Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease.
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.
Division of General and Thoracic Surgery, Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada. jacob.langer@sickkids.ca.|Division of Pediatric General and Thoracic Surgery, Hospital for Sick Children, Rm 1524, 555 University Ave, Toronto, ON, M5G 1X8, Canada. jacob.langer@sickkids.ca.
J C
JC
Langer
Primary Children's Hospital, University of Utah School of Medicine, Salt Lake City, UT, USA.
M D
MD
Rollins
Department of Pediatric Surgery, Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, Columbus, OH, USA.
M
M
Levitt
Division of Pediatric Surgery, Department of Surgery, Le Bonheur Children's Hospital, Children's Foundation Research Institute, University of Tennessee Health Science Center, Memphis, TN, USA.
A
A
Gosain
Colorectal Center for Children at Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
L de la
L
Torre
Department of Laboratories, Seattle Children's Hospital and University of Washington, Seattle, WA, USA.
R P
RP
Kapur
Section of Pediatric Surgery, Department of Surgery, Yale University School of Medicine, New Haven, CT, USA.
R A
RA
Cowles
Madigan Army Medical Center, Tacoma, WA, USA.
J
J
Horton
Department of Pediatric Surgery, Women and Children's Hospital of Buffalo, Buffalo, NY, USA.
D H
DH
Rothstein
Department of Pediatric Surgery, Harvard Medical School, Massachusetts General Hospital, Boston, MA, USA.
A M
AM
Goldstein
D001905:Botulinum Toxins / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004733:Enema; D005260:Female; D006627:Hirschsprung Disease / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D007415:Intestinal Obstruction / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D008297:Male; D011183:Postoperative Complications / Q000175:diagnosis* / Q000628:therapy*; D017410:Practice Guidelines as Topic
D001905:Botulinum Toxins
Aganglionosis; Enterocolitis; Functional megacolon; Hirschsprung disease; Hirschsprung-associated enterocolitis; Hirschsprung’s disease; Hirschsprung’s-associated enterocolitis; Hypoganglionosis; Internal sphincter achalasia; Obstruction; Transition zone pull-through