OBJECT The goal of this study was to determine the value of the 3D sampling perfection with application-optimized contrasts using different flip-angle evolutions (3D-SPACE) technique in the evaluation of endoscopic third ventriculostomy (ETV) patency. METHODS Twenty-six patients with ETV were examined using 3-T MRI units. Sagittal-plane 3D-SPACE with variant flip-angle mode, 3D T1-weighted (T1W), and 3D heavily T2-weighted (T2W) images were obtained with isotropic voxel sizes. Also, sagittal-axial plane phase-contrast cine (PC)-MR images were obtained. The following findings were evaluated: diameters of stoma and third ventricle, flow-void sign on 3D-SPACE and PC-MR images, integrity of the third ventricle on heavily T2W images, and quantitative PC-MRI parameters of the stoma. Obtained sequences were evaluated singly, in combination with one another, and all together. RESULTS The mean area, flow, and velocity values measured at the level of stoma in patients with patent stoma were significantly higher than those measured in patients with closed stoma (p < 0.05). There was significant correlation among PC-MRI, 3D-SPACE, and 3D heavily T2W techniques regarding assessment of ETV patency (p < 0.001). The 3D-SPACE technique provided the lowest rate of ambiguous results. CONCLUSIONS The 3D-SPACE technique seems to be the most efficient one for determination of ETV patency. The authors suggest the use of 3D-SPACE as a stand-alone first-line sequence in addition to routine brain MRI protocols in assessing patients with ETV, thereby decreasing scan time and reserving the use of a combination of additional sequences such as PC-MRI and 3D heavily T2W images in suspicious or complex cases.

L-2-Hydroxyglutaric aciduria (LHGA) is a rare autosomal recessively inherited neurodegenerative disorder. It is characterized by psychomotor retardation, progressive ataxia and typical magnetic resonance imaging findings, and presents in early infancy. On the other hand, medulloblastomas are very common solid tumors of childhood and infancy. We present a 3-year-old boy with LHGA who developed a medulloblastoma during the course of the disease. There has been no previous report of the coexistence of medulloblastomas with LHGA. Central nervous system tumors are encountered in children with other metabolic neurodegenerative disorders. The aim of this paper is to focus on the difficulties in the diagnosis and treatment of an intracranial tumor in a child already neurologically impaired due to metabolic neurodegenerative disease.