Mükerrem UYSAL, Yusuf ÜNAL, Bayazıt DİKMEN, Sevil BALTACI ÖZEN, Metin ALKAN

Morquio Sendromlu Bir Vakada Anestezi

Morquio Sendromu mukopolisakkaridozların (MPS IV) alt tipi olan ve otozomal resesif geçiş gösteren bir lizozomal depo hastalığıdır. Morquio Sendromlu hastalar adenoid-tonsil hipertrofisi veya ortopedik problemler nedeniyle genel anestezi altında operasyonlar geçirmektedir. Servikal instabilite ve myelopati, göğüs duvarı deformiteleri, üst ve alt hava yolu tıkanıklıkları gibi sorunlar da anestezi yönetimini güçleştirmektedir. Ayrıca hastalardaki yaygın organ ve doku tutulumları morbidite ve mortalitenin artışına katkıda bulunmaktadır. Bu yazıda; Morquio Sendromu tanılı genu valgum nedeniyle bilateral hemiepifizyodez operasyonu planlanan hastada uygulanan anestezi yaklaşımı sunularak literatür eşliğinde tartışılmasını amaçladık

Anesthetic Management in a Patient with Morquio Syndrome

Morquio Syndrome is an autosomal recessive lysosomal storage disease. It is one of the subgroup of mucopolysaccharidosis (MPS IV). Anesthesiologist usually deal with patients diagnosed Morquio Syndrome as they require repeated anesthesia administrations for adenoid and tonsil hypertrophia or ortophedic deformability, upper and lower airway obstructions may complicate the anesthetic management of patients who will undergo surgical procedures. Besides, generalized organ and tissue involvement leads to increase in mortality and morbidity. Our aim is to report and discuss the anesthetic management of a patient with Morquio Syndrome who undergoes hemiepiphysiodesis operation for bilateral genu valgum in the light of current literature.

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