అనస్థీషియా & క్లినికల్ రీసెర్చ్
అందరికి ప్రవేశం

నైరూప్య

Dexmedetomidine: A Saviour in a Child with Hand Schuller Christian Disease for MRI

Geeta Ahlawat, Savita Saini, Swati Chhabra, Amita Singh, Kirti Kshetrapal and Jaswant Singh

Hand Schuller Christian disease (HSC) primarily affects infants and children. Its classical presentation includes triad of exophthalmos, diabetes insipid us, and calvariallytic lesions. Systemic involvement may include hepatomegaly, lymphadenopathy, dermatological, gastrointestinal tract, renal, pulmonary and CNS involvement. CNS involvement includes convulsions, increased intracranial pressure, focal neurological deficits, mental retardation, hearing disturbance, and tremors. We encountered a 4 year old female child with HSC disease who was posted for elective MRI scan under anaesthesia. Patient’s history, physical examination, and lab reports revealed multitude of problems enumerated in case report. Such patients when posted for anaesthesia pose a major challenge in view of multiple organ involvement. Polydipsia made difficult to keep the patient fasting and giving general anaesthesia as per standard protocol became risky. The loose teeth with bleeding gums and protruded mandible would have compounded the mask ventilation, LMA placement or laryngoscopy and intubation, manifolds. We managed this case successfully with intravenous sedation with inj. dexmedetomidine at 1 μg/kg/min over 10 min and then maintenance infusion at 0.1 μg/kg/min. Spontaneous ventilation was supplemented with oxygen (FiO2 0.60). MRI scanning was completed in 45 minutes comfortably. Hence, dexmeditomidine offers advantage in such patients if anaesthesia has to be given in remote locations like MRI suite.