Our anesthetic management for brainstem evoked response audiometry in a child with Ondine's curse
Yeliz Kılıç, Sema Şanal Baş, Sevil Tultak, Çağdaş Alp Uzan
Osmangazi Üniversitesi, Anesteziyoloji ve Reanimasyon Ana Bilim Dalı
Congenital central hypoventilation syndrome (CCHS), also known as Ondine’s curse, is a rare disease characterized by autonomic respiratory dysfunction without any pulmonary, cardiac or neuromuscular disorder. Here, a case of anesthetic management for brainstem evoked response audiometry (BERA) in a little girl with CCHS was presented. A 2-year-old girl with CCHS presented with hearing loss. She was diagnosed with CCSH two months after delivery. She had not respiratory distress, seizure, or gastrointestinal symptom for the last one year, thus did not take any medication. Monitoring included electrocardiography, noninvasive blood pressure and pulse oximetry. After preoxygenation, inhalation induction done by 8% sevoflurane in oxygen. Subsequently, anesthesia was induced with remifentanyl (2 μg/kg), lidocaine (2.5 mg/kg), and propofol (4 mg/kg), without a muscular relaxant. Laryngeal mask placement was performed using 2.5 no mask. Anesthesia was maintained with (50%/50%) medical air/oxygen and 2% sevoflurane (via inhalation). The test was lasted one hour without any complication. She was closely followed up in the ward for 24 hours. She was uneventfully discharged the next day. The main concern to the anesthesiologists is the failure in control of ventilation. The patients with CCHS are more sensitive to the central respiratory depressant effects of opioids, preoperative anesthetics, anxiolytics, inhalation agents, muscular relaxants, and benzodiazepines. Sevoflurane seems to be more suitable due to its rapid establishment of an anesthetic concentration and lack of airway irritation. CCHS is often accompanied by GERD which is associated with increased risk of aspiration. Anesthetic agents with short activity, such as sevoflurane, remifentanil, and nitrous oxide, may be suitable choices. Because seizure is common, anticonvulsant drugs should not be discontinued throughout the perioperative period. Anesthesiologists should be aware of this syndrome and associated disorders. Detailed preoperative evaluation and appropriate medications are of great importance for a successful anesthetic management.
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