PSEUDOHIRCHSPRUNG’S DISEASE- A CLINICOPATHOLOGICAL CORRELATIVE STUDY

Background: Intestinal obstruction in the neonate or infantile period poses problem in the management. The diagnosis and the management can be resolved by taking rectal biopsy and proving them as Hirchsprung’s or Pseudohirchsprung’s disease by histo-pathological examination. It helps in better patient management by avoiding unnecessary surgeries in cases of Pseudohirchsprung’s disease. Aim: To study the pathology of Pseudohirchsprung’s disease and its staining pattern in immunohistochemistry with Neuron specific enolase (NSE) and S- 100. Material & methods: Study included all cases with clinical diagnosis of Hirchsprung’s disease, received in the department of pathology of a Govt. Medical college. Duration of study was 2 years from 2005-2007. A total of 90 cases were studied. Results: Out of the 90 cases, 61(67.7%) were diagnosed as Hirchsprung’s disease, 23 cases (25.60%) as Pseudohirchsprung’s disease. Of the 23 cases of Pseudohirchsprung’s disease, 7 cases showed hyperganglionosis, 7 cases showed hypoganglionosis and 9 cases showed normal ganglionic segment. Immunohistochemistry with NSE & S-100 in selected cases showed increased staining of sub mucosal nerve trunks. Conclusion: One fourth of clinically diagnosed cases of Hirchsprung’s disease were actually Pseudohirchsprung’s disease. No definite IHC pattern could be clearly defined. Further studies are recommended for setting up standards for diagnosis of Pseudohirchsprung’s disease, which will aid in better management of the cases.