Characteristic roentgen findings of colon in exudative enteropathy secondary to Hirschsprung's disease.

Abstract

Examination of the colon can be diagnostic for exudative enteropathy in infants with Hirschsprung's disease. Thus, it is not enough for the radiologist to suspect and confirm the occurrence of Hirschsprung's disease on initial abdominal scout films and barium enema studies. He must look one step beyond the identification of an aganglionic segment, to see whether characteristic mucosal changes are present, to make the diagnosis of associated plasma protein loss into the gastrointestinal tract. This added information may mean the difference between successful and unsuccessful management by the responsible pediatrician and surgeon. In children with exudative enteropathy without aganglionosis, the presenting ages, symptoms, and associated roentgen findings are nonspecific (4). The clinical and extensive laboratory studies of the series of cases to be reported here will be completely discussed elsewhere. Briefly, exudative enteropathy is primarily a disease of childhood in which protein loss through the intestinal tract is the direct cause of hypoproteinemia without associated renal or hepatic disease. Edema, distention, gastrointestinal distress, diarrhea, failure to thrive, etc., are nonspecific findings. The I131 polyvinylpyrrolidone (PVP) excretion test is the most specific laboratory procedure among many others (1–9). Study Material At the time of writing, we have studied 10 patients with confirmed exudative enteropathy at The University of Minnesota Hospitals. These have been divided into two groups. The first was made up of 4 patients with infantile Hirschsprung's disease and considerable loss of protein through the intestinal tract. The second was a nonspecific group of 6 with varying degrees of abdominal distention, diarrhea, and/or constipation, ascites, and, of course, loss of plasma proteins. A third control group of 6 nephrotic patients with a nonexudative enteropathic form of hypoproteinemia was studied by the same laboratory and roentgen technics as the other two groups. Methods of Study Exclusive of routine hemograms, electrolyte studies, total serum protein analyses, blood vitamin A and carotene studies, I131-labeled polyvinylpyrrolidone (I131 PVP), Fe59, and Cr51Cl3 tests were used to determine whether the hypoproteinemia was caused by exudative enteropathy (1–3; 5–9). As mentioned, the intricacies of the clinico-laboratory approach will be presented elsewhere. All patients were studied by means of preliminary abdominal scout films and barium enemas. Some in the second and third groups also had upper gastrointestinal tract and small-intestine roentgenologic studies. Findings In the 4 cases of aganglionosis, the diagnosis was confirmed by punch biopsy, surgical excision, or autopsy. No ganglion cells were demonstrated in the tissue specimens. Hypoproteinemia and loss of protein into the intestinal tract were observed in each patient by conventional and special laboratory examinations.