Mukopolisakkaridozisli Hastaların Anestezi ve Perioperatif Komplikasyonlar Açısından Retrospektif Olarak Değerlendirilmesi

Abstract

Mucopolisaccharidozis (MPS) is a chronic, hereditary, longlasting, progressive, multi eclipsed metabolic disasea which origins from the deficiancy of a specific enzyme in lizozoms with aggregation of glikozaminoglikan. Typical clinical symptoms are; rough face picture; ear, nose, throat problems, spinal displazi, growth retardation, servical instability, servical cord compression, visiual and auditory defects, joint contra, herny and cardiopulmoner problems. The anesthesia of these patients needs special care because of the systemic problems from the frequent operations regarding organ pathologies and the existence of the restriktive and cardiovascüler disasea with the air way obstructions. As a matter of fact, MPS patients show anesthesic problems and higher risks at perioperative, peroperaitve and postoperative stages. Since the MPS patients are a risk group, the aim of this study is to evaluate the anesthesia risk and practice strategies retrospectively with the records of the patients under surgery and anesthesia at our hospital. For his purpose 23 patients’ case which had operations between January 2005- January 2013 were evaulated. Different forms of anaesthesia were recorded in order to evaulate 35 single surgery operations. Patients’ age vary between 2 and 15 years. Patients were mostly under ear nose throat surgery. Most frequently patients were induced by propofol and intravenous. Diffucult intubation was the primer diffullty observed. 22 of 25 direct laryngoscopy performed were succesfull regarding different airway approach. For only one patient emergecy tracheatomy existed. In our study the most frequent respiratory problem was OSAS. All patients had cardiac risks so it is always critical to evaluate the cardiac risks together with the surgeon before operation. There were no clear findings regarding the relation between the age and airway problems incidence. As a result; for MSP patients it is obligatory to approach patients carefully because of diffucult intubation and cardiopulmoner pathologies are risks to be overcomed. The more data collected about the physiopathology of mucopolysaccharidosis patients with a careful team work and usage of advanced airway techniqes, the less mortality at perioperative stage will exist.