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Neuroradiology Case of the Week
Case 317
Adam J. Weller, MSIV and Virendra Kumar, MD
Clinical
Presentation: Patient is a 16-year-old male with Sturge-Weber syndrome and epilepsy.
Imaging Findings: A dilated, serpiginous conglomeration of abnormal vessels can be seen in the posterior portion of the left occipital lobe as well as adjacent to the posterior horn of the left lateral ventricle. These findings enhance with contrast on T1-weighted images and demonstrates low signal on T2-weighted images. Gyral calcifications can also be visualized in the same region as low signal intensity on gradient echo imaging. Enlargement of the left occipital horn choroid plexus can be visualized. Also, an area of ocular enhancement in the left lateral and posterior globe, concerning for ocular hemangioma is seen.
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Figure 1: Axial T1 with contrast. Enhancing conglomeration of vessels can be visualized in the posterior left occipital lobe.
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Figure 2. Axial T1 with contrast. Visualization of the enlarged left occipital horn choroid plexus. |
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Figure 3: Axial Gradient Echo image. Gyral calcifications demonstrating low signal intensity on gradient echo imaging.
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Figure 4. Axial T1 with contrast. Enhancing hemangioma can be visualized in lateral aspect of the left orbit. Ipsilateral Diffuse cortical atrophy can also be visualized. |
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Figure 5. Axial T2. Hemangioma of left orbit. |
Diagnosis: Sturge-Weber syndrome with intraoccular hemangioma
Discussion: Sturge-Weber syndrome is a congenital disorder involving angiomas of the leptomeninges and skin of the face. The symptoms are caused by residual embryonal blood vessels and their secondary effects on the surrounding tissue and brain. The typical presentation of the cutaneous angioma is a port wine stain following the ophthalmic and maxillary distributions of the trigeminal nerve. Intercranial the angiomas are usually unilateral and neurological side effects depending on location include seizures (80%), stroke like episodes, headaches, and developmental disorders.
Complications of the eye are usually ipsilateral and consist of such entities as buphthalmos and glaucoma, which much be treated to avoid eventual rise in intraocular pressure and progressive vision loss and blindness. Conjunctival, episcleral, and choroidal hemangiomas are also common findings and are thought to contribute to the high intraocular pressure by hyper secretions of fluid.
This case is an excellent example as most of the common imaging findings of Sturge-Weber syndrome are visualized. The primary pathologic abnormality is the meningeal angioma which consists of multiple small venous channels matted together near the surface of the brain (Fig. 1). Another common finding seen here is enlarged enhancing choroid plexus (Fig. 2). Cortical calcifications are the most frequent CT finding of Sturge-Weber syndrome but also seen on T2-weighted scans and gradient echo imaging as in this case (Fig. 3). The calcifications typically occur exclusively in areas of the brain subjacent to the angioma. The ipsilateral cerebral hemisphere ultimately becomes atrophic in most patients and will be bilateral if patient has bilateral angiomas (Fig. 4). Cerebral hemiatrophy usually results in cranial asymmetry with ipsilateral cranial thickening and enlarged paranasal sinuses and mastoid airspaces. Abnormalities of the globe are seen in 30% of patients and usually are angiomata of the choroid and sclera (Figs. 4 and 5).
References:
- Barkovich J. Pediatric Neuroimaging, 3rd Edition, Philadelphia, Lippincott Williams & Wilkins 2000, pg 415-421.
- Khan AN, Turnbull IA, Macdonald S, Raja RC. Sturge-Weber syndrome. Emedicine, February 20, 2007. www.emedicine.com/radio/topic660.htm
- Takeoka M, Riviello JJ Jr. Sturge-Weber syndrome. Emedicine, June 7, 2006.www.emedicine.com/neuro/topic356.htm
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