475a large head, and multiple pigmented, hairy nevi were observed over her whole body (Fig. 1). Computerized tomography (CT) showed vermian hypoplasia and bi-compartmental hydrocephalus. The fourth ventricle was enlarged and seemed to be continuous with the cisterna magna, suggesting a Dandy-Walker malformation ( Fig. 2A). Magnetic resonance imaging (MRI) was also compatible with Dandy-Walker malformation and high-pressure hydrocephalus. The images demonstrated a large posterior fossa and communication with the enlarged fourth ventricle through the remaining cerebrospinal fluid (CSF) pathway, and aplasia of the cerebellar vermis, hypoplasia of the cerebellar hemisphere, and a high position of the tentorium (Fig. 2B). The patient developed generalized seizures, and a repeated radiological study suggested a possible tonsillar herniation. She underwent a ventriculoperitoneal shunting procedure (program- INTRODUCTIONThe association of congenital abnormalities of the skin and the central nervous system is well recognized. Neurocutaneous melanosis is a rare dysmorphogenesis that is associated with single and multiple giant pigmented cutaneous nevi and the involvement of benign and/or malignant melanocytic tumors of the leptomeninges. After the first description by Rokitansky 17) , around 100 cases of neurocutaneous melanosis have been reported in the literature [1][2][3]5,10,11,[13][14][15][16]18,19) . Eight to 10% of these were associated with Dandy-Walker malformation, indicating a common origin of these developmental abnormalities [1][2][3]7,10,11,13,14,18,19) . We report the clinical features, neuroimaging findings, and therapeutic management of a rare case of neurocutaneous melanosis associated with Dandy-Walker malformation. CASE REPORTA two-month-old female patient was first admitted to our hospital with complaints of vomiting and a gradually enlarging head circumference. She did not have an eventful history or pregnancy except for multiple melanocytic nevi over her whole body. She had attained normal neurodevelopmental milestones until 2 months of age.In her initial physical examination, she was lethargic and had Neurocutaneous melanosis associated with Dandy-Walker malformation is a rare dysmorphogenesis that is associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the central nervous system. In this article, we present a 2-month-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation. In addition, we reviewed the literature and discussed the pathogenesis based on the preferred hypotheses.
We report an unusual case of lateral medullary infarction after successful embolization of the vertebral artery dissecting aneurysm (VADA). A 49-year-old man who had no noteworthy previous medical history was admitted to our hospital with a severe headache. Computed tomography (CT) revealed a subarachnoid hemorrhage, located in the basal cistern and posterior fossa. Cerebral angiography showed a VADA, that did not involve the origin of the posterior inferior cerebellar artery (PICA). We treated this aneurysm via endovascular trapping of the vertebral artery distal to the PICA. After operation, CT revealed post-hemorrhagic hydrocephalus, which we resolved with a permanent ventriculoperitoneal shunt procedure. Postoperatively, the patient experienced transient mild hoarsness and dysphagia. Magnetic resonance image (MRI) showed a small infarction in the right side of the medulla. The patient recovered well, though he still had some residual symptom of dysphagia at discharge. Such an event is uncommon but can be a major clinical concern. Further investigation to reveal risk factors and/or causative mechanisms for the medullary infarction after successful endovascular trapping of the VADA are sorely needed, to minimize such a complication.
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