Pleural Effusion In Chronic Hereditary Lymphedema (Nonne, Milroy, Meige's Disease)

Milroy's disease is a congenital familial type of lymphedema which generally involves only the lower extremities up to the inguinal ligament. Letessier in 1865 was the first to report the condition in an account of 4 cases of lymphedema in a single German family. Milroy in 1892 reported 22 cases of an “undescribed variety of hereditary edema” in 6 generations of a single family. In 1899 Meige described an additional 8 cases in a French family. In these 8 cases, however, the onset of edema occurred at puberty rather than at birth. In spite of a considerable amount of literature concerning the disease since that time, we have been unable to find any published report of pleural effusion in association with chronic hereditary lymphedema. Case Reports Case I: E. F., a 44-year-old white male, had suffered from edema of both legs since birth, as had his mother and one sister (Case II). The mother died in 1923 at the age of thirty-three from “septic poisoning.” No previous cases of lymphedema were known to have occurred in the family. The patient's edema was first confined to the legs, but gradually extended up the thighs and after puberty involved the scrotum. The abdominal wall, hands, and conjunctiva also gradually became edematous. The legs, however, were always most markedly involved. Because of the edema the patient has worn high-laced shoes, but never bandages on the legs. He gave a history of multiple erysipelas-like attacks which responded to penicillin therapy. Early in 1958 the patient experienced a fairly severe respiratory infection. He was first seen in October 1958, at which time he was dyspneic on exertion, and physical and roentgenographic examination revealed bilateral pleural effusions. Within three weeks, a total of 3,200 c.c. of a slightly turbid amber fluid was removed from the left hemithorax on 4 thoracenteses. Because of the rapid reaccumulation of fluid, the patient was hospitalized. Laboratory examination at the time of admission revealed the total serum protein to be 5.5 gm. Per cent with 3.1 gm. per cent of albumin and 2.4 gm. per cent of globulin. Thoracentesis was performed on both sides of the chest. Fluid from the left chest showed a total protein content of 2.9 gm. per cent, and from the right, 2.3 gm. per cent. Following multiple thoracenteses, the patient appeared improved and was discharged ten days after admission. Fluid, however, re-accumulated while he was an out-patient. That in the right chest gradually became more turbid and the pleura felt thicker when thoracentesis was repeated. Pulmonary function studies in July 1959 demonstrated a markedly reduced vital capacity and a low maximum breathing capacity. On bronchospirometry vital capacity of the right lung was only 9 per cent of its predicted value.