rocuronium administration. Multimodal analgesia included a single-shot caudal block with bupivacaine and clonidine. Entropy values were maintained between 40 and 60, with stable hemodynamics and normothermia throughout the procedure. At the end of surgery, sugammadex was administered at a TOF ratio of 0.4, achieving complete neuromuscular recovery prior to awake extubation. Postoperative recovery was uneventful. This case highlights the role of advanced monitoring, sugammadex-facilitated reversal, videolaryngoscopy, and regional anesthesia in enhancing perioperative safety in pediatric patients with AMC.