Treatment of encephalotrigeminal angiomatosis (Sturge-Weber disease) by hemispherectomy.

Encephalotrigeminal angiomatosis or SturgeWeber's disease is an uncommon congenital disorder which can vary greatly in the extent of its manifestations, but which in severe cases can lead to infantile hemiplegia associated with fits, behavioural disorders and mental backwardness. Until comparatively recently specific therapy was largely limited to anticonvulsant drugs. Only a few isolated reports of surgical intervention have been recorded. The purpose of this paper therefore is to describe the findings and results of surgery in five consecutive patients with infantile hemiplegia treated by hemispherectomy and since followed up for from three to eight years who improved as regards epilepsy and personality disorders, although not as regards hemiplegia. A preliminary report on the first two of these cases has already been presented (Polani, 1952). Excellent reviews of the clinical manifestations and pathogenesis of the condition have recently been given by several authors (Medoc, Sotelo, Arana Iniiquez and Estable Puig, 1953-1954; Lichtenstein, 1954; Wohlwill and Yakovlev, 1957; Norman, 1958; Peterman, Hayles, Dockerty and Love, 1958). The essential lesion appears to be a venous angiomatosis of the leptomeninges generally over one cerebral hemisphere, most often in the posterior parietal and occipital regions, associated with a port-wine naevus of the skin of the face in the territory of one or more divisions of the trigeminal nerve, on the same side of the body. Occasionally both cerebral hemispheres are involved, and the cutaneous naevus may also be in other parts of the body. In all cases the affected areas of cortex become atrophic with the deposition of calcium and ferruginous materials that give rise to a characteristic curvilinear appearance on radiographs. Other lesions that may be present include angioma of the choroid of the eye and ipsilateral buphthalmos or glaucoma. Dural and diploic vascularity is exceptional. The first recorded case description is that of Rudolf Schirmer (1860) who described a 36-year-old patient with an extensive naevus involving both sides of the face, left more than right, as well as the chest and abdomen. He did not mention any neurological features, but pointed out that the patient's left eye protruded, and that there was hydrophthalmos and varicosities of the retinal veins. Allan Sturge (1879) demonstrated to the Clinical Society of London a girl of 6j years of age who had an extensive 'port-wine mark' over the right side of the head and face with enlargement of the right eye and vascular changes in the choroid. He inferred that her left-sided fits were caused by a similar vascular lesion of the right side of the brain. Kalischer (1897) gave the first autopsy report of this condition. He (1897; 1901) described an extensive telangiectasis of the scalp and the surface of the brain, all on the same side. He did not, however, observe any calcification, presumably because his subject was only 11 years old. Hebold (1913) was the first to note the cerebral calcification, but did not determine its precise situation. However, Weber (1922; 1929) was the first to publish a radiograph of the double curvilinear shadows that outline the sulcal pattern, although according to Krabbe (1934) Wissing, in 1921, had demonstrated these features to the Radiological Society of Copenhagen. It was Krabbe (1934) himself who demonstrated that the calcium deposits occurred within the cortex and not in the angiomatous vessels. Dimitri (1923), from the Argentine, also published an early description of the radiological appearances. By this time the essential features of the disease were all recorded, but there has been some difference of opinion over nomenclature. 'Sturge-Weber disease' or 'Sturge-Weber syndrome' have become sanctioned by usage, although Weber (1936) disclaimed the use of his name and suggested that it be called 'Sturge-Kalischer disease'. It has also been called 'Krabbe's disease', and 'Sturge-WeberDimitri disease' (Lichtenstein, 1954), and 'SturgeWeber-Krabbe's disease' (Krayenbiihl, Ya,argil and