Sturge-Weber sendromu; klinik ve radyolojik değerlendirme

Mehmet Canpolat; Hüseyin Per; Ali Yıkılmaz; Hakan Gümüş; Alper Özcan; Hatice Gamze Poyrazoğlu; Abdulhakim Çoşkun; Sefer Kumandaş

doi:10.5455/GMJ-30-2012-120

Sturge-Weber syndrome: clinical and radiological evaluation

Year 2013, Volume: 19 Issue: 1, 30 - 34, 01.02.2013

Mehmet Canpolat Hüseyin Per Ali Yıkılmaz Hakan Gümüş Alper Özcan Hatice Gamze Poyrazoğlu Abdulhakim Çoşkun Sefer Kumandaş

https://doi.org/10.5455/GMJ-30-2012-120

Abstract

In this study,we aimed to evaluate the clinical and neuroimaging features in children with Sturge-Weber syndrome (SWS). Eleven patients with SWS were included in this study. Chart analysis, clinical evaluation, neurological and ophthalmological examinations, electroencephalographic and neuroimaging studies were evaluated retrospectively. The study approved by the Erciyes University Faculty of Medicine Ethics committee (07.08.2012/486). The mean age was 61.82 ± 39.73 months (range from 16 to 132 months ). The most common symptoms were convulsion and facial angioma. Port-wine stains was observed in all cases. Epilepsy, hemiparesis, psychomotor retardation and glaucoma were the most common issues. Cortical calcifications on cranial tomography (CT) scan was present in 6 cases. On magnetic resonance imaging (MRI) of cranial, there were cerebral atropy in 9 cases, leptomeningeal angioma in 7 cases, diploic proimence in 5 cases, enlargement of the choroid plexus in 5 cases, choroid plexus cyst in 3 cases and venous anomalies in 3 cases. Port-wine stains, epilepsy, hemiparesis, psychomotor retardation, glaucoma, cortical calcifications on CT, cerebral atrophy and leptomeningeal angiomatosis on MRI were the most frequent features of patients with Sturge-Weber syndrome in this series.

Keywords

Child; magnetic resonance imaging; port-wine stains; Sturge-Weber syndrome.

References

1. Di Rocco C, Tamburrini G. Sturge-Weber syndrome. Childs Nerv Syst 2006;22(8):909-21
2. Zhou J, Li NY, Zhou XJ, Wang JD, Ma HH, Zhang RS. Sturge- Weber syndrome: a case report and review of literatures. Chin Med J (Engl) 2010;123:117-21
3. Mıhçı E. Hamartomatous Syndromes. Türkiye Klinikleri Journal of Pediatric Sciences 2011;7:73-82
4. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg 2004;23:87-98
5. Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol 2004;30:303-10
6. Comi AM. Pathophysiology of Sturge-Weber syndrome. J Child Neurol 2003;18:509-16
7. Comi AM. Update on Sturge-Weber syndrome: diagnosis, treatment, quantitative measures, and controversies. Lymphat Res Biol 2007;5:257-64
8. Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, Viaño J. Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci 2008;35:301-7
9. Sharan S, Swamy B, Taranath DA, Jamieson R, Yu T, Wargon O, Grigg JR. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS 2009;13:374-8
10. Sujansky E, Conradi S. Sturge-Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol 1995;10:49-58
11. Sujansky E, Conradi S. Outcome of Sturge-Weber syndrome in 52 adults. Am J Med Genet 1995;57:35-45
12. Puttgen KB, Lin DD. Neurocutaneous vascular syndromes
Childs Nerv Syst 2010;26:1407-15
13. Comi AM. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. Neurologist 2011;17:179-84
14. Comi AM. Sturge-Weber syndrome and epilepsy: an argument for aggressive seizure management in these patients. Expert Rev Neurother 2007;7:951-6
15. Daniel RT, Thomas SG, Thomas M. Role of surgery in pediatric epilepsy. Indian Pediatr 2007;44:263-73
16. Slasky SE, Shinnar S, Bello JA. Sturge-Weber syndrome: deep venous occlusion and the radiologic spectrum. Pediatr Neurol 2006;35:343-7
17. Welch K, Naheedy MH, Abroms IF, Strand RD. Computed tomography of Sturge-Weber syndrome in infants. J Comput Assist Tomogr 1980;4:33-6
18. Martí-Bonmatí L, Menor F, Mulas F. The Sturge-Weber syndrome: correlation between the clinical status and radiological CT and MRI findings. Childs Nerv Syst 1993;9:107-9
19. Terdjman P, Aicardi J, Sainte-Rose C, Brunelle F
Neuroradiological findings in Sturge-Weber syndrome (SWS) and isolated pial angiomatosis. Neuropediatrics 1991;22:115-20
20. Benedikt RA, Brown DC, Walker R, Ghaed VN, Mitchell M, Geyer CA. Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol 1993;14:409-15
21. Griffiths PD, Blaser S, Boodram MB, Armstrong D, Harwood- Nash D. Choroid plexus size in young children with Sturge- Weber syndrome. AJNR Am J Neuroradiol 1996;17:175-80
22. Curé JK, Holden KR, Van Tassel P. Progressive venous occlusion in a neonate with Sturge-Weber syndrome: demonstration with MR venography. AJNR Am J Neuroradiol 1995;16:1539-42
23. Bentson JR, Wilson GH, Newton TH. Cerebral venous drainage pattern of the Sturge-Weber syndrome. Radiology 1971;101:111-8
24. 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;11:131-4
25. Stimac GK, Solomon MA, Newton TH. CT and MR of angiomatous malformations of the choroid plexus in patients with Sturge-Weber disease. AJNR Am J Neuroradiol 1986;7:623-7
26. Sperner J, Schmauser I, Bittner R, Henkes H, Bassir C, Sprung C, Scheffner D, Felix R. MR-imaging findings in children with Sturge-Weber syndrome. Neuropediatrics 1990;21:146-52
27. Griffiths PD, Coley SC, Romanowski CA, Hodgson T, Wilkinson ID. Contrast-enhanced fluid-attenuated inversion recovery imaging for leptomeningeal disease in children. AJNR Am J Neuroradiol 2003;24:719-23

Sturge-Weber sendromu; klinik ve radyolojik değerlendirme

Year 2013, Volume: 19 Issue: 1, 30 - 34, 01.02.2013

Mehmet Canpolat Hüseyin Per Ali Yıkılmaz Hakan Gümüş Alper Özcan Hatice Gamze Poyrazoğlu Abdulhakim Çoşkun Sefer Kumandaş

https://doi.org/10.5455/GMJ-30-2012-120

Abstract

Bu çalışmada Sturge-Weber sendromu (SWS) tanısı ile izlenen olguların klinik ve radyolojik olarak değerlendirilmesi amaçlandı. SWS tanısı konulan 11 olgunun klinik özellikleri, nörolojik ve göz muayene bulguları, elektroensefalografi ve kranial görüntüleme bulguları hasta takip dosyalarından geriye dönük olarak incelendi. Çalışma için Erciyes Üniversitesi Tıp Fakültesi etik kurul onayı (07.08.2012/486) alındı. Çalışmaya ortalama yaşları 61.82 ± 39.73 arasında değişen (16-132 ay) 11 olgu alındı. Olguların başvuruda en sık gözlenen yakınmaları, konvülsiyon ve yüzde leke idi. Porto şarabı lekesi tüm olgularda gözlendi. Epilepsi, psikomotor gerilik, hemiparezi ve glokom en sık gözlenen bulgulardı. Beyin Tomografisinde (BT) 6 olguda kalsifikasyon saptandı. Kranial manyetik rezonans görüntülemede (MRG), 9 olguda serebral atrofi, 7 olguda leptomeningeal anjiyoma, 5'er olguda kalvaryal kalınlaşma ve genişlemiş koroid pleksus, 3'er olguda da koroid pleksus kisti ve venöz anormaliler saptandı. Porto şarabı lekesi, epilepsi, hemiparezi, psikomotor gerilik, glokom, kortikal kalsifikasyon, serebral atrofi ve leptomeningeal anjiyoma en sık saptanan bulgulardı.

Keywords

Çocuk; manyetik rezonans görüntüleme; porto şarabı lekesi; Sturge-Weber sendromu.

References

1. Di Rocco C, Tamburrini G. Sturge-Weber syndrome. Childs Nerv Syst 2006;22(8):909-21
2. Zhou J, Li NY, Zhou XJ, Wang JD, Ma HH, Zhang RS. Sturge- Weber syndrome: a case report and review of literatures. Chin Med J (Engl) 2010;123:117-21
3. Mıhçı E. Hamartomatous Syndromes. Türkiye Klinikleri Journal of Pediatric Sciences 2011;7:73-82
4. Baselga E. Sturge-Weber syndrome. Semin Cutan Med Surg 2004;23:87-98
5. Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol 2004;30:303-10
6. Comi AM. Pathophysiology of Sturge-Weber syndrome. J Child Neurol 2003;18:509-16
7. Comi AM. Update on Sturge-Weber syndrome: diagnosis, treatment, quantitative measures, and controversies. Lymphat Res Biol 2007;5:257-64
8. Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R, Viaño J. Sturge-Weber syndrome: study of 55 patients. Can J Neurol Sci 2008;35:301-7
9. Sharan S, Swamy B, Taranath DA, Jamieson R, Yu T, Wargon O, Grigg JR. Port-wine vascular malformations and glaucoma risk in Sturge-Weber syndrome. J AAPOS 2009;13:374-8
10. Sujansky E, Conradi S. Sturge-Weber syndrome: age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol 1995;10:49-58
11. Sujansky E, Conradi S. Outcome of Sturge-Weber syndrome in 52 adults. Am J Med Genet 1995;57:35-45
12. Puttgen KB, Lin DD. Neurocutaneous vascular syndromes
Childs Nerv Syst 2010;26:1407-15
13. Comi AM. Presentation, diagnosis, pathophysiology, and treatment of the neurological features of Sturge-Weber syndrome. Neurologist 2011;17:179-84
14. Comi AM. Sturge-Weber syndrome and epilepsy: an argument for aggressive seizure management in these patients. Expert Rev Neurother 2007;7:951-6
15. Daniel RT, Thomas SG, Thomas M. Role of surgery in pediatric epilepsy. Indian Pediatr 2007;44:263-73
16. Slasky SE, Shinnar S, Bello JA. Sturge-Weber syndrome: deep venous occlusion and the radiologic spectrum. Pediatr Neurol 2006;35:343-7
17. Welch K, Naheedy MH, Abroms IF, Strand RD. Computed tomography of Sturge-Weber syndrome in infants. J Comput Assist Tomogr 1980;4:33-6
18. Martí-Bonmatí L, Menor F, Mulas F. The Sturge-Weber syndrome: correlation between the clinical status and radiological CT and MRI findings. Childs Nerv Syst 1993;9:107-9
19. Terdjman P, Aicardi J, Sainte-Rose C, Brunelle F
Neuroradiological findings in Sturge-Weber syndrome (SWS) and isolated pial angiomatosis. Neuropediatrics 1991;22:115-20
20. Benedikt RA, Brown DC, Walker R, Ghaed VN, Mitchell M, Geyer CA. Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR Am J Neuroradiol 1993;14:409-15
21. Griffiths PD, Blaser S, Boodram MB, Armstrong D, Harwood- Nash D. Choroid plexus size in young children with Sturge- Weber syndrome. AJNR Am J Neuroradiol 1996;17:175-80
22. Curé JK, Holden KR, Van Tassel P. Progressive venous occlusion in a neonate with Sturge-Weber syndrome: demonstration with MR venography. AJNR Am J Neuroradiol 1995;16:1539-42
23. Bentson JR, Wilson GH, Newton TH. Cerebral venous drainage pattern of the Sturge-Weber syndrome. Radiology 1971;101:111-8
24. 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;11:131-4
25. Stimac GK, Solomon MA, Newton TH. CT and MR of angiomatous malformations of the choroid plexus in patients with Sturge-Weber disease. AJNR Am J Neuroradiol 1986;7:623-7
26. Sperner J, Schmauser I, Bittner R, Henkes H, Bassir C, Sprung C, Scheffner D, Felix R. MR-imaging findings in children with Sturge-Weber syndrome. Neuropediatrics 1990;21:146-52
27. Griffiths PD, Coley SC, Romanowski CA, Hodgson T, Wilkinson ID. Contrast-enhanced fluid-attenuated inversion recovery imaging for leptomeningeal disease in children. AJNR Am J Neuroradiol 2003;24:719-23

There are 29 citations in total.

Details

Primary Language	Turkish
Journal Section	Articles
Authors	Mehmet Canpolat This is me Hüseyin Per This is me Ali Yıkılmaz This is me Hakan Gümüş This is me Alper Özcan This is me Hatice Gamze Poyrazoğlu This is me Abdulhakim Çoşkun This is me Sefer Kumandaş This is me
Publication Date	February 1, 2013
Published in Issue	Year 2013 Volume: 19 Issue: 1

Cite

APA	Canpolat, M., Per, H., Yıkılmaz, A., Gümüş, H., et al. (2013). Sturge-Weber sendromu; klinik ve radyolojik değerlendirme. Gaziantep Medical Journal, 19(1), 30-34. https://doi.org/10.5455/GMJ-30-2012-120
AMA	Canpolat M, Per H, Yıkılmaz A, Gümüş H, Özcan A, Poyrazoğlu HG, Çoşkun A, Kumandaş S. Sturge-Weber sendromu; klinik ve radyolojik değerlendirme. Gaziantep Medical Journal. February 2013;19(1):30-34. doi:10.5455/GMJ-30-2012-120
Chicago	Canpolat, Mehmet, Hüseyin Per, Ali Yıkılmaz, Hakan Gümüş, Alper Özcan, Hatice Gamze Poyrazoğlu, Abdulhakim Çoşkun, and Sefer Kumandaş. “Sturge-Weber Sendromu; Klinik Ve Radyolojik değerlendirme”. Gaziantep Medical Journal 19, no. 1 (February 2013): 30-34. https://doi.org/10.5455/GMJ-30-2012-120.
EndNote	Canpolat M, Per H, Yıkılmaz A, Gümüş H, Özcan A, Poyrazoğlu HG, Çoşkun A, Kumandaş S (February 1, 2013) Sturge-Weber sendromu; klinik ve radyolojik değerlendirme. Gaziantep Medical Journal 19 1 30–34.
IEEE	M. Canpolat, H. Per, A. Yıkılmaz, H. Gümüş, A. Özcan, H. G. Poyrazoğlu, A. Çoşkun, and S. Kumandaş, “Sturge-Weber sendromu; klinik ve radyolojik değerlendirme”, Gaziantep Medical Journal, vol. 19, no. 1, pp. 30–34, 2013, doi: 10.5455/GMJ-30-2012-120.
ISNAD	Canpolat, Mehmet et al. “Sturge-Weber Sendromu; Klinik Ve Radyolojik değerlendirme”. Gaziantep Medical Journal 19/1 (February 2013), 30-34. https://doi.org/10.5455/GMJ-30-2012-120.
JAMA	Canpolat M, Per H, Yıkılmaz A, Gümüş H, Özcan A, Poyrazoğlu HG, Çoşkun A, Kumandaş S. Sturge-Weber sendromu; klinik ve radyolojik değerlendirme. Gaziantep Medical Journal. 2013;19:30–34.
MLA	Canpolat, Mehmet et al. “Sturge-Weber Sendromu; Klinik Ve Radyolojik değerlendirme”. Gaziantep Medical Journal, vol. 19, no. 1, 2013, pp. 30-34, doi:10.5455/GMJ-30-2012-120.
Vancouver	Canpolat M, Per H, Yıkılmaz A, Gümüş H, Özcan A, Poyrazoğlu HG, Çoşkun A, Kumandaş S. Sturge-Weber sendromu; klinik ve radyolojik değerlendirme. Gaziantep Medical Journal. 2013;19(1):30-4.

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