The edema in Milroy disease is present from birth
in contrast to the edema, particularly severe below the waist, that develops
around puberty in Meige syndrome. Involvement of the upper limbs, face, and
larynx are notable features in Meige syndrome. There is also an association
with deafness, cleft palate, primary pulmonary hypertension, and
cerebrovascular malformations. In the autosomal recessive form, there is
intestinal lymphedema, facial anomalies (antimongoloid slant of palpebral
fissures, euryblepharon, retrognathia, chemosis), coarctation of aorta,
patent ductus arteriosus, and hypoalbuminemia. At the beginning, the edema
is pitting and disappears with elevation of the affected part. Subsequently,
with development of fibrosis, the edema becomes nonpitting. The overlying
skin becomes hyperkeratotic and waters. Fissures and secondary infections
often occur. Treatment is often symptomatic with elastic stocking, active
muscle exercises, and centripetal massage. Diuretics may be used.
Antibiotics are often required for recurrent infection. Surgical management
of lymphedema does not amount to more than 10% of cases. Excisional
procedures, such as the Thompson operation, are done. Liposuction is an
attractive alternative. The most effective types of surgical treatment are
microsurgical lymphovenous or lymphovenous-lymphatic bypass. Secondary
lymphedema of the lower extremities (more rarely of the upper limbs) is the
primary indication for surgical treatment.