Pachydermoperiostosis with Multiple Basal Cell Carcinomata

showed the same typical funnelled appearence of the aortic root with supravalvar narrowing (Figure 1), although at catheterization the gradient was found to occur at the level of the valve, presumably as a result of the valve thickening and restricted cusp movement. The xanthomatous deposits may result in narrowing of the coronary ostia causing angina. Such deposits were found at operation in our patient and although she did not have angina, coronary artery bypass grafting to one artery was carned out. Aortic surgery was recommended in this patient in view of the severity of the aortic stenosis. The aortic valve was replaced following enlargement of the aortic root to allow the use of an adequately sized prosthesis, using a technique first described by Manouguian & Seybold-Epting (1979). The aortotomy incision was extended down through the aortic valve ring into the anterior leaflet of the mitral valve, and the defect so created was repaired using a pericardial patch to reconstitute the mitral valve and aortic root while enlarging the valve ring. A more radical surgical approachto this problem, involving combined cardiac and liver transplantation, has been described recently by Starzl et al. (1984), but this would not have been justified in our patient. Her early progress and symptomatic improvement, with rapid increase in both weight and stature, have been excellent and compare very well with the experience of others (Allen et al. 1980). Her prognosis is greatly improved with relief of the aortic stenosis, but more effective control of her hypercholesterolaemia (current level 10.5 mmol/l) will be necessary to prevent further xanthoma formation. The method of choice will be lowdensity lipoprotein apheresis using adsorption columns (Borberg et al. 1983) when this becomes available in the United Kingdom.