A New Scoring System Improves Diagnostic Accuracy of Intestinal Dysganglionosis --a Prospective Study

Efficacy and Accuracy of a New Diagnostic Scoring System to Differentiate Hirschsprung Disease From Hirschsprung Disease Allied Disorders in the Patients With Suspected Intestinal Dysganglinosis: a Prospective Study

1. The investigators previously reported a simple diagnostic scoring system to differentiate Hirschsprung disease (HD) from Hirschsprung disease allied disorders (HAD) in the patients with suspected intestinal dysganglionosis. In the retrospective study, the investigators concluded that the patients with a predicting score of more than 5 are more likely to be diagnosed with HD, whereas a score less than 5 is mostly indicative of HAD.
2. Since it is essential to confirm the accuracy and efficacy of the scoring system in a prospective manner before it is used as a standard procedure, this prospective study is designed and performed.

1. Patients have hard or firm stools for 2 or less per week, and with ages from newborn to 3 years old are recruited in this study.
2. The recruited patients is undergoing preoperative work-up including barium enema (BE), anorectal manometry (ARM) and histochemical acetylcholinesterase (AChE) staining of rectal mucosa. Known risk factors for intestinal dysganglionosis (IDs) are recorded. The predicting score is calculated by summing the scores of the risk factors and 3 preoperative tests.
3. The patients with a predicting score of more than 5 are diagnosed with HD, and are performed surgery to remove the aganglionic bowel.
4. The patients with a score less than 5 are mostly indicative of HAD, and receive conservative therapies that included colonic irrigation, enema, high dose lactulose, and oral paraffin oil for at least 6 months. When there is no clinical improvement, patients are consented for surgical procedures to remove the dysganglionic bowel segments.

Inclusion Criteria:

• Hard or firm stools for 2 or less per week
• Age are from newborn to 3 years old

Exclusion Criteria:

• Children>3 years of age
• Patients presented severe inflammation or malnutrition, unconsciousness or perforation of intestine