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

Study Overview
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Brief Summary
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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. 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.

Detailed Description
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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. 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. The patients with a predicting score of more than 5 are diagnosed with HD, and are performed surgery to remove the aganglionic bowel. 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.

Official Title
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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

Conditions
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Hirschsprung Disease

Intervention / Treatment
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* Drug: high dose lactulose
* Behavioral: conservative treatment
* Drug: paraffin oil


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 1 Day
Maximum Age: 3 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: surgery treatment<br>The patients with a predicting score of more than 5 are diagnosed with HD, and are performed surgery to remove the aganglionic bowel. The patients with a score less than 5 are mostly indicative of HAD, and receive conservative treatments 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. one stage pull through procedure to remove the dysganglionic bowel segments. | Drug: high dose lactulose<br>* 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.<br>* Other names: Duphalac®;Behavioral: conservative treatment<br>* 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.<br>* Other names: colonic irrigation;Drug: paraffin oil<br>* 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.<br>* Other names: Shilayou®;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| the predicting score calculation | 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 IDs are recorded. The predicting score is calculated by summing the scores of the risk factors and 3 preoperative tests. The patients with a predicting score of more than 5 are diagnosed with HD, and are performed surgery to remove the aganglionic bowel. 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. | 6-12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| pathological diagnosis | The intestinal specimens were reviewed by 3 experienced pathologists all of whom had participated in a consensus meeting on diagnostic criteria of IDs. | 12-18 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Intestinal dysganglinosis, Hirschsprung disease, Hirschsprung disease allied disorders, diagnostic scoring system, Prospective study