Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease

Abstract

Although most children with Hirschsprung disease ultimately do well, many experience a variety of ongoing problems after pull-through surgery. The most common include obstructive symptoms, soiling, enterocolitis and failure to thrive. The purpose of this guideline is to present a rational approach to the management of postoperative obstructive symptoms in children with Hirschsprung disease. The American Pediatric Surgical Association Board of Governors established a Hirschsprung Disease Interest Group. Group discussions, literature review and expert consensus were then used to summarize the current state of knowledge regarding causes, methods of diagnosis, and treatment approaches to children with obstructive symptoms following pull-through for Hirschsprung disease. Causes of obstructive symptoms post-pull-through include mechanical obstruction; persistent or acquired aganglionosis, hypoganglionosis, or transition zone pull-through; internal sphincter achalasia; disordered motility in the proximal intestine that contains ganglion cells; or functional megacolon caused by stool-holding behavior. An algorithm for the diagnosis and management of obstructive symptoms after a pull-through for Hirschsprung disease is presented. A stepwise, logical approach to the diagnosis and management of patients experiencing obstructive symptoms following pull-through for Hirschsprung disease can facilitate treatment. Level of evidence V.

Does Hirschsprung-Associated Enterocolitis Differ in Children With and Without Down Syndrome?

“…Other adjuvant therapies, including Botox administration into the anal sphincters are currently being studied at our institution and can be considered in select high-risk patients with HD in whom an anatomic cause for their obstructive symptoms has been ruled out. 28 We acknowledge several limitations to our study. First, the retrospective design relies on the accurate and complete input of data into the medical record, and important clinical details including the administration of rectal irrigations before admission could not be determined in cases where it is not explicitly stated.…”

Obstructive complications after pull-through for Hirschsprung’s disease: different causes and tailored management

“…This long spur leads to obstructive symptoms and subsequent recurrent enterocolitis. It could be managed effectively by simple division of this spur, and the gastrointestinal anastomosis (GIA) stapler could be used for this purpose with good results [11]. In our series, two patients had this complication and were successfully managed by this technique.…”

Redo surgery with longitudinal resection for dilated bowel in Hirschsprung disease: an illustrative case series

“…sex, age at primary pull-through, age at longitudinal resection), (2) disease characteristics and clinical course (i.e. length of the initial affected and resected segment, previous interventions), (3) the procedure of longitudinal resection (i.e. what bowel segment was treated with longitudinal resection, how long this segment was, whether an open or laparoscopic procedure was performed, what type of suturing was used, whether the operation was performed under the protection of an ostomy) and (4) clinical course after longitudinal resection (i.e.…”

“…Thirty to 50% of patients experience defecation problems after primary surgical treatment [1,2], of which persistent obstructive problems such as constipation are the most common. Possible causes for persistent obstructive complaints include anastomotic stricture, residual aganglionosis, mechanical obstruction due to adhesions, sphincter problems and stool holding behaviour [3]. Persistent obstructive defecation problems are primarily treated with conservative measures, including laxatives, rectal irrigation, intrasphincteric botulinum toxin injections or anal dilatation [3].…”

“…Possible causes for persistent obstructive complaints include anastomotic stricture, residual aganglionosis, mechanical obstruction due to adhesions, sphincter problems and stool holding behaviour [3]. Persistent obstructive defecation problems are primarily treated with conservative measures, including laxatives, rectal irrigation, intrasphincteric botulinum toxin injections or anal dilatation [3]. If obstructive symptoms are treated inadequately, this may lead to recurrent faecal stasis and impaction resulting in progressing and permanent distension of the bowel.…”