ANESTHETIC MANAGEMENT OF A PEDIATRIC PATIENT WITH COCKAYNE SYNDROME FOR CORRECTIVE EQUINOCAVOVARUS DEFORMITY SURGERY

Abstract

Background: Cockayne syndrome (CS) is a rare, autosomal recessive progeroid disorder presenting significant anesthetic challenges, including potential difficult airway, neurological sensitivity, and multiorgan dysfunction. We report the successful anesthetic management of a pediatric patient with CS undergoing corrective orthopedic surgery.

Case Presentation: A 13-year-old male, with a genetically confirmed diagnosis of Cockayne syndrome, presented with bilateral equinocavovarus deformity. His clinical features included microcephaly and developmental delay. He was scheduled for corrective surgery on the right foot. General anesthesia was induced with intravenous thiopentone, with the airway secured uneventfully using a 4.5 mm endotracheal tube. Anesthesia was maintained with sevoflurane and atracurium.

Discussion: The anesthetic plan was tailored to mitigate potential risks associated with CS. Atracurium was specifically chosen for neuromuscular blockade due to its organ-independent metabolism, a prudent choice in a syndrome with potential subclinical systemic dysfunction. A landmark-guided ankle block was utilized as part of a multimodal analgesic strategy to minimize systemic opioid requirements and the associated risk of postoperative respiratory depression in a neurologically vulnerable patient.

Conclusion: The intraoperative and postoperative periods were uneventful. This case demonstrates that with meticulous preoperative evaluation, a tailored anesthetic plan, and a multimodal approach to analgesia, patients with Cockayne syndrome can be safely managed for major orthopedic procedures.