Lymphedema is present from the knees downward and
scrotal swelling may be considerable in males. Lymphedema usually presents
during the second half of the first decade of life. One side may be affected
many years before the other. Irritation of the cornea caused by the eyelashes, with
corneal ulceration and scarring in some cases, brings the patient to the
attention of an ophthalmologist. This form of lymphedema is associated with
other congenital malformations, including (a) congenital heart disease
(tetralogy of Fallot, patent ductus arteriosus, ventricular septal defect,
atrial septal defect, coarctation of aorta, and pulmonary stenosis) and
development of dilated cardiomyopathy; (b) spinal arachnoid cysts (in some
patients, neurologic signs may be present); and (c) diverse anomalies of the
vertebral column. The course and prognosis depend upon the extent and
severity of the edema and the development of spinal column complications.
Symptomatic management (compression, elevation, diuretics) of the edema is
common. Removal of the lid hair to prevent ocular complications is done.