Anesthesia management in a patient with Waardenburg syndrome

Abstract

Waardenburg syndrome (WS) is most often inherited as an autosomal dominant trait characterized with complete or partial absence of melanocytes from the beginning of embryogenesis. Ten year old patient with WS underwent cochlear implant operation because of hearing loss in the left ear. Physical examination was normal except a white forelock, heterochromia of iris, characteristic bright blue iris in the right eye, brown iris in the left eye, broad nasal root, partial synophrys, bilateral sensorineural hearing loss, hypopigmented areas on the 2nd, 3rd, 4th and 5th fingers of the left hand and the wrist. Airway examination was evaluated as Mallampati II. After premedication with midazolam, anesthesia was induced with 1 µgr kg-1 fentanyl, 2.5 mg kg-1 propofol. Endotracheal intubation was facilitated by 0.6 mg kg-1 atracurium. The maintenance of anesthesia was provided with intravenous infusion of propofol (0.75 µgr kg-1 min-1) and remifentanil (0.25 µgr kg-1 min-1) has been used. After the operation the patient was extubated and transferred to the ward without problem. We believe that, awareness of the characteristics of WS, well-planned anesthesia management, controlled hypotension and the use of total intravenous anesthesia are the essentials of the successful anesthesia management in these cases.