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Year : 2003  |  Volume : 13  |  Issue : 2  |  Page : 233-235
Choroidal hemangioma with a bleed in Sturge-Weber syndrome : MRI


Department of Radiodiagnosis and Imaging, Christian Medical College and Hospital, Vellore-632004, India

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How to cite this article:
Singh S, Thomas S. Choroidal hemangioma with a bleed in Sturge-Weber syndrome : MRI. Indian J Radiol Imaging 2003;13:233-5

How to cite this URL:
Singh S, Thomas S. Choroidal hemangioma with a bleed in Sturge-Weber syndrome : MRI. Indian J Radiol Imaging [serial online] 2003 [cited 2019 Mar 21];13:233-5. Available from: http://www.ijri.org/text.asp?2003/13/2/233/28668
Sir,

In  Sturge- Weber syndrome More Details More Details, common ocular findings include: buphthalmos, choroidal angiomas, and episcleral telangiectasias [1]. Buphthalamos (occurs in 15% of cases) is caused by congenital glaucoma due to increased pressure, which may be related to impaired reabsorption of ocular fluid [1]. In almost one third of the cases either the sclera or the ocular choroid may show a dilated plexus of vessels [1]. These abnormal vessels are immature and have histologic features resembling both dilated capillaries as well as small viens and are often described as telangiectasias [1]. Slit-lamp examination help identify this vascular abnormality. We described the MRI findings in a case of choraidal haemangioma.

An 11-year-old girl presented with two episodes of generalized tonic clonic seizures of 1-year duration, and gradual loss of right eye vision of 2-years' duration. Her physical examination revealed an ipsilateral facial naevus and red eye with no light perception. Slit-lamp examination revealed a shallow anterior chamber and prominent episcleral vessels and a choroidal haemangioma with exudative retinal detachment. The intraocular pressure was 40 mm Hg. Electroencephalography showed interictal record of generalized epilepsy with left parietal lobe predominance. On MRI, the long TR-images showed a sliver of mildly hyperintense lesion (of 5 mm thickness) in the region of the choroid [Figure - 1]. On short TR-image, the lesion was isointense [Figure - 2]A, which showed intense enhancement [Figure - 2]B. Evidence of a subretinal and vitreous bleed (hyperintense on all pulse sequences) was noted. The ipsilateral optic nerve and extraconal compartment and left eye globe were normal. The MRI features were consistent with choroidal haemangioma with retinal detachment and subretinal and vitreous hemorrhage. The cerebral CT scan showed gyriform calcification in the parietooccipital lobes.

The Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a neurocutaneous syndrome manifesting as facial and leptomeningeal angiomas, and ocular involvement manifesting as glaucoma and vascular malformation of the conjunctiva, episclera, choroid and retina [2],[3]. The clinical diagnostic criteria include ipsilateral neveus flammeus, seizures, hemiparesis, mental retardation, buphthalamos, ipsilateral bone and sinus hypertrophy, facial hemihypertrophy, and somatic hemiatrophy [3]. The radiological diagnosis is based on intracranial calcification, ipsilateral leptomeningeal capillary or venous malformation, cerebral atrophy (predominantly parietooccipital lobes), cerebral hemiatrophy, and gyral enhancement and enlarged choroid plexus, cranial diploe prominence and venous abnormalities [2],[3]. The ocular manifestations include retinal vascular tortuosity, iris heterochromia, choroid and retinal angioma, retinal detachment and strabismus [5].

CT can demonstrate cerebral atrophy, ipsilateral bone and sinus hypertrophy, calcification, gyral enhancement, prominent deep venous system and enlarged choroid plexuses [3]. MRI is better than CT in showing the extent and degree of brain parenchymal atrophy, vascular anomalies and presumed ischaemic changes affecting the gray and white matter [2]. MRI after contrast administration could also show leptomeningeal angiomatous malformation and the parenchymal venous anomalies better than CT scan [2]. Unenhanced CT should be used, if MRI is normal, to exclude the presence of intracranial calcifications, as in our case [2]. Conventional spin-echo MRI revealed fewer calcifications, and those visualized appeared smaller than with CT. Gradient-echo acquisition sequences were more effective than conventional spin-echo in the detection of intracranial calcification [3].

Our case shows choroidal haemangioma, with a bleed. The choroid haemangioma was mildly hyperintense (to adjacent muscles) on long TR-images and isointense on short TR. Post contrast images show intense enhancement. The vitreous and subretinal hemorrhage was seen as hyperintense signal on all pulse sequences.

In conclusion, this case demonstrates magnetic resonance imaging feature of choroidal hemangioma with a subretinal and vitreous bleed.

 
   References Top

1.Atlas WS. Magnetic Resonance Imaging of the Brain and Spine. In: Smirniotopoulos GJ, Murphy MF. Central Nervous System manifestation of the Phakomatoses and Other Inherited Syndromes; Chapter11, pp 371-413; 3rd ed. 2002, Lippincott Williams& Wilkins, Philadelphia, USA.  Back to cited text no. 1    
2.Marti-Bonmati L, Menor F, Poyatos C, Cortina H. Diagnosis of Sturge-Weber syndrome: comparison of the efficacy of CT and MR imaging in 14 cases. AJR Am J Roentgenol 1992; 158 (4): 867-71.  Back to cited text no. 2    
3.Wasenko JJ, Rosenbloom SA, Duchesneau PM, Lanzieri CF, Weinstein MA. The Sturge-Weber syndrome: comparison of MR and CT characteristics. AJNR Am J Neuroradiol 1990; 11(1): 131-4.   Back to cited text no. 3    
4.Griffiths PD, Boodram MB, Blaser S, Altomare F, Buncic JR, Levin AV, Jay V, Armstrong D, Harwood-Nash D. Abnormal ocular enhancement in Sturge-Weber syndrome: correlation of ocular MR and CT findings with clinical and intracranial imaging findings. AJNR Am J Neuroradiol. 1997; 18 (5): 993.  Back to cited text no. 4    
5.Sullivan TJ, Clarke MP, Morin JD. The ocular manifestations of the Sturge-Weber syndrome. J Pediatr Ophthalmol Strabismus 1992; 29 (6): 349-56.  Back to cited text no. 5    

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Correspondence Address:
S Singh
Department of Radiodiagnosis and Imaging, Christian Medical College and Hospital, Vellore-632004
India
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Source of Support: None, Conflict of Interest: None


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