ESPN 51th Annual Meeting

ESPN 2018


 
Anesthetic Management in Mucopolysaccharidosis type 2 (Hunter syndrome, MPS II): Case Report
CEYDA ÖZHAN ÇAPARLAR 1 EVGINAR SEZER 1

1- HEALTH OF UNIVERSITY DISKAPI YILDIRIM BEYAZIT TRAINING AND RESEARCH HOSPITAL
 
Introduction:

 Mucopolysaccharidosis type 2 (Hunter syndrome) is a rare autosomal recessive metabolic disorder, which is accumulate dermatan and heparan sulfate.This syndrome includes muscle-sketal, cardiovascular, respiration and upper airways organs lead to problems about anesthesia.

Material and methods:

 

An 8 year old boy was referred to Health of university Dıskapı Yıldırım Beyazıt training and research hospital with upper airway obstruction.  The boy had been followed regularly by pediatric units with the diagnosis of MPS. Medical history that he had frequently respiratory infections with obstruction. Permanent trakeostomy opened two monts ago because of dermatan glucose amino glycanaccumulation in upper airway.

Laryngeal structures were not visible even after direct laryngoscope in emery room.

Standard anesthetic monitored and protocol followed. His trakeostomy was connected to mechanic ventilation and manual ventilation started. Surgery of Otolaryngology applied rigid bronchoscopy from trakeostomy. 

            Under general anesthesiarigid bronchoscopy is commonly used for diagnosis respiratuar tract obstruction. We observed granulation tissue and tracheaomalasia in trachea lumens via bronchoscopy. Posterior trachea was narrowed by %50. All respiratory organs could not be distinguished via bronchoscopy. After then tracheal stenosis was treated using the balloon to dilate the trachea for 10s during which the mechanical ventilation was stopped. Subsequently, the pressure started to decrease and the mechanical ventilation was recovered. The balloon dilatation was repeated 3 times. The bronchial lumen expanded only slightly following the procedure. The wheeze and stridor of lungs were reduced under application.

Results:

 Bronchoscopy has become a preferred option in the management of subglottic and bronchial stenosis. In Hunters syndrome, the incidence of airway related problems such as narrowing of airway, laryngomalacia, copious secretions, and pulmonary dysfunction has been reported to be as high as 53% because of restrictive lung disease, thoracic dystrophy, pulmonary hypertension, and obstructive sleep apnea1.

 Local or regional anesthetic techniques may be unsuitable as the sole form of anesthesia in these patients because they may fail as a result of deposition of Mucopolysaccharidosisin the nervous system2,3

Conclusions:

 Patient with Hunter syndrome even with tracheostomy even anesthetic managements is difficult. Rigid bronchoscopy under general anesthesia is safe and effective.