PEDIATRICS Vol. 28 No. 1 July 1961, pp. 65-76
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ANGIOMATOSIS (HAMARTOMATOUS HEM-LYMPHANGIOMATOSIS)

Report of a Case with Diffuse Involvement

Peter J. Koblenzer M.D.1 and Martin J. Bukowski M.D.1

1 The Children's Hospital of Philadelphia

A case is described of a diffuse, possibly generalized, abnormality of a hamartomatous nature of the peripheral vascular system. A number of cases from the literature, which also appear to belong in this category, are summarized. Histologic examination shows that lymph and blood vessels may both be involved, though this may be essentially an abnormality of the lymphatic system in which extensive venolymphatic communications occur.

The clinical manifestations vary according to the site of involvement and the extent of dissemination and also according to whether the lesions are predominantly hemangiomatous or lymphangiomatous.

The main features are osteolytic lesions, visceromegaly, cutaneous hemangiomas or lymphangiomas and massive effusions into any body cavity. The effusions are usually chylous, sanguineous or a mixture of both. Any or all of these features may be present in any one case.

The disease frequently has its onset in childhood or adolescence and tends to be progressive. If the lesions are widely disseminated or an effusion into a body cavity is present the outlook is grave.

Treatment so far has been unsuccessful. Surgery may occasionally have a place.

The term angiomatosis is employed to denote this condition not only to underline its potentially extensive nature but also to avoid debate as to whether it is essentially hemangiomatosis or lymphangiomatosis.




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