AUTHORS:

DR MITHRA, DR SURESH KUMAR, DR SRINIVASAN,DR SUNEETH P LAZARUS.

BACKGROUND:

MAURIAC SYNDROME consists of a triad of poorly controlled diabetes, profound growth retardation, and hepatomegaly which is a rare disorder which proves difficulty to the anesthetist in maintaining the airway during surgery.

CASE REPORT:

A 22 year old male presented with history of RTA 1 month ago. He had head injury associated with loss of consciousness, cervical cord injury and left shaft of humerus fracture with radial nerve palsy. Patient was posted for open reduction and internal fixation with plating. CT BRAIN showed chronic left frontoparietal SDH and cervical cord compression for which neuro surgery opinion was obtained for surgery. Detailed pre-operative assessment was done and found he had developmental delay, short stature, hyperglycemia and dyslipidemia. General medicine opinion was obtained and was suspected to be Mauriac syndrome.
Patient was taken up for surgery with general anesthesia and ultrasound guided supraclavicular brachial plexus block.Premedication was given with inj glycopyrrolate0.005mg/kg, midazolam 0.05 mg/kg, and inj fentanyl 2 mcg/kg and pre-oxygenated with 8L/min O2. He was induced with inj propofol 2 mg/kg and paralysed with inj atracurium 0.5 mg/kg, intubated under video laryngoscope assistance with 8 size endotracheal tube with neck in neutral position. Tube position was verified with bilateral air entry and capnography. Under ultrasound guidance left supraclavicular brachial plexus was blocked with 20 cc of 0.5 % bupivacaine. Adequate level of block was achieved and patient was put in right lateral position with cervical collar. At the end of the procedure the patient was reversed with inj neostigmine 0.05 mg/kg and glycopyrrolate 0.01 mg/kg and extubated after through oral suction and fully awake. Surgery was uneventful and patient was shifted to post anesthesia care unit for monitoring.

Discussion:

Patient with features of Mauriac syndrome characterized by uncontrolled type 1 diabetes, dwarfism, obesity and hepatomegaly had come with head injury associated with loss of consciousness, cervical cord injury and left shaft of humerus fracture with radial nerve palsy. Patient was posted for open reduction and internal fixation with plating, planned for surgery in right lateral position. Maintainingthe airway of this patient would be challenging, hence we have secured the airway with ET tube using video laryngoscopy to avoid undue neck manipulation. For anesthesia and analgesia the supraclavicular brachial plexus block was given. It is important to maintain and secure airway of anticipated difficult airway with cervical cord injury to prevent further exaggeration of cervical cord injury.

CONCLUSION:

Airway was successfully maintained with ET tube using video laryngoscopy to prevent neck manipulation during intubation and positioning of patient intraoperatively.