Article Text
PDF
Abstract
Background and Aims Mccune Albright syndrome (MAS) consists of a triad of Fibrous dysplasia, café-au-lait macules and autonomous hyperfunctoning endocrinopathies, caused by mutation in Gsα subunit of G-protein coupled receptors (GPCRs). Anaesthetic management of these patients could be complicated by difficult airway, increased risk of perioperative arrhythmias, other manifestations of associated endocrinopathies and pathological fractures.
Methods We present the anaesthetic management of a 13 years old boy, case of MAS posted for optic nerve decompression. He had history of multiple fractures involving upper and lower limbs. Drug history included tab carbimazole for hyperthyroidism, calcitriol, calcium and sodium acid phosphate for dysplastic bone. Airway examination revealed coarse facial features with large head and tongue with restricted neck movement towards the right side. NCCT orbit revealed narrowed optic canal secondary to expanded dysplastic bone, causing vision loss in right eye. On the day of surgery, radial artery cannulation was done under local anaesthesia. Intravenous induction was done with fentanyl, propofol and atracurium along with dexmedetomidine infusion. C-Mac video laryngoscope and bougie guided intubation done.
Results Anaesthesia was maintained with oxygen, nitrous oxide, isoflurane, atracurium boluses and dexmedetomidine infusion. Patient positioning was done with extreme care. Paracetamol, ketorolac and morphine were given for pain control. Surgery was uneventful. The patient was shifted to ICU for postoperative monitoring and extubated the next day.
Conclusions Throughout surgery, the patient had increased requirement of anaesthetic agents. GPCRs have been considered as potential targets of inhalational anaesthetics, whether this could have an implication on patients with MAS needs further research.