Anesthetic Management of a Patient with MIRAGE Syndrome: A Case Report
Begüm Nemika Gökdemir, Nedim Çekmen
Baskent University Faculty of Medicine, Department of Anesthesiology
Giriş: MIRAGE Syndrome is an acronym that stands for Myelodysplasia,Infection,Growth restriction,Adrenal hypoplasia,Genital phenotypes and Enteropathy. This syndrome is characterized by congenital adrenal hypoplasia with extra-adrenal features and is severely mortal therefore most patients die before the age of 2 years. Here, we presented a patient with MIRAGE syndrome undergoing dental surgery in our hospital. Here, we aim to better understand this rare syndrome and learn about convenient and safe anesthesia management.
Olgu: A 6-year-old female patient was admitted to our dentistry clinic due to dental abnormalities. She was diagnosed with MIRAGE syndrome at age 3. She consulted nephrology,endocrinology and immunology in her preoperative assessment. Endocrinology suggested blood glucose level regulation during operation. Nephrology and immunology departments have no additional advice. She has congenital hypothyroidism but has no clinical outcome. Her preoperative physical examination showed poor dental health and restricted mouth opening,so we prepared anesthesia workstation for difficult airway. When she came for surgery,standard monitoring was applied to the patient .Heated blanked was applied under the bed to prevent hypothermia.Anesthesia induction was performed with fentanyl, lidocaine,rocuronium and propofol. After that,we preoxygenated the patient with mask via an 80% fraction of inspired oxygen (FiO2) for 3 minutes and endotracheal intubation was facilitated with 5.5 mm ETT uneventfully at the first attempt. However,we had LMA,gum bougie,tube guide and videolaryngoscope for difficult intubation. Anesthesia was maintained with 2.2% sevoflurane and 40% FiO2-air mixture. We followed blood glucose levels with a glucometer in every 15 minutes.After 5 teeth extraction and hemostasis control, surgery was done without complications.
Tartışma ve Sonuç: Anesthetic management of syndromes are challenging. An anesthesiologist must know all features of the disease and its relative complications. Comprehensive preoperative evaluation and careful anesthesia plan are a must for these population. Because of complexity of syndrome multidisciplinary approach and managing accordingly are essential in treating psychological and functional problems.
|