Anesthetic management of an adolescent with Dandy-Walker syndrome

Corresponding author: Jae Jun Lee, M.D., Department of Anesthesiology and Pain Medicine, Chuncheon Sacred Heart Hospital, 153, Kyo-dong, Chuncheon 200-704, Korea. Tel: 82-33-240-5353, Fax: 82-33-251-0941, E-mail: [email protected] This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http:// creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. CC Dandy-Walker syndrome is classically described as a neuropathological triad consisting of hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle, and hydrocephalus. Clinical manifestations of the syndrome usually appear in the first year of life, but can occur during the neonatal period. Asai et al. [1] reported that as many as 89% of patients are diagnosed before 1 year of age. However, in the absence of prenatal diagnosis, the symptoms usually become apparent in early infancy and include slow motor development, bulging anterior fontanel, and progressive enlargement of the skull. We report a rare case of anesthetic experience for an adolescent with Dandy-Walker syndrome. A 13-year-old, 140 cm, 29 kg, male adolescent who had been born prematurely at 28 weeks, weighing 850 g visited our urology for surgical treatment of scrotal hydrocele. The patient had mental retardation and general muscular dystrophy. Six months prior to admission, he had an episode of seizure with vomiting, and brain magnetic resonance imaging was performed. Magnetic resonance imaging showed a cyst in the posterior fossa, hypoplastic cerebellar hemispheres, and elevation of the hypoplastic cerebellar vermis due to the cyst (Fig. 1). He was diagnosed with Dandy-Walker syndrome. In the preoperative evaluation, he was found to have macrocephalus and micrognathia, which could render intubation difficult. He was also afflicted with general muscular dystrophy. During surgery, his vitals were monitored by electrocardiogram, pulse oximetry, noninvasive blood pressure (BP) monitoring, endtidal CO2, bispectral index (Aspect Medical Systems, Newton,