Abstract
Introduction
Sturge–Weber syndrome (SWS) is a rare neurocutaneous syndrome the main clinical features of which are facial, mostly unilateral nevi, leptomeningeal angiomatosis, and congenital glaucoma. The interest of this syndrome for pediatric neurosurgeons is mainly related to the association of SWS with epilepsy in 75–90% of the cases. Seizures are resistant to medical treatment in almost 60% of these patients that consequently should be evaluated for epilepsy surgery.
Indications to surgical treatment
Children with SWS and drug-resistant epilepsy are optimal candidates for disconnective or resective surgical procedures in terms of both seizure control and intellectual outcomes. Controversies, however, still exist between the advantages of early “prophylactic” operation vs later surgical interventions. Though better results in terms of seizures control and psychomotor development were reported in a limited series of children operated on early in life, the insufficient number of subjects who underwent the surgical treatment does not allow definite conclusions yet.
Neurosurgical techniques
Visually guided lobectomy with complete excision of the angiomatous cortex should be considered as the primary surgical procedure in patients with focal lesions. Hemispherectomy is the treatment of choice in children with extensive hemispheric lesions.
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References
Alexander GL (1972) Sturge–Weber disease. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology. The phakomatoses, vol. 14. Elsevier, New York, pp 223–240
Alexander G, Norman RM (1960) The Sturge–Weber Syndrome. J Wright and Sons, Bristol
Arzimanoglou A, Aicardi J (1992) The epilepsy of Sturge–Weber syndrome: clinical features and treatment in 23 patients. Acta Neurol Scand 140:S18–S22
Arzimanoglou A, Andermann F, Aicardi J, Sainte-Rose C, Beaulieu MA, Villemure JG, Olivier A, Rasmussen Th (2000) Sturge–Weber syndrome. Indications and results of surgery in 20 patients. Neurology 55:1472–1479
Bergstrand H, Olivecrona H, Tonnis W (1936) Gefassmissbildungen and gefassgeschwulte des gehrins. Thieme, Leipzig, p 181
Chao DHC (1959) Congenital neurocutaneous syndromes of childhood. III. Sturge–Weber disease. J Pediatr 55:635–648
Chapieski L, Friedman A, Lachar D (2000) Psychological functioning in children and adolescents with Sturge–Weber syndrome. J Child Neurol 3:181
Chugani HT (1994) The role of PET in childhood epilepsy. J Child Neurol 9(1):S82–S88
Comi A (2003) Pathophysiology of Sturge–Weber syndrome. J Child Neurol 18:509–516
Comi A, Hunt P, Vawter MP, Pardo CA, Becker KG, Pevsner J (2003) Increased fibronectin expression in Sturge–Weber syndrome fibroblasts and brain tissue. Pediatr Res 53(5):762–769
Cunha e Sa M, Barroso CP, Caldas MC, Edvinsson L, Gulbenkian S (1997) Innervation pattern of malformative vessels in Sturge–Weber disease: an histochemical. Immunohistochemical and ultrastructural study. Neurosurgery 41(4):872–877
Cushing H (1906) Cases of spontaneous intracranial hemorrhage associated with trigeminal nevi. JAMA 47:178–183
Dimitri V (1923) Tumor cerebral congénito (angioma cavernosum) Rev Asoc Med Argent 36:1029–1037
Di Rocco C (1992) Sturge–Weber disease. In : Raimondi AJ, Choux M, Di Rocco C (eds) Cerebrovascular diseases in children, Springer, Berlin Heidelberg New York, pp 168–187
Di Trapani G, Di Rocco C, Abbamonti AL, Caldarelli M (1982) Light microscopy and ultrastructural studies of Sturge–Weber disease. Childs Brain 9:23–36
Duncan DB, Herholz K, Pietrzyk U, Heiss WD (1995) Regional cerebral blood flow and metabolism in Sturge–Weber disease. Clin Nucl Med 20(6):522–523
Elster AD, Chen MY (1990) MR imaging of Sturge–Weber syndrome: role of Gadopentate dimeglumine and gradient-echo techniques. AJNR Am J Neuroradiol 11:685–689
Falconer MA, Rushworth RG (1960) Treatment of encephalotrigeminal angiomatosis (Sturge–Weber disease) by hemispherectomy. Arch Dis Child 35:433–447
Faller L, Lemmer J (2003) Encephalotrigeminal angiomatosis. SADJ 58(9):370–373
Goscinski I, Kunicki A (1972) On surgical treatment of Sturge–Weber syndrome. Acta Med Pol 13:229–236
Griffiths PD (1996) Sturge–Weber syndrome revisited: the role of neuroradiology. Neuropediatrics 27:284–294
Griffiths PD, Boodram MB, Blaser S, Armstrong D, Gilday DL, Harwood-Nash D (1997) 99mTechnetium HMPAO imaging in children with the Sturge–Weber syndrome: a study of nine cases with CT and MRI correlation. Neuroradiology 39:219–224
Gururaj AK, Sztriha L, Johansen J, Nork M (2000) Sturge–Weber syndrome without facial nevus: a case report and review of the literature. Acta Pediatr 89:740–743
Hoffman HJ, Hendrick EB, Dennis M (1979) Hemispherectomy for Sturge–Weber syndrome. Childs Brain 5:233–248
Horita H, Nozaki H, Hamano S, Aihara T (1990) SPECT of the brain in Sturge–Weber syndrome using N-isopropyl-p[123-I]. No To Hattatsu 22:341–348
Ichinose T, Tsuyuguchi N, Morino M, Sunada I, Ohata K, Takami T, Shimonishi Y, Kwabe J, Shiomi S, Hara M (2003) Discrepancy between 18F FDG and 11C-methionine positron emission tomography findings in Sturge–Weber syndrome—case report. Neurol Med Chir 43(9):461–464
Ito M, Sato K, Ohnuki A, Uto A (1990) Sturge–Weber disease: operative indications and surgical results. Brain Dev 12(5):473–477
Juhasz C, Chugani DC, Muzik O et al (2000) Is epileptogenic cortex truly hypometabolic on interictal positron emission tomography? Ann Neurol 48:89–96
Kalischer S (1897) Demonstration des Gehirns eines kindes mit teleangiektasie der linksseitigen gesichts-kopfhaut und hirnoberflache. Berl Klin Wochenschr 34:1059–1067
Kossoff EH, Buck C, Freeman JM (2002) Outcomes of 32 hemispherectomies for Sturge–Weber syndrome worldwide. Neurology 59(1):1735–1738
Krabbe KH (1934) Facial and meningeal angiomatosis associated with calcification of the brain cortex: a clinical and anatomopathological contribution. A M A Arch Neurol Psych 32:737–755
Kuhl DE, Bevilacqua JE, Mishkin MM, Sanders PP (1972) The brain scan in Sturge–Weber syndrome. Radiology 103:621–625
Lee JS, Asano E, Muzik O Chugani DC, Juhasz C, Pfund Z, Philip S, Behen M, Chugani HT (2001) Sturge–Weber syndrome: correlation between clinical course and FDG PET findings. Neurology 57(2):189–195
Lin DM, Barker PB, Kraut MA, Comi A (2003) Early characteristics of Sturge–Weber syndrome, shown by perfusion MR imaging and proton MR spectroscopic imaging. AJNR Am J Neuroradiol 24:1912–1915
Lund M (1949) On epilepsy in Sturge–Weber disease. Acta Psychiatr Neurol 24:569–586
Maria BL (2000) Current management in child neurology. BC Decker, London
Maria BL, Neufeld JA, Rosainz LC et al (1998) Central nervous system structure and function in Sturge–Weber syndrome: evidence of neurologic and radiologic progression. J Child Neurol 13:606–618
Moore GJ, Slovis TL, Chugani HT (1998) Proton magnetic resonance spectroscopy in children with Sturge–Weber syndrome. J Child Neurol 13:332–335
Muller RA, Chugani HT, Muzik O, Rothermel RD, Chakraborty PK (1997) Language and motor function activate calcified hemisphere in patients with Sturge–Weber syndrome: a positron emission tomography study. J Child Neurol 12(7):431–437
Namer IJ, Battaglia F, Hirsch E, Constantinesco A, Marescaux C (2005) Subtraction Ictal SPECT co-registered to MRI (SISCOM) in Sturge–Weber syndrome Clin Nucl Med 30(1):39–40
Ongumekan AO, Hwang PA, Hoffman HJ (1989) Sturge–Weber–Dimitri disease. Role of hemispherectomy in prognosis. Can J Neurol Sci 16:78–80
Pagni CA, Wildi E (1977) Pathogenetic hypothesis of intracortical calcifications in Sturge–Weber disease. Case report following lobectomy. Mod Probl Paediatr 18:250–257
Penfield W (1952) Ablation of abnormal cortex in cerebral palsy. J Neurol Neurosurg Psychiatry 15:73–78
Peterman AF, Hayles AB, Dockerty MB, Love JC (1958) Encephalotrigeminal angiomatosis (Sturge–Weber disease) Clinical study of thirty-five cases. JAMA 167:2169–2176
Pinton F, Chiron C, Enjolras O, Motte J, Syrota A, Dulac O (1997) Early single photon emission computed tomography in Sturge–Weber syndrome. J Neurol Neurosurg Psychiatry 63:616–621
Rappaport ZH (1988) Corpus callosum section in the treatment of intractable seizures in the Sturge–Weber syndrome. Childs Nerv Syst 4:231–232
Rasmussen Th, Mathieson G, Le Blanc F (1972) Surgical therapy of typical and a forme fruste variety of the Sturge–Weber syndrome. Schweiz Arch Neurol Psychiatr 111: 393–409
Reid DE, Maria BL, Drane WE, Quisling RG, Hoang KB (1997) Central nervous system perfusion and metabolism abnormalities in Sturge–Weber syndrome. J Child Neurol 12:218–222
Roach ES (1992) Neurocutaneous syndromes. Pediatr Clin North Am 39:591–620
Roach ES, Bodensteiner J (1999) Neurologic manifestations of Sturge–Weber syndrome. In: Bodensteiner JB, Roach ES (eds) Sturge–Weber syndrome. The Sturge–Weber Foundation, Mount Freedom, NJ, pp 27–38
Roach ES, Riela AR, Chugani HT et al (1994) Sturge–Weber syndrome. Recommendations for surgery. J Child Neurol 9:190–192
Schrimer R (1860) Ein fall von telangiektasie. Albrecht von Graefes. Arch Ophtalmol 7:119–121
Sturge WA (1879) A case of partial epilepsy apparently due to a lesion of the vasomotor centers of the brain. Trans Clin Soc Lond 12:162–167
Sujansky E, Conradi S (1995) Outcome of Sturge–Weber syndrome in 52 adults. Am J Med Genet 57:35–45
Sujansky E, Conradi S (1995) Sturge–Weber syndrome. Age of onset of seizures and glaucoma and the prognosis for affected children. J Child Neurol 10:49–58
Tallman B, Tan OT, Morelli JG et al (1991) Location of port-wine stains and the likelihood of ophthalmic and/or central nervous system complications. Pediatrics 87:323–327
Thomas-Sohl KA, Vaslow DF, Maria BL (2004) Sturge–Weber Syndrome: a review. Pediatr Neurol 30(5):303–310
Tuxhorn IEB, Pannek HW (2002) Epilepsy surgery in bilateral Sturge–Weber syndrome. Pediatr Neurol 26(5):394–397
Venes JL, Linder S (1989) Sturge–Weber–Dimitri syndrome. Encephalotrigeminal angiomatosis. In: Edwards MSB, Hoffman HJ (eds) Cerebral vascular disease in children and adolescents. Williams & Wilkins, Baltimore, pp 337–341
Ville D, Enjolras O, Chiron C, Dulac O (2002) Prophylactic antiepileptic treatment in Sturge–Weber disease. Seizure 11(3):145–150
Weber FP (1922) Right-sided hemi-hypertrophy resulting from right sided congenital spastic hemiplegia. J Neurol Psychopathol 3:134–139
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Di Rocco, C., Tamburrini, G. Sturge–Weber syndrome. Childs Nerv Syst 22, 909–921 (2006). https://doi.org/10.1007/s00381-006-0143-2
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DOI: https://doi.org/10.1007/s00381-006-0143-2