Cranial MR Imaging of Osteopetrosis
Joel K. Curé
,a,
Lyndon L. Keya,
David D. Goltraa and
Pamela VanTassela
a From the Departments of Diagnostic Radiology (J.K.C., D.D.G., P.V.T.) and Pediatrics (L.L.K.), Medical University of South Carolina, Charleston.
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FIG 1. Calvarial thickening.
A, AROP. Sagittal T1-weighted MR image shows thickening of the calvaria and facial bones. Note hypointensity of skull and cervical vertebra, and cerebellar tonsillar ectopia.
B, ADOP I. Sagittal T1-weighted image shows calvarial thickening and hypointensity, as well as tonsillar ectopia.
C, ADOP II. Sagittal T1-weighted image shows only a moderately thickened calvaria; however, the skull base is more significantly thickened than in the example of ADOP I (B). Compare the small sellar volume with that in ADOP I (B).
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FIG 2. Optic nerve/sheath manifestations.
A, Prominent optic nerve sheath dilatation, ADOP I. Axial T2-weighted FSE image shows scalloped inner table. The optic canals are not stenotic.
B, AROP. Axial T2-weighted FSE image shows optic canal stenosis and optic nerve atrophy.
C, IOP. Axial T2-weighted FSE image shows optic canal stenosis and optic nerve atrophy, with more severe atrophy evident on the right in this case.
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FIG 3. Dural venous sinus stenosis at 2D-TOF MR venography in two patients with AROP.
A, In one patient, lateral projection shows occlusion or high-grade stenosis of both sigmoid sinuses/jugular bulbs. Note prominent scalp collateral venous flow and sinus pericranii (arrow).
B, In another patient (same child as in fig 4A), lateral projection shows bilateral sigmoid sinus stenosis or occlusion (arrowhead). Note superior displacement of superior sagittal sinus by protruding brain.
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FIG 4. Cephaloceles, nonsurgical.
A, AROP. Sagittal T1-weighted image shows brain protruding upward into the anterior fontanel as a result of limited intracranial volume. The superior sagittal sinus is superiorly displaced (See fig 3B). Note tonsillar ectopia.
B, AROP. Axial T2-weighted FSE image shows bilateral meningoceles at the craniocervical junction (arrows).
C, ADOP I. Sagittal T1-weighted image shows brain protruding downward through the foramen ovale (arrow). Note scalloping of the floor of the anterior cranial fossa.
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FIG 5. Acquired cephaloceles, surgical.
A, AROP. Axial T2-weighted FSE image. Bilateral decompressive craniectomies have resulted in a "Mickey Mouse"-like herniation of brain into and through the calvaria.
B, AROP. Axial T2-weighted FSE image. A posterior fossa decompressive craniectomy was complicated by a cephalocele. Cerebellar tissue, CSF, and meninges protrude through the surgical defect.
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FIG 6. AROP. 3D-TOF MR angiogram, anteroposterior projection, shows bilateral petrous ICA stenosis (arrows), with more diffuse stenosis also evident in the left ICA.
FIG 7. AROP. 2D-TOF MR angiogram, left lateral projection, shows segmental vertebral artery stenoses with collateral flow in the ascending cervical arteries, anastomosing with the vertebral arteries above the level of the most cranial transverse foramen.
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FIG 8. Extramedullary hematopoiesis, AROP.
A, Axial contrast-enhanced T1-weighted image shows homogeneously enhancing extraaxial tissue along the parietal convexities and falx cerebri.
B, Right lateral projection of 99mTc-sulfur colloid study shows uptake in these deposits of extramedullary hematopoiesis.
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AJNR - American Journal of Neuroradiology