Hirschsprung’s disease (HD) is a functional intestinal obstruction due to an abnormality of the enteric nervous system, most of affected patients with HD are presented during the neonatal period, but the diagnosis is still non-specific and leading to missing some patients.
THE AIM OF THE STUDY:
Is to construct a simple clinical predictor scoring system to assist early diagnosis, allow early treatment of HD and to avoid doing rectal biopsy as much as possible.
A prospective cohort study was done in Children Welfare Teaching Hospital from the first of January 2016 to the end of December 2017; the study included 52 patients who were admitted to the pediatric surgery department at neonatal period with suspicion of HD. Clinical variables which include (low birth weight, prematurity, family history of HD, male gender, passage of meconium over 48 hours, passage of meconium in first stimulation, feeding intolerance, vomiting, abdominal distention, constipation, diarrhea, dependence on anal stimulation or enemas and important radiological findings) were taken for diagnosis of HD. Rectal biopsy (full thickness) or segment biopsy were done for the included patients to divide them into negative and positive (HD) groups.
According to the statistical results, a new clinical scoring system was constructed and only the variables with significant correlation with the dependent variable (histopathology) were selected.
Male to female ratio among HD patients was (3.7:1). The transitional zone by contrast enema; dependence on anal stimulation and constipation show accuracy of: 92%; 90%; and 90% respectively; while passage of meconium over 48 hours, male gender and diarrhea show accuracy of: 73%; 69%; and 60% respectively. The probability of the variables in the scoring system were measured by the area under (ROC) curve on which constipation, transitional zone by contrast enema and dependence on anal stimulation achieved 2 scores for each, while delayed passage of meconium; diarrhea and male gender achieved 1 score for each. The developed scoring system designed as 9 points, at the cut-off point (4), the score achieves sensitivity; specificity and accuracy of: 93.9%; 100%; and 96% respectively.
CONCLUSION AND RECOMMENDATION:
A developed clinical scoring system is simple and easily applied, and it is useful tool in the early diagnosis and treatment of HD by allowing a single stage pull through operation in small infants. Rectal biopsy is spared for those patients with ≤ 4 scores who are still suffering in the follow up period.