Abstracts / Posters

Anethetic And Critical Care Management Of A Patient With PraderWilli Syndrome

Dr A Srikanth

Institute : YashodaSuperspecality Hospital

Praderwilli syndrome is a rare genetic disorder due to non expression / deletion ofgenes on paternal chromosome­15 with an incidence of 1 in 25000 live births.Itisassociated with multiple comorbidties and is a challenging problem for both anesthesiaand / critical care specialist to manage this rare case in their Practice.

A 21 years young adult male with praderwilli syndrome developedgastroenteritis and AKI. He was also on ventilator was shifted from USA to our center.Atthe entry patient had tracheostomy and full support on ventilaton and required dialysis onregularbasis.His blood pressure was on the lower side and he had pseudomonasinfection in the lung as well severe PAH.This patient was put on appropriate antibioticsand weaned from ventilator in a matter of a week.However he continued to be dialysed onalternatedays.The patient couid not tolerate the tolerate the weaning and decanulation o tracheostomy due to subglottic tracheal stenosis which was revealed on the CT scan with virtual bronchoscopy.A stent was placed in the trachea and patient was ventilated with endotracheal tube.Later patient was weaned and extubated.He made uneventful recovery.Herecevied steroids in the view of low cortisol levels.The case will be discussed in detail

KEYWORDS:Praderwillisyndrome,ventilator,tracheostomy, tracheal stenosis