X-ray Findings And Pathologic Basis In Hirschsprung Allied Disease

Objective To explore the X-ray findings and pathologic basis of Hirschsprung allied disorders,improve the understanding to X-ray findings of the neuronal intestinal dysplasia.Methods Forty-seven cases of Hirschsprung's disease and fourteen cases of its allied disorders confirmed by pathology were performed with barium enema examination, the left lateral recumbent , the right recumbent and supine position film were obtained to demonstrate the narrow zone, transition zone, barium retained at 24 hours and irregular contractions. Fourteen cases of Hirschsprung allied disease were also given immunohistochemical staining with neuron-specific enolase , S-100 protein and cathepsin D besides rotine HE stain. A comparison between the X-ray findings of megacolon and Hirschsprung allied disease was made and the sensitivity,specificity,positive predictive value,false positive rate,negative predictive value,false negative rate,Youdens index of the narrow zone,transition zone,barium retained at 24 hours and irregular contractions were calculated.Results The pathology of 14 cases of Hirschsprung allied disease suggested 2 cases of neuronal intestinal dysplasia, 10 cases of hypoganglionosis,1 case of immaturity of ganglion cells,1 case of neuronal hypogenesis. On barium enema, three cases appeared as severe stenosis in rectum,descending colon and sigmoid colon, obvious expansion of the proximal colon, no transition zone, the long narrow zone with more small spasm notch and barium retained at 24 hours, five cases appeared as mild stenosis in the rectum, descending colon or sigmoid colon ,mild expansion of the proximal colon , no transition zone, the long narrow zone with more small spasm notch, barium retained at 24 hours, three cases appeared as "sausage-like" colon with little or no colon expansion accompanied with a small spasm notch, no stenosis, no transition zone, no colonic pouch , bowel dilatation, barium retained at 24 hours,2 cases had jumping narrow zone and 1 case had a thin and small colon that appeared as a short colon frame with small spasm notch, ileum dilation, rectal spasticity. A comparison of the X-ray findings of Hirschsprung's disease with its allied disorders showed that there is great difference in transition zone and the irregular contractions(P<0.05),no difference was found in the narrow zone and the barium retained at 24 hours (P>0.05).Among 47 cases of Hirschsprung's disease, there were12 cases of common type,24 cases of short colon type,10 cases of long colon type and 1 case of total colon type. The sensitivity ,specificity,positive predictive value,false positive rate,negative predictive value , false negative rate and Youdens index about the narrow zone were 85.1%,35.7%,81.6%,64.3%,41.7%,14.9%,20.8% respectively, those about transition zone were 68.1%,92.9%,97.0%,7.1%,46.4%,31.9%,61% respectively, those about barium retained at 24 hours were 89.3%,7.1%,76.4%,92.9%,16.7%,10.6% and 0% respectively,and those about irregular contractions were 36.2%,14.3%,58.6%,85.7%,6.3%,63.8% and 0% respectively.The pathology of Hirschsprung allied disease is characterized by ganglion cells change in quantity and quality, but not ganglion cell deficiency, including neuronal intestinal dysplasia, hypoganglionosis, immaturity of ganglion cells, neuronal hypogenesis. On the contrary, the pathology of Hirschsprung's disease is characterized by no ganglion cells, hyperplasia of neuron fibrosis in the myenteric and submucosal of distal narrow zone of colon. Conclusions1. The pathology of Hirschsprung allied disease is characterized by ganglion cells change in quantity and quality, but not ganglion cell deficiency. Its X-ray findings were varied and not typical. The following X-ray signs on barium enema should be alert to Hirschsprung's allied disease: (1) the long narrow zone without the transition zone between the narrow zone and the expansion zone, the intestinal spasm notch; a large amounts of residual barium at 24-hour review.(2) the entire colon appeared as "sausage-like",no narrow zone, no transition zone, small spasm notch of intestine, a large amounts of residual barium at 24-hour review.(3) Special type: "Jump" stenosis or "total colon" stenosis. These findings may provide clues for further clinical biopsy 2. On the contrary, the pathology of Hirschsprung's disease is characterized by no ganglion cells, hyperplasia of neuron fibrosis in the myenteric and submucosal of distal narrow zone of colon. The transition zone is the most reliable in all the X-ray signs of the megacolon. The barium retained at hour 24, especially mixed barium-stool, had important value to the early diagnosis of Hirschsprung's disease.