Brown-Vialetto-Van Laere (BVVL) Sendromlu İki Olguda Anestezi Yönetimi: Olgu Sunumu

Brown-Vialetto-Van Laere (BVVL) syndrome is a disease characterized by lower cranial nerve involvement, lower and upper motor neuron extremity findings, and sensorineural deafness. Respiratory failure (the most common non-neurological finding), muscle weakness, and slurred speech are other features. Less than 100 patients have been reported in Europe in the last century. Since it is a very rare disease, there is not enough data in the literature regarding the anesthesia approach. In motor neuron diseases, there is a risk of prolonged respiratory depression with general anesthesia because neuromuscular blocking drugs can increase paralysis and muscle weakness and residual neuromuscular block may occur. However, since the etiopathogenesis of BVVL syndrome remains unclear, our general anesthesia management in two different patients with BVVL syndrome is described. Keywords: Brown-Vialetto-Van Laere syndrome, pontobulbar palsy, sensorineural deafness, malignant hyperthermia