Evaluation and Management of Postsurgical Patient With Hirschsprung Disease
Summary of Recommendations
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.
Recommendations for a patient with postoperative obstructive symptoms
- The evaluation of children with postoperative obstructive symptoms should begin with a detailed history and physical examination.
- Diagnostic testing, such as contrast enema, should be performed to evaluate for mechanical causes of obstruction.
- 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.
- Rectal exam under anesthesia should be performed to evaluate for mechanical causes of obstruction.
- Children with a negative anatomic and pathologic evaluation should be started on daily stimulant laxatives. The dose should be tailed to achieve adequate colonic emptying.
- Children who are compliant but are not responsive to oral laxative therapy should be referred to a Neurogastroenterology & Motility Center for evaluation and management.
- Children with obstructive symptoms who have normal or elevated IAS pressures on ARM should receive IAS botulinum toxin administration into the anal sphincter.
Recommendations for the approach of a patient with postoperative constipation
- The evaluation of children with postoperative constipation and FI should begin with a detailed history and physical examination.
- Diagnostic testing, including abdominal X-ray and radiopaque marker study, can be used to differentiate between retentive and non-retentive FI.
- Contrast enema should be performed to evaluate for mechanical causes of obstruction.
- 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.
- Rectal exam under anesthesia should be performed to evaluate for mechanical causes of obstruction.
- Children with a negative anatomic and pathologic evaluation should be started on daily stimulant laxatives.
- Children with fecal impaction should undergo fecal disimpaction prior to initiation or escalation of daily stimulant medications. The dose of daily stimulant medications should be tailored to achieve adequate colonic emptying.
- Children who are compliant but are not responsive to oral laxatives therapy should be referred to a Neurogastroenterology & Motility Center for evaluation and management.
- Functional assessment of the anorectum and the colon should be considered using anorectal and CM respectively.
- Management should entail escalation of daily stimulant medication and implementation of high volume retrograde and antegrade enemas that facilitate colonic emptying.
Recommendations for a patient with postoperative FI
- The evaluation of children with postoperative FI should begin with a detailed history and physical examination.
- Diagnostic testing including abdominal X-ray and radiopaque marker study can be used to differentiate between retentive and non-retentive FI.
- Rectal exam under anesthesia should be performed to assess the integrity of the anorectum, specifically the anal sphincter complex and the dentate line.
- Initial management should involve constipating diet and soluble fiber to increase stool consistency and bulk. Children should be encouraged to have timed toilet seating after each meal and to limit snacking between meals.
- Children who are compliant but are not responsive to the changes in diet and behavior should be referred to a Neurogastroenterology & Motility Center for evaluation and management.
- Functional assessment of the anorectum and the colon should be considered using anorectal and CM respectively.
- Management should entail initiation of antimotility agents that modify stool consistency and transit and implementation of low volume retrograde and antegrade enemas that facilitate colonic emptying.
Dosages of frequently used oral and rectal medications (including antegrade enemas)
| 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. |
Recommendation Grading
Disclaimer
Overview
Title
Evaluation and Management of Postsurgical Patients with Hirschsprung Disease
Authoring Organization
North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition
Publication Month/Year
March 3, 2023
Last Updated Month/Year
April 1, 2024
Document Type
Consensus
Country of Publication
US
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.
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
Scope
Diagnosis, Assessment and screening, Management
Diseases/Conditions (MeSH)
D006627 - Hirschsprung Disease
Keywords
Hirschsprung Disease
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.