Intestinal lymphangiectasia.

Abstract

In 1959 Gordon (1) investigated a group of patients who had been diagnosed as having “hypercatabolic hypoproteinemia.” He measured the fecal content of 131I polyvinylpyrrolidone which had been injected intravenously and was able to demonstrate an increased loss of this material through the intestinal mucosa. He concluded that their hypoproteinemia resulted from the loss of albumin in a similar fashion. Despite minimal or nonexistent intestinal symptoms, he termed this condition “exudative enteropathy.” In 1961 Waldmann et al. (2) further defined the role of the gastrointestinal tract in a group of patients with the diagnosis of “idiopathic hypoproteinemia” by studying their albumin turnover and loss. In 12 of the patients, known causes of gastrointestinal protein loss (regional enteritis, ulcerative colitis, giant hypertrophy of the gastric mucosa, neoplasm, and sprue) had been excluded. This group consisted of 5 males and 7 females, all under twenty-eight years of age at the onset of symptoms. Each had generalized edema at some time in the course of the disease, and in all but one some diarrhea developed, usually mild to moderate in degree. In 3 patients, however, the diarrhea was severe and accompanied by clinical steatorrhea. The fecal fat determinations were elevated in 8 of the 9 tested. Pathologic material was obtained from the intestine in all 12 cases. The predominant histologic feature was dilatation of lymphatics, which contained fat-laden macrophages in the mucosa and submucosa. Waldmann named this condition “intestinal lymphangiectasia.” In some of Waldman's cases and in subsequent reports lymphatic abnormalities were found beyond the gastrointestinal tract. Hypoplasia of the lower extremity lymphatics, occlusion of the thoracic duct, partial obstruction at the diaphragm, agenesis of retroperitoneal nodes, and a tortuous double thoracic duct have been demonstrated by lymphography (3, 4). Case Report This twenty-two-year-old white female had a history of diarrhea consisting of eight watery stools per day for four years prior to her initial visit to our institution. During the past two to three years edema developed about her extremities and mouth. Carpopedal muscle spasms occurred on several occasions, and considerable weight was lost. The patient appeared thin and pale, with generalized muscle-wasting. Her liver was palpable 2 cm beneath the right costal margin. Pertinent laboratory findings included a calcium of 6.1 mg per 100 cc, phosphorus of 2.7 mg per 100 cc, albumin of 1.9 g per 100 cc, and globulin of 1.8 g per 100 cc. There was an elevated neutral fat content in the stools and impaired absorption of 131I triolein. Additional isotope studies were interpreted as showing increased catabolism of albumin and an abnormal loss of albumin into the gastrointestinal tract. There was no protein loss into the urine.