[Anesthetic management of a patient with Coffin-Lowry syndrome]

Area of Science:

• Anesthesiology
• Medical Genetics

Background:

• Coffin-Lowry syndrome (CLS) is a genetic disorder associated with intellectual disability, distinctive facial features, and skeletal deformities.
• Patients with CLS often present with complex medical histories, including cardiac anomalies, necessitating specialized anesthetic management.

Observation:

• A 33-year-old female diagnosed with Coffin-Lowry syndrome, who also had an atrial septal defect and ventricular tachycardia, required elective surgery for an anterior cervical cyst.
• Anticipation of difficult intubation guided the anesthetic approach, prioritizing hemodynamic stability.

Findings:

• Anesthesia was induced using a continuous propofol infusion, followed by vecuronium bromide, midazolam, and fentanyl after confirming mask ventilation.
• The surgical procedure and anesthetic management were completed without intraoperative or postoperative complications.

Implications:

• This case highlights the successful use of a slow propofol induction for maintaining hemodynamic stability in a patient with Coffin-Lowry syndrome and significant comorbidities.
• Careful anesthetic planning and monitoring are crucial for managing patients with CLS undergoing surgical procedures.