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Case 7: Hydranencephaly

¹ Department of Radiology, Thomas Jefferson University Hospital, Gibbon Bldg 3350AB, 111 S 11th St, Philadelphia, PA 19107.

View larger version (127K): [in a new window]   Figure 1a. US images of the fetal head at 36 weeks gestation. (a) Transaxial image near the vertex demonstrates a discontinuous falx midline echo (curved arrow). There is no identifiable cortical mantel. (b) Transaxial image at the level of the normal thalami (T) again shows the disrupted falx midline echo (curved arrow). Normal hyperechoic choroid plexuses (straight arrows) are seen posterior to the thalami, and a small amount of occipital cortex remains, posterior to both. There again is no demonstrable cortical mantle (the echoes seen are artifactual). (c) Transaxial image slanted posteriorly to depict the posterior fossa demonstrates the midbrain (M) and the disrupted falx echo (curved arrow). The triangular posterior fossa with an intact cerebellum (straight arrows) and a normal cisterna magna (*) are seen.

View larger version (125K): [in a new window]   Figure 1b. US images of the fetal head at 36 weeks gestation. (a) Transaxial image near the vertex demonstrates a discontinuous falx midline echo (curved arrow). There is no identifiable cortical mantel. (b) Transaxial image at the level of the normal thalami (T) again shows the disrupted falx midline echo (curved arrow). Normal hyperechoic choroid plexuses (straight arrows) are seen posterior to the thalami, and a small amount of occipital cortex remains, posterior to both. There again is no demonstrable cortical mantle (the echoes seen are artifactual). (c) Transaxial image slanted posteriorly to depict the posterior fossa demonstrates the midbrain (M) and the disrupted falx echo (curved arrow). The triangular posterior fossa with an intact cerebellum (straight arrows) and a normal cisterna magna (*) are seen.

View larger version (148K): [in a new window]   Figure 1c. US images of the fetal head at 36 weeks gestation. (a) Transaxial image near the vertex demonstrates a discontinuous falx midline echo (curved arrow). There is no identifiable cortical mantel. (b) Transaxial image at the level of the normal thalami (T) again shows the disrupted falx midline echo (curved arrow). Normal hyperechoic choroid plexuses (straight arrows) are seen posterior to the thalami, and a small amount of occipital cortex remains, posterior to both. There again is no demonstrable cortical mantle (the echoes seen are artifactual). (c) Transaxial image slanted posteriorly to depict the posterior fossa demonstrates the midbrain (M) and the disrupted falx echo (curved arrow). The triangular posterior fossa with an intact cerebellum (straight arrows) and a normal cisterna magna (*) are seen.

View larger version (148K): [in a new window]   Figure 2a. CT scan of the newborn's head, without use of intravenous contrast material. (a) Transaxial view near the vertex shows a disrupted falx (curved arrow). No normal cortical mantle remains. (b) Transaxial view at the level of the normal thalami (T) shows normal choroid plexuses (solid arrows) posteriorly. Some occipital cortex (open arrows) remains. (c) Transaxial view through the base shows a normal posterior fossa, including cerebellum.

View larger version (130K): [in a new window]   Figure 2b. CT scan of the newborn's head, without use of intravenous contrast material. (a) Transaxial view near the vertex shows a disrupted falx (curved arrow). No normal cortical mantle remains. (b) Transaxial view at the level of the normal thalami (T) shows normal choroid plexuses (solid arrows) posteriorly. Some occipital cortex (open arrows) remains. (c) Transaxial view through the base shows a normal posterior fossa, including cerebellum.

View larger version (133K): [in a new window]   Figure 2c. CT scan of the newborn's head, without use of intravenous contrast material. (a) Transaxial view near the vertex shows a disrupted falx (curved arrow). No normal cortical mantle remains. (b) Transaxial view at the level of the normal thalami (T) shows normal choroid plexuses (solid arrows) posteriorly. Some occipital cortex (open arrows) remains. (c) Transaxial view through the base shows a normal posterior fossa, including cerebellum.

View larger version (141K): [in a new window]   Figure 3. Moderate to severe hydrocephalus secondary to aqueductal stenosis. A transaxial US scan of a fetus at 30 weeks gestation demonstrates an enlarged fetal head with thinned but present temporoparietal cortical mantle (arrows) along the posterolateral aspect of the calvaria. The third ventricle (*) is also dilated between the thalami. Although the temporoparietal cortical mantle is also present anteriorly, it cannot be appreciated because of reverberation artifacts.

View larger version (190K): [in a new window]   Figure 4. Alobar holoprosencephaly in a fetus at 22 weeks gestation. Coronal US image of a small fetal head shows fused thalami (T). A monoventricle (V) is identified without a normal falx echo. L = left, R = right.