The distinctive features of this case of encephalofacial angioinatosisthe bilateral intracranial calcifications denionstratcd radiologically at birth and the early death of the child with angioniatosis of the skull, leptoineningeal melanosis and cerebral and cerebellar malforinations found at autopsy-extend present knowledge of this entity. An extentive search of the literature on the Sturge-Weber syndrome prompted by this profoundly involved case also led to the conclusion that a detailed review of those contributions which defined this syndrome, with citation of the original sources, was in order. Case R e p o r t : J. H. H., a Caucasian male, horn on August 27, 1962 at Michael ReeseHospital, Chicago, exhibited wide-spread portwine staining of the scalp, face, trunk and extremities (Figures 1 a and b). Birth weight was 4.2 Kg ( 9 lbs. 4 0 2 . ) ; the head circumference measured 35.5 cm. Left-sided buphthalmos and mild glaucoma were observed, for which the infant was placed on acetazoleamide and pilocarpine eye-drops.' General physical examination was reported to he otherwise unremark-

Despite successful surgical revascularization of ischemic limbs, a local and systemic reperfusion injury may occur after normal blood reperfusion. Recent experimental and clinical application of controlled limb reperfusion in Europe has demonstrated superior results, with lower morbidity and mortality. This new surgical technique includes modification of the reperfusate (calcium, pH, substrates, osmolarity, free radical scavenger) and the circumstances of initial reperfusion (time, temperature, pressure). This report describes the first application of controlled limb reperfusion after reperfusion injury. A 16-year-old boy underwent femoral access cardiopulmonary bypass for repeat cardiac repair with an ischemic time of 245 minutes. Postoperatively, severe ischemia/reperfusion syndrome developed with muscle contracture, immobility, and anesthesia of the right leg with a second ischemic time of about 6 hours. The systemic creatine phosphokinase level was 88,000 U/L; myoglobin was 27,000 ng/mL. He underwent controlled limb reperfusion by withdrawing blood from the aorta and mixing it with a crystalloid solution (calcium-reduced, hyperosmolar, hyperglycemic, alkalotic, glutamate- and aspartate-enriched, and containing a free radical scavenger) under controlled conditions (blood:crystalloid solution 6:1, for 30 minutes, reperfusion pressure < 50 mm Hg, and normothermia) before establishing normal blood reperfusion. Metabolic data from the central and femoral vein demonstrated a significant reduction of all previous elevated enzyme levels, avoidance of hyperkalemia, normalization of acidosis, and avoidance of systemic reperfusion injury with no multiorgan failure. Limb salvage was accomplished and functional recovery almost complete. To the authors' knowledge, this is the first application of controlled limb reperfusion reported in North America. With this surgical technique we were able to prevent metabolic local and systemic reperfusion changes after prolonged ischemia and also reduced previous reperfusion changes. This report confirms former experimental data, and further clinical studies are warranted.

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