ABSTRACT
If the lymphedema occurs around the time of puberty and is sporadic, it is called lymphedema praecox (Figure 87.1); however, it is called Meigs’ disease if it is inherited – probably as an autosomal dominant but with variable penetrance.Approximately 80% of patients with congenital lymphedema have the onset of their
disease at the time of puberty; the majority of cases are seen in girls near the menarche.1 If the lymphedema first becomes manifest as an adult it is referred to as lymphedema tarda. Patients with congenital lymphedema rarely have it isolated to the external genitalia but rather have lower extremity involvement which may be symmetric or one extremity may be more involved than the other. Rarely upper extremity lymphedema occurs as well.3 There are varying degrees of associated lymphedema of the external genitalia in these patients. Congenital lymphedema has been associated with Turner’s, Noonan’s, Klinefelter’s, yellow nail and intestinal lymphangiectasis syndromes.1 Pathologically the lymphatics of the affected tissues are dysplastic and may be categorized into one of three groups: aplastic; hypoplastic, the majority of cases; and hyperplastic.4
