Rapid-Onset Obesity, Hypothalamic Dysregulation, Hypoventilation, Autonomic Dysregulation, and Neuroendocrine Tumour: Rare Syndrome with Myriad Anaesthesia Implications.

Abstract

Anaesthesia management of a child with rapid-onset obesity, hyperphagia, and hypothalamic dysfunction syndrome is complex due to the multisystem involvement, the most important features being morbid obesity, autonomic dysfunction, and dyselectrolytemia due to hypothalamic dysfunction. The acronym of the disease was amended in 2008 to rapid-onset obesity, hyperphagia, hypothalamic dysfunction neural crest tumour to include the risk of ganglioneuroma or ganglioneuroblastoma. Patients usually require removal of tumour in the prone position. Obstructive sleep apnea, difficult airway and intravenous access, and haemodynamic lability all add to the trials faced by the paediatric anaesthesiologist. Invasive haemodynamic monitoring, ultrasonography, bispectral index monitoring, and meticulous calculation of drug dosages help in smoothening the course of anaesthesia in the presence of constant vigilance.