Congenital Enlargement of the Extremities

Abstract

In the past, there has been a tendency to designate as elephantiasis all types of overgrowth of the extremities without proper regard to the existence of various kinds of hypertrophy, distinct from the point of view of their differing etiologic associations. That there is a real need of applying to the problem of skeletal overgrowth the type of effort that has long been directed toward the correction of conditions which produce arrested growth is a fresh and valuable point of view emphasized by Chandler.1 A discussion of congenital enlargement of the extremities may profitably include both conditions involving the soft parts alone and conditions in which the entire extremity is affected. Three chief types of overgrowth can thus be discussed: congenital lymphedema (lymphangiectasis), congenital hemangiectatic hypertrophy of the extremities and Recklinghausen's neurofibromatosis. CONGENITAL LYMPHEDEMA (LYMPHANGIECTASIS) This condition is characterized by the presence at birth of nonpitting edema restricted to the skin and subcutaneous tissue of part or all of an extremity, which results in disproportionate enlargement of the limb.2 There is no associated pain, increase of temperature or ulcération of the involved skin, and the afflicted subject is otherwise in good health.Mason and Allen 2a divided congenital lymphedema into two types, the simple and the familial (Milroy's disease). While these types are indistinguishable clinically, they are differentiated simply by the occur¬ rence in the latter type of more than 1 instance of the condition in a family. Undoubtedly, as pointed out by Allen,3 a number of the cases From the Northern Michigan Children's Clinic.