Fig 6.

Single-shot fast spin-echo sagittal midline (A) and axial posterior fossa (B) images in a 34-week fetus with multiple findings of dystroglycanopathy suggesting Walker-Warburg syndrome. Postnatal correlation includes a sagittal T1-weighted sequence (C) and an axial T2-weighted sequence (D). Sagittal views of both pre- and postnatal MR imaging demonstrate a hypoplastic kinked brain stem (white arrowheads) and a markedly hypoplastic cerebellar vermis. Note also the dysplastic midbrain with thickening of the tectum causing stenosis of the cerebral aqueduct (arrows). Lateral and third ventricles are markedly enlarged. Incidentally noted was a small occipital cephalocele (asterisk). Axial views show cerebellar dysplasia with irregular cerebellar margins (black arrowheads) and multiple small cerebellar cysts, which account for the increased white matter T2 signal on fetal MR imaging (asterisk). Note also a midline pontine cleft (white arrowheads in B and D), another common finding in Walker-Warburg syndrome.