Evaluation and Management of Total Colonic Hirschsprung Disease: A Comprehensive Review From the American Pediatric Surgical Association (APSA) Hirschsprung Disease Interest Group.

Bradley Segura, Kristy L Rialon, Jacob C Langer, Michael D Rollins, Prathima Nandivada, Marc A Levitt, Elizaveta Bokova, Irene Isabel P Lim, Rebecca M Rentea, Richard J Wood, Jason S Frischer, Ankush Gosain

Journal: Journal of pediatric surgery 2024;59(11):161677

PMID: 39217005

Full Text on Elsevier Science View on PubMed

Abstract

BACKGROUND

Children with total colonic Hirschsprung disease (TCHD) are a unique group of patients with pre- and postoperative management challenges. This review provides a rational, expert-based approach to diagnosing and managing TCHD.

METHODS

The guidelines were developed by the Hirschsprung Disease Interest Group members established by the American Pediatric Surgical Association (APSA) Board of Governors. Group discussions, literature review, and expert consensus were used to summarize the current knowledge regarding diagnosis, staged approach, the timing of pull-through, and pre-and postoperative management in children with TCHD.

RESULTS

This paper presents recommendations for managing TCHD before and after reconstruction, including diagnostic criteria, surgical approaches, bowel management, diet, antibiotic prophylaxis, colonic irrigations, and post-surgical considerations.

CONCLUSIONS

A clear understanding of the unique challenges posed by TCHD and consensus on its treatment are lacking in the literature. This review standardizes this patient group's pre- and postoperative management.

LEVEL OF EVIDENCE

Address: Comprehensive Colorectal Center, Section of Colorectal and Pelvic Reconstructive Surgery, Department of Pediatric Surgery, Children's Mercy Kansas City, Kansas City, MO, 64108, USA; Department of Surgery, University of Missouri-Kansas City, Kansas City, MO, 64108, USA. Electronic address: [email protected].; Comprehensive Colorectal Center, Section of Colorectal and Pelvic Reconstructive Surgery, Department of Pediatric Surgery, Children's Mercy Kansas City, Kansas City, MO, 64108, USA.; Colorectal Center for Children, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.; Department of Pediatric Surgery, Children's Hospital of Colorado, University of Colorado, Aurora, CO, USA.; Department of Surgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.; Division of Colorectal and Pelvic Reconstruction, Children's National Medical Center, Washington, DC, 20001, USA.; Colorectal and Pelvic Malformation Center, Department of Surgery, Boston Children's Hospital, Boston, MA, 02115, USA.; Division of Pediatric Surgery, Department of Surgery, Texas Children's Hospital, Texas, USA.; Department of Surgery, Primary Children's Hospital, University of Utah, Salt Lake City, UT, USA.; University of Minnesota, Department of Pediatric Surgery, MHealth Fairview Masonic Children's Hospital, Minneapolis, MN, USA.; Department of Pediatric Colorectal and Pelvic Reconstructive Surgery, Nationwide Children's Hospital, Columbus, OH, USA.; Comprehensive Colorectal Center, Section of Colorectal and Pelvic Reconstructive Surgery, Department of Pediatric Surgery, Children's Mercy Kansas City, Kansas City, MO, 64108, USA; Department of Surgery, University of Missouri-Kansas City, Kansas City, MO, 64108, USA.

DOI: 10.1016/j.jpedsurg.2024.08.017

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MeSH Terms: Child, Humans, Antibiotic Prophylaxis, Colectomy, Hirschsprung Disease, Postoperative Care, Postoperative Complications