Gökçen KÜLTÜROLU, Jülide ERGİL, Derya ÖZKAN, Aslı DÖNMEZ

MANAGEMENT OF DIFFICULT AIRWAY IN A PATIENT WITH MUCOPOLYSACCHARIDOSIS TYPE 6

Mukopolisakkaridoz tip 6 veya Maroteaux-Lamy sendromu, Glukozaminoglikan (GAG) katabolizmasındaki N-asetil-galaktozamin4-sülfataz enziminin eksikliği nedeniyle dokularda kondroitin sülfat ve dermatan sülfat birikimi ile karakterize bir lizozomal depohastalığıdır. Kısa boyun, makroglossi, gingival hiperplazi, mandibula anormallikleri, hava yolu obstruksiyonu, servikal instabilite gibisebepler havayolu hakimiyetini zorlaştırır. Beraberindeki obstruktif/restriktif akciğer hasarı ve kardiovasküler problemler iseentübasyon sonrasında hemodinamik dengesizliğe neden olarak anestezi idamesini zorlaştırır. Bu olgu sunumunda;adenotonsillektomi ve bilateral ventilasyon tüpü takılan mukopolisakkaridoz tip 6 hastasındaki anestezi yönetimi tartışılmaktadır

MUKOPOLİSAKKARİDOZ TİP 6 HASTASINDA ZOR HAVA YOLU YÖNETİMİ

Mucopolysaccharidosis type 6 or Maroteaux-Lamy syndrome is a lysosomal storage disease which is characterized by theaccumulation of chondroitin sulfate and dermatan sulfate in the tissue due to the lack of the N-acetyl galactosamine 4-sulfataseenzyme. Causes such as short neck, macroglossia, gingival hyperplasia, mandibular abnormalities, airway obstruction, cervicalinstability makes the airline dominance difficult. Accompanying obstructive/restrictive lung damage and cardiovascular problemsas the cause of hemodynamic instability after intubation are the possible difficulties in anesthesia sustainment. In this casereport, anesthetic management in patients who underwent adenotonsillectomy and attached bilateral ventilation tube withmucopolysaccharidosis type 6 are discussed

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