Evaluation of suspicious Hirschsprung disease in children using radiologic investigation method: a prospective observational study

Abstract

Background: Hirschsprung disease (HD) is a developmental disorder characterized by absence of ganglia in the distal colon, resulting in a functional obstruction. It is a common cause of pediatric intestinal obstruction. Objective of present study was to evaluate a checklist of radiologic and clinical signs to determine the probability of HD in suspicious patients.Methods: In a diagnostic accuracy study, 19 children with clinical manifestations of HD attended pediatric OPD in a tertiary care teaching hospital, Haldia from January 2014 to December 2016 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 19 patients, 11 (57.89%) cases had HD and 08 (42.11%) cases were without HD. The mean age was 2.793 ± 4.21 months. Abdominal distension, lack of meconium defecation, and constipation were the most common clinical symptoms with frequencies of 15 (78.9%), 11 (57.8%), and 14 (73.68%), 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 59.09%, 49.99%, 59.09% and 56.06% respectively.Conclusions: 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.

Usefulness of Delayed Films of Contrast Enema for Detecting Hirschsprung’s Disease

“…We found the sensitivity of the delayed film to be 81.25% which is similar to data from prior studies that have shown sensitivities between 60% to 99% [9][10][11][12]. With respect to specificity and PPV, in comparison to previous studies, we obtained a high value of 90.9% and 97.5%.…”

“…With respect to specificity and PPV, in comparison to previous studies, we obtained a high value of 90.9% and 97.5%. In a previous study, specificity and PPV were reported as 7.6% and 20.6%, respectively, while another study described the specificity and PPV to be 73.7% and 83.9% [9,12].…”

“…Mortality from HD has seen a reduction from 80% to 13%; this trend is likely due to and highlights the role of early diagnosis [7]. Biopsy remains the gold standard for diagnosis; however, the first investigation is usually a CE examination [8][9][10]. In this study, our main aim was to evaluate the usefulness of the 24-hour delayed film in our center and validate the use of additional radiation that is given to the child via the delayed film.…”

Barium Enema Revisited in the Workup for the Diagnosis of Hirschsprung's Disease

“…The presence of megarectum, common in patients with severe intestinal constipation, the occurrence of air leak during the insufflation of the balloon, and the incorrect positioning of the catheter in the sphincter complex are potential causes for misdiagnosis (2,4). In addition, this method is less available than BE, because of the equipment costs and to the need for an experienced examiner, mainly to evaluate infants younger than 1 year (2,9).…”

“…Previous studies have evaluated the applicability and diagnostic capacity of the RSI in the diagnostic investigation for HD (7)(8)(9)(10). They have shown important differences and conflicting results (7)(8)(9)(10).…”