PMC5223002_01
PMC5223002_02
(a) Axial FLAIR: agyria enlarged ventricle, bilateral periventricle cystic hypodenisty, and delayed myelination of cerebral cortex. (b) Sagittal T2: enlarged lateral and third ventricle. Hypoplastic cerebellum, large posterior fossa CSF fluid space. There is no visible aqueduct (arrow), kinked pontomesencephalic kink. (c) Axial T2-weighted image reveals typical changes related to cobblestone lissencephaly. White matter is diffusely hyperintensity. Enlarged ventricle.
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fig1
However, it demonstrated significant findings of delayed myelination of cerebral cortex, hypoplastic cerebellum, and cobblestone lissencephaly (Figures 1(a)-1(c)), all of which are well-characterized in WWS.
(a) Ventriculostomy was fenestrated at infundibulum recess. Blue arrow: ventriculostomy, green arrow: clivus, and red arrows: bilateral mammillary body. (b) Endoscopic view through ventriculostomy in the interpeduncular cistern. Red arrow: basilar artery and yellow arrows: bilateral oculomotor nerves.  *Foramen of Monro.  **Endoscope.
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Aesculap PaediScope  Endoscope was inserted in right lateral ventricle. Anatomical distortion was seen during the endoscopic examination of the ventricle (Figure 2(a)). Success of ETV was confirmed as the endoscope was advanced into the prepontine cistern to observe the basilar artery and oculomotor cranial nerves (Figure 2(b)). Since the third ventriculostomy site demonstrated vigorous pulsation (Figures 2(c) and 2(d)), additional risk was not warranted.
PMC5223002_03
(a, b, c) MRI CSF flow study: arrow shows CSF flow at the floor of third ventricle.
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At 15 months after surgery, a follow-up brain MRI with flow study showed robust CSF pulsation at floor of third ventricle (Figures 3(a), 3(b), and 3(c)).
Patient occipital-frontal circumference chart. Blue arrow showed time of ETV with CPC.
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The patient's head circumference followed a stable growth curve (Figure 4).