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Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Published online by Cambridge University Press:  18 September 2015

A. Oluwole Ogunmekan ,
Paul A. Hwang  and
Harold J. Hoffman
A. Oluwole Ogunmekan*
Affiliation:
Division of Neurology, The Hospital for Sick Children, Toronto
Paul A. Hwang
Affiliation:
Division of Neurology, The Hospital for Sick Children, Toronto
Harold J. Hoffman
Affiliation:
Division of Neurosurgery, The Hospital for Sick Children, Toronto
*
Department of Paediatrics, Lagos University Teaching Hospital, P.M.B. 12,003 Lagos, Nigeria
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Abstract:

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We report 12 patients with Sturge-Weber-Dimitri disease treated surgically between January 1975 and December 1987. Hemispherectomy was performed on ten, two others underwent occipital lobectomy for intractable seizures. All operations were performed between the ages of 3 months and 20 months, except in two at age 8 and 9 years. The onset of seizures in all was between 2 and 8 months of age, except for two at 15 months. There were no postoperative deaths. Postoperative shunt procedures were required in 3 out of 12 (25%). Postoperative seizure control for one year or more was achieved in 11 out of 12 patients. The remaining patient is on medication with no seizures, but follow-up is less than a year. Intellectual deterioration was not seen after surgery except in 2 patients who had late operations. We conclude that patients with Sturge-Weber-Dimitri syndrome who have intractable seizures in the first 6 months of life and unilateral hemispheric involvement should be considered for early resection of the involved hemisphere.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 16 , Issue 1 , February 1989 , pp. 78 - 80
Copyright
Copyright © Canadian Neurological Sciences Federation 1989

References

REFERENCES

1.Sturge, W. A case of partial epilepsy, apparently due to a lesion of one of the vasomotor centers of the brain. Trans Clin Soc London 1879; 12: 162–167.Google Scholar
2.Weber, FP. Right sided hemi-hypertrophy resulting from right-sided congenital spastic hemiplegia, with morbid condition of the left side of the brain revealed by radiograms. J Neurol Psychopathol 1922; 3; 134–139.CrossRefGoogle Scholar
3.Dimitri, V. Tumor cerebral congnito (angioma cavernoso). Revista Asoc Méd Argentina 1923; 36: 1029.Google Scholar
4.Ambrosetto, P, Ambrosetto, G, Michelucci, R. Sturge-Weber syndrome without port-wine facial naevus. Report of 2 cases stud-ied by CT. Child’s Brain 1983; 10: 387–392.Google Scholar
5.Taby, AB, Nagaraja, D, Das, S, et al. Sturge-Weber-Demitri disease without facial nevus. Neurology 1987; 37: 1063–1064.Google Scholar
6.Chaudary, R. Sturge-Weber syndrome with extensive intracranial calcification contralateral to the bulk of the facial nevus. Am J Neurol Res 1987; 8 July p. 786.Google Scholar
7.Cohen, HJ, Kay, MN. Associated facial hemangioma and intracranial lesion (Weber-Dimitri disease). Am J Dis Child 1941; 62: 606–612.Google Scholar
8.O’Brien, CS, Porter, WC. Glaucoma and nevus flammeus. Arch Ophthalmol 1933; 9: 715–728.CrossRefGoogle Scholar
9.Geerarts, WJ. Ocular syndromes, 3rd ed. Philadelphia, Lea and Febiger, 1976; pp410.Google Scholar
10.Kramer, W. Klippel-Trenaunay syndrome. In: Vinken, PJ, Bruyn, GW, (eds.). Handbook of Clinical Neurology, Vol 14. Phakomatoses. New York, Elsevier, 1972; pp. 390–404.Google Scholar
11.Stimac, GK, Solomon, MA, Newton, TH. CT and MRI of angiomatous malformations of the choroid plexus in patients with Sturge-Weber disease. Am J Neurol Res 1986; 7: 623–627.Google ScholarPubMed
12.Kobayashi, J, Wong, A, Hwang, P, et al. Surgery for intractable seizures in infants and children. Can J Neurol Sci 1985; 12: 178.Google Scholar
13.Hwang, P, Gilday, DL, Ash, JM, et al. Pertubations in Regional Cerebral Blood Flow Detected by SPECT Scanning with 99mTc-HmPAO Correlate with EEG abnormalities in children with Epilepsy. J Cer blood Flow and Metab 1987; 7: Suppl 1, S573.Google Scholar
14.Alexander, GL, Norman, R-M. The Sturge-Weber syndrome. John Wright, Bristol. 1960, pp. 55–70, 73, 79, 80.Google Scholar
15.Lund, M. On epilepsy in Sturge-Weber disease. Acta Psychiat Neurol 1949; 24: 578–586.CrossRefGoogle Scholar
16.Ramirez de Arrelano, M. Hemidecortication in monkeys. Comparison of rate and degree of recovery of neurological deficit as related to age at time of operation. Excerpta Medica Int Congr Series 38 1961; 150–151.Google Scholar
17.Hoffman, HJ, Hendrick, EB, Dennis, M, et al. Hemispherectomy for Sturge-Weber syndrome. Child’s Brain 1979; 233–248.Google ScholarPubMed