Title: A Prospective Observational Study to Evaluate the Cases of Suspicious Hirschsprung’s Disease in Neonates and Children Using Radiologic Investigation Method

Authors: Pankaj Gupta, Pramod Sakhi, Amlendu Nagar, Kumud Julka, Sheetal Singh, Mamta Gupta

 DOI:  https://dx.doi.org/10.18535/jmscr/v5i9.41

Abstract

Background: Hirschsprung's disease (HD) is a congenital intestinal disorder characterised by aganglionosis of the distal bowel. It is a common cause of pediatric intestinal obstruction.

Aims & Objectives: A standard contrast enema for HD can sometimes be inconclusive in delineating a transition zone especially in neonates and infants. The aim of this study was to determine the utility and diagnostic accuracy of different radiologic methods to evaluate a checklist of radiologic and clinical signs to determine the probability of HD in suspicious patients.

Methods: In a diagnostic accuracy study, 22 children with clinical manifestations of suspected HD attended pediatric OPD in a tertiary care teaching hospital, Indore from June 2015 to June 2017 were assessed. A checklist was used to evaluate the items proposed by contrast enema (CE), based on six subscales, including transitional zone, rectosigmoid index (RSI), irregular contractions in aganglionic region, cobblestone appearance, filling defect due to fecaloid materials and lack of meconium defecation during the first 48 hours after birth. The patients were classified as high score and low score. Sensitivity and specificity were calculated for identifying HD, in comparison with pathologically proved or ruled out HD.

Results: Of the 22 patients, 14 (63.6%) cases had HD and 08 (36.4%) cases were without HD. The mean age was 4.68 ± 5.24 months. Abdominal distension, constipation and lack of meconium defecation were the most common clinical symptoms with frequencies of 18 (81.82%), 15 (68.18%) and 13 (59.09%) respectively. In summary, the mean sensitivity of detecting the radiological signs of transition zone, spastic colon, reversed recto-sigmoid index and the overall impression in histological confirmed HD patients are 67.86%, 53.75%, 60.72% and 60.77% respectively. The mean specificity of detecting the absence of the radiological signs of transition zone, spastic colon, reversed recto-sigmoid index and the overall impression in histological confirmed non-HD patients are 68.75%, 81.25%, 87.5% and 79.17% respectively. This would in turn give an overall mean specificity rate of 79.17% in successfully excluding HD with the above mentioned radiological signs from the contrast enema.

Conclusion: In conclusion, our study underscores the importance of combining the information of a transition zone on a plain abdominal radiograph and contrast enema to decide the surgical approach for the correction of Hirschsprung's in developing countries where laparoscopic facilities are not available.

Keywords: Hirschsprung’s Disease, Neonate, Children, Constipation, Diagnosis, Radiological investigation, Contrast Enema, Sensitivity, Specificity

 

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