Evaluation and Management of Postsurgical Patient With Hirschsprung Disease

Publication Date: March 4, 2023
Last Updated: March 31, 2023

Summary of Recommendations

Overview of assessment of a symptomatic postsurgical patient

  1. Children who experience postoperative problems should have a detailed history to assess for new onset or worsening symptoms with emphasis on identifying postoperative complications.
  2. Thorough physical examination should be performed including growth parameters, abdominal examination, and perineal/rectal examination if possible.
  3. 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
  4. Diagnostic imaging, including abdominal X-ray and contrast enema, should be considered in order to evaluate for mechanical or anatomical abnormalities.
  5. 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.
  6. 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.
  7. 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.
  8. 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.



Evaluation and Management of Postsurgical Patients with Hirschsprung Disease

Authoring Organization