[Arthrogryposis multiplex congenita: special anesthesiological aspects]

Area of Science:

• Anesthesiology
• Pediatric Orthopedics
• Medical Genetics

Background:

• Arthrogryposis multiplex congenita (AMC) presents unique anesthetic challenges, including difficult airway management and potential for malignant hyperthermia.
• Patients with AMC often exhibit contractural deformities, skin abnormalities, and vascular anomalies, complicating venous access and intubation.
• This case report focuses on a 2.5-year-old girl with AMC requiring significant orthopedic intervention for equinovarus.

Observation:

• Preoperative challenges included anticipated difficult intubation and venipuncture, necessitating central venous catheter placement under local anesthesia.
• Intraoperative management involved a "trigger-free" anesthetic technique using fentanyl, thiopental, and vecuronium, followed by intubation and ventilation.
• A caudal catheter was utilized for intraoperative anesthetic titration and postoperative analgesia, administered with mepivacaine and bupivacaine.

Findings:

• The anesthetic management was successful, avoiding volatile anesthetics to mitigate the risk of malignant hyperthermia.
• The patient tolerated a 7-hour surgery without adverse reactions.
• Postoperative pain was effectively managed via the caudal catheter, providing excellent analgesia for 24 hours.

Implications:

• This case highlights the feasibility and safety of a "trigger-free" anesthetic approach in pediatric AMC patients.
• Effective regional anesthesia techniques, such as caudal blocks, are crucial for managing postoperative pain in these complex cases.
• Careful preoperative assessment and planning are essential for optimizing anesthetic outcomes in patients with AMC.