Anesthetic Management of Prader Willi Syndrome pediatric patient undergoing Tonsillectomy Surgery: A Case Report
Begüm Nemika Gökdemir, Nedim Çekmen
Department of Anesthesiology, Baskent University Faculty of Medicine
Giriş: Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by various abnormalities, such as hypotonia, high body mass index, craniofacial anomalies and OSA. Anesthetic management of these patients requires special planning. In this case report we present the anesthetic considerations and perioperative management.
Olgu: We present a case of a 9 years old male patient, with PWS scheduled for adenoidectomy-tonsillectomy surgery, for OSA treatment. His growth was slower than the normal pattern and receiving GH. Preoperative physical examination was unremarkable concerning respiratory or cardiac symptoms but no significant findings were found. Also revealed a large tongue relative to mouth, malocclusion and a large mandibula. The Mallampati score was III. In the OR, standard monitoring was applied to the patient and a heated blanked was also applied under the patient to prevent hypothermia. Anesthesia was induced initially with sevoflurane inhalation and an iv line was inserted. Anesthesia induction was achieved after intubation was facilitated with rocuronium and ETT was inserted uneventfully with videolaryngoscopy. After intubation, we connected patient to ventilator and for anesthesia maintenance sevoflurane and remifentanil infusion were used. Intraoperatively after ten minutes, a drop of SpO2 was noted (from 98% to 92%). Widespread wheezing was revealed on auscultation. Misplacement and occlusion of tracheal tube and breathing circuit were ruled out. With elevated airway pressure , bronchospasm was diagnosed. FiO2 and sevoflurane was increased, propofol, salbutamol and methylprednisolone was given. Manual ventilation and recruitment maneuvers performed. Five minutes later, SpO2 gradually improved. After that, surgery proceeded uneventfully.
Tartışma ve Sonuç: PWS is a genetic disorder that poses a significant challenge to anesthesiologists. Detailed physical examination and precision preparation are necessary in order to reduce morbidity and mortality. Early diagnosis of GH deficiency and its treatment has improved the quality of life of affected children. A multidisciplinary approach and comprehensive perioperative management may prevent complications.
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