Summary of Recommendations

Document Overview

Design and created by Guideline Central in participation with the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.

Evaluation and Management of Postsurgical Patient With Hirschsprung Disease

North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition

Publication Date: Mar 4, 2023

Page Last Updated: May 5, 2026

Overview of assessment of a symptomatic postsurgical patient

Children who experience postoperative problems should have a detailed history to assess for new onset or worsening symptoms with emphasis on identifying postoperative complications.
Thorough physical examination should be performed including growth parameters, abdominal examination, and perineal/rectal examination if possible.
The original pathology should be reviewed by an experienced pathologist to ensure that there is no residual aganglionosis or transition zone pull-through. If the pathology is inadequate or not available, a re-biopsy can be done at the time of rectal exam under anesthesia to ensure normal enteric innervation of the pull-through bowel
Diagnostic imaging, including abdominal X-ray and contrast enema, should be considered in order to evaluate for mechanical or anatomical abnormalities.
Rectal exam under anesthesia should be performed by an experienced surgeon to assess for mechanical causes for obstruction and to evaluate the integrity of the anorectum.
Once the anatomic and pathologic evaluation has been completed and is negative, then a functional assessment of the anorectum and colon should be considered at a designated Neurogastroenterology & Motility Center.
Anorectal Manometry (ARM) is used to evaluate the voluntary (sensation, squeeze, and ability to bear down) and the involuntary (resting anal pressure and the presence and characteristics of the recto-anal inhibitory reflex) properties of the anorectum (23). Some children may have elevated intra-anal pressures and benefit from botulinum toxin injection into the anal sphincter. Others may have abnormal (deficient) intra-anal pressures or squeeze pressures indicative of damage to the anal sphincter complex.
Colonic manometry (CM) is used to evaluate the neuromuscular integrity of the colon and for planning medical and surgical management (23–26). CM by a neurogastroenterology and motility expert should be considered in patients who continue to have symptoms refractory to treatment in whom all anatomic and pathologic causes for obstruction have been ruled out.

Document Overview

Document Title: Evaluation and Management of Postsurgical Patients with Hirschsprung Disease

Authoring Society: North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition

Document Publication Date: Mar 4, 2023

Page Last Reviewed/Updated: May 5, 2026

Document Type: Consensus Statement

Country of Publication: United States

Full Text Freely Available: Yes

Full Text Guideline: d1pij0k2lbf86p.cloudfront.net/wp-content/uploads/2023/03/Evaluation_and_Management_of_Postsurgical_Patient.27-4.pdf

Source Citation: Ambartsumyan L, Patel D, Kapavarapu P, Medina-Centeno RA, El-Chammas K, Khlevner J, Levitt M, Darbari A. Evaluation and Management of Postsurgical Patient With Hirschsprung Disease Neurogastroenterology & Motility Committee: Position Paper of North American Society of Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN). J Pediatr Gastroenterol Nutr. 2023 Apr 1;76(4):533-546. doi: 10.1097/MPG.0000000000003717. Epub 2023 Jan 31. PMID: 36720091.

Document Scope, Criteria, and Use Cases

Document Objectives: This position paper offers evidence-based general principles for the evaluation and management of postoperative patients with HD who present with defecation problems. It demonstrates the indispensable collaborative effort between Gastroenterology and Surgery subspecialties in the care of patients with HD. Children with Hirschsprung disease have postoperative long-term sequelae in defecation that contribute to morbidity and mortality and significantly impact their quality of life. Pediatric patients experience ongoing long-term defecation concerns, which can include fecal incontinence (FI) and postoperative obstructive symptoms, such as constipation and Hirschsprung-associated enterocolitis. The American Pediatric Surgical Association has developed guidelines for management of these postoperative obstructive symptoms and FI. However, the evaluation and management of patients with postoperative defecation problems varies among different pediatric gastroenterology centers. This position paper from the Neurogastroenterology & Motility Committee of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition reviews the current evidence and provides suggestions for the evaluation and management of postoperative patients with Hirschsprung disease who present with persistent defecation problems.

Scope: Assessment and Screening, Diagnosis, Management

Diseases/Conditions (MeSH): D006627 - Hirschsprung Disease

Keywords: Hirschsprung Disease

Target Patient Population: Postoperative patients with Hirschsprung disease who present with defecation problems

Target Provider Population: Pediatricians, pediatric gastroenterologists, pediatric hepatologists and other allied providers

Inclusion Criteria: Male, Female, Adolescent, Child, Infant

Health Care Settings: Ambulatory

Intended Users: Dietician/Nutritionist, Nurse, Nurse Practitioner, Physician, Physician Assistant

Recommendation Development Processes & Methodology

Number of Source Documents: 112

Includes peer/external review process?: Yes

Includes public comment process?: No

Methodologist involvement?: Yes

Patient involvement?: No

Includes multi-disciplinary group?: Yes

Includes systematic review?: Yes

Grades quality of strength of evidence?: No

Grades quality of strength of recommendation?: No

Includes benefits/harms analysis with recommendations?: No

Medication	Dosage
Oral stimulants
Sennosides	Tablet (8.6 mg/tab)/liquid (8.8 mg/5 mL)/chewable (15 mg/chew): 1 mo to < 2 y: 2.2 mg QD–8.8 mg QD 2 y to < 6 y: 4.4 mg QD–17.6 mg QD 6 y to < 12 y: 8.8 mg QD–35.2 mg QD >12 y: 17.6 mg QD–70.4 mg QD
Bisacodyl	Tablet (5 mg/tab) 3–10 y: 5 mg QD 10 to <12 y: 5–10 mg QD >12 y: 5–15 mg QD
Anti-motility agents
Loperamide	0.1–0.8 mg/kg/d divided TID to QID (max 16 mg/d)
Atropine/diphenoxylate	0.3–0.4 mg/kg/d divided QID (max 10 mg/d diphenoxylate)
Hyoscyamine Oral drops, elixir, tablet (sublinual)	Dosing based on weight and age
Dicyclomine	10 mg PO TID-QID (max 40 mg/d) (children >8 y)
Cholestyramine	Toddler 2 g QID Child 2 g BID to 4 g TID Adolescent 4 g BID to 4 g TID/QID
Suppositories/low volume enemas
Glycerin	Suppository Infants to 6 y: pediatric 1 g > 2 w > 6 y: adult 2 g Enema Infants to 6 y: PediaLax 2.8 g (4mL) >6 y: Fleet 5.4 g (7.5 mL)
Bisacodyl	Suppository/enema 2–8 y: 5 mg QD >8 y and adolescents: 5–10 mg QD
Docusate	Enema 2 to < 12 y: 100 mg/5 mL QD, 283 mg/5 mL QD >12 y: 283 mg/5 mL QD to TID
High volume enemas/ACE solution
Normal Saline / GoLytely / Tap Water	10–20 mL/kg (maximum 1 L)
Additives
Glycerin	Start with 5% concentration and can increase to max of 15% (ie, 20 mL glycerin/400 mL of normal Saline → 60 mL glycerin/400 mL normal Saline)
Castile Soap	Typically, castile soap is maxed out at 18–20 mL
Bisacodyl	Can be given 10–15 min pre or post enema/ACE solution. Some centers mix the bisacodyl into the flush which can dilute the bisacodyl.

Evaluation and Management of Postsurgical Patient With Hirschsprung Disease

Summary of Recommendations

Overview of assessment of a symptomatic postsurgical patient

Recommendations for a patient with postoperative obstructive symptoms

Recommendations for the approach of a patient with postoperative constipation

Recommendations for a patient with postoperative FI

Dosages of frequently used oral and rectal medications (including antegrade enemas)

Document Overview

Document Scope, Criteria, and Use Cases

Recommendation Development Processes & Methodology