Congenital Lymphangiectatic Edema

Abstract

Last year 1 we reported a rare case of congenital edema which presented unusual features with reference to the lymphatic system. In this report we mentioned certain characteristics which clearly defined this type of edema as an entity different in many respects from congenital elephantiasis, further emphasized by a review of the literature.A few months after publication of this report, the child developed an acute infection which in a short time terminated fatally. Necropsy was performed, which brought to light some unusual and interesting pathologic conditions. However, before describing in detail the necropsy observations, we shall present a brief r\l=e'\sum\l=e'\of the clinical features as embodied in our previous article. CASE REPORTHistory.\p=m-\H.B., a white girl, born June 10, 1923, presented the following picture: (1) a markedly edematous left foot, leg and thigh; (2) edema of the left labium majus and the right shoulder, arm, hand and fingers; (3) a complete macroglossia with edema of the right lower lip, with a sharp line of demarcation at the center, and (4) edema of the right ear, the right upper and lower lids and enlargement of the right axillary lymph nodes. The general appearance was one of gigantism or elephantiasis. The affected areas were of firm consistency with slight pitting, and the right hand presented an extreme degree of puffiness, with a sharp crease marking the wrist line. The tongue practically filled the mouth. The left side of the face, the left shoulder, arm, hand and fingers were normal in appearance.