Zur Operativen Behandlung des Sturge-Weber-Syndroms

Abstract

New interest in the neurosurgical treatment of the Sturge-Weber-syndrome has been revived since Alexander and Norman (1960) as well as Rasmussen (1972) proposed the idea, that early exstirpation of the lesion may have beneficial effect on the neuropsychological development of children with this syndrome.

In this survey, 66 cases with Sturge-Weber-syndrome, who underwent lobectomy or hemispherectomy have been reviewed. In 35 cases the authors were convinced, that the patient improved after surgery. In all cases, however, the evidence of this statement is if not exclusively mainly based on decrearing frequency of convulsions.

Lobectomy is the most promising procedure, in particular, if the lesion is restricted to one lobe. Prognosis seems to be best if only the occipital lobe is envolved.

Surgical treatment of the Sturge-Weber-syndrome is indicated, if epilepsy cannot be controlled by anticonvulsive drugs. Hemispherectomy must be restricted to cases with additional permanent paralysis and mental defects.

As yet, there is no evidence in the literature on this syndrome that early operation, such as lobectomy before the onset of recurrent fits, paresis or mental defect can prevent the natural course of events. However, after careful consideration it is held that it is justified to recommend early surgery once it has become obvious, that the disease is running a progressive and malignant course.

Keyword

Sturge-Weber-syndrome - lobectomy - hemispherectomy