Abstract
Alkaptonuria is an uncommon autosomal recessive disorder of tyrosine metabolism. It poses a significant anesthetic challenge due to systemic ochronosis, which is caused by the deposition of ochronotic pigments in connective tissues. We report the case of a 68-year-old male with alkaptonuria who required surgical fixation for a D11 fracture. The patient presented classical features of ochronotic effects comprising scleral melanosis, arthropathy, and constrictive lung functions. The multidisciplinary assessment revealed hypertension, mixed delirium, and low functional capacity. A difficult airway was expected due to rigidity of the cervical spine and possible laryngeal involvement; therefore, tracheal intubation was done with the aid of videolaryngoscopy. Induction and intubation of the case was done with intravenous propofol, fentanyl, rocuronium, sevoflurane and oxygen and maintenance of the anesthesia was done with sevoflurane, rocuronium and oxygen. Invasive hemodynamic monitoring was also done. The patient had an uncomplicated postoperative recovery in the high-dependency unit under careful monitoring. Three important anesthetic considerations concerning alkaptonuria are provided by this case: (1) preoperative evaluation of airway, cardiovascular, and renal systems is mandatory; (2) advanced airway management is required because of the involvement of ochronotic cartilage; (3) a personalized pharmacologic approach should take into consideration possible metabolic changes. The interdisciplinary planning aspect has been proven effective over our experience in maximizing the outcomes of such complex patients.
Keywords
Alkaptonuria, Anesthesia management, Ochronosis, Musculoskeletal complications, Joint degeneration, Connective tissue damage