Beschreibung:
ABSTRACT Introduction: Arthrogryposis multiplex congenita (AMC) is a group of musculoskeletal disorders characterized by congenital joint contractures. Patients with AMC often require anesthetic care to undergo correction of musculoskeletal deformities, and their peri-operative care can be challenging for anesthesiologists. Case report: We present the case of an infant with AMC, who underwent a two-stage surgical correction of lower limbs deformities. The main concerns were the possibility of a difficult airway management, the risk of hyperthermia and the challenge of achieving adequate pain control in an aggressive reconstructive surgical procedure. For each surgery, two different regional anesthesia strategies were conducted. Discussion: There are several anesthetic considerations for patients with AMC. We highlight the importance of being prepared for a potential difficult airway, risk of malignant hyperthermia, cardiorespiratory problems and difficult regional blockade. In this case, we had a careful airway evaluation, perioperative body temperature monitoring, total intravenous anesthesia, and the use of ultrasound guided regional anesthesia all of which ensured the safe care of this patient. The addition of pre-emptive and continuous regional-analgesia significantly improved the post-operative course. Conclusion: Is important for the anesthesiologist to understand the pathophysiology and potential risks of complications in patients with AMC in order to provide them with safe and optimal anesthetic care. Adequate pre-emptive pain management aided by ultrasound guided continuous regional techniques represents an excellent option that may help improving postoperative results. Highlights: