High Le Fort I and bilateral split sagittal osteotomy in Crouzon syndrome

Area of Science:

• Genetics
• Craniofacial Surgery
• Anesthesiology

Background:

• Crouzon syndrome, an autosomal dominant genetic disorder caused by fibroblast growth factor receptor 2 gene mutations, leads to premature craniosynostosis and characteristic facial abnormalities.
• Patients often present with jaw malocclusion and obstructive respiratory symptoms, necessitating surgical intervention.
• Midface advancement surgery, such as High Le Fort I and bilateral split sagittal osteotomy, is employed to correct these deformities.

Observation:

• A 32-year-old adult patient with Crouzon syndrome underwent elective midface advancement surgery.
• The surgical procedure involved significant manipulation of the midface, posing risks to the airway and ocular structures.
• Permissive hypotensive anesthesia was utilized to optimize the surgical field and minimize blood loss.

Findings:

• The surgery was uneventful, with the patient extubated successfully post-operation.
• Perioperative considerations included potential airway difficulties, prevention of ocular, auditory, and neurological injuries, and specialized anesthetic techniques.
• Effective postoperative analgesia and multidisciplinary team communication were crucial for recovery.

Implications:

• This case highlights the importance of understanding the multisystemic implications of Crouzon syndrome for anesthetic management.
• Successful surgical correction and recovery depend on meticulous perioperative planning and execution.
• Effective communication among surgical, anesthetic, and nursing teams is vital for managing complex craniofacial cases.