The European Reference Network guideline recommends that children with Hirschsprung disease receive regular interdisciplinary follow-up to adulthood.[27]Kyrklund K, Sloots CEJ, de Blaauw I, et al. ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease. Orphanet J Rare Dis. 2020 Jun 25;15(1):164.
https://pmc.ncbi.nlm.nih.gov/articles/PMC7318734
http://www.ncbi.nlm.nih.gov/pubmed/32586397?tool=bestpractice.com
Follow-up should be frequent in the first year of life and every 1-2 years afterward, and should include assessment of growth, nutrition, development, and social functioning.[27]Kyrklund K, Sloots CEJ, de Blaauw I, et al. ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease. Orphanet J Rare Dis. 2020 Jun 25;15(1):164.
https://pmc.ncbi.nlm.nih.gov/articles/PMC7318734
http://www.ncbi.nlm.nih.gov/pubmed/32586397?tool=bestpractice.com
Guidelines have been proposed for the evaluation of postoperative complications in children with Hirschsprung disease.[88]Ambartsumyan L, Patel D, Kapavarapu P, et al. 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):533-46.
https://onlinelibrary.wiley.com/doi/10.1097/MPG.0000000000003717
http://www.ncbi.nlm.nih.gov/pubmed/36720091?tool=bestpractice.com
[89]Langer JC, Rollins MD, Levitt M, et al. Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int. 2017 May;33(5):523-6.
http://www.ncbi.nlm.nih.gov/pubmed/28180937?tool=bestpractice.com
[90]Saadai P, Trappey AF, Goldstein AM, et al. Guidelines for the management of postoperative soiling in children with Hirschsprung disease. Pediatr Surg Int. 2019 Aug;35(8):829-34.
http://www.ncbi.nlm.nih.gov/pubmed/31201486?tool=bestpractice.com
[102]Ahmad H, Levitt MA, Yacob D, et al. Evaluation and management of persistent problems after surgery for Hirschsprung disease in a child. Curr Gastroenterol Rep. 2021 Oct 11;23(11):18.
http://www.ncbi.nlm.nih.gov/pubmed/34633517?tool=bestpractice.com
[103]Ahmad H, Yacob D, Halleran DR, et al. Evaluation and treatment of the post pull-through Hirschsprung patient who is not doing well; update for 2022. Semin Pediatr Surg. 2022 Apr;31(2):151164.
http://www.ncbi.nlm.nih.gov/pubmed/35690463?tool=bestpractice.com
Importantly, parents and caregivers should seek urgent medical care for any signs and symptoms of Hirschsprung-associated enterocolitis (HAEC).[11]Gosain A, Frykman PK, Cowles RA, et al. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int. 2017 May 33(5):517-21.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5395325
http://www.ncbi.nlm.nih.gov/pubmed/28154902?tool=bestpractice.com
Recurrent episodes of enterocolitis should prompt evaluation of an anatomic cause.[11]Gosain A, Frykman PK, Cowles RA, et al. Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int. 2017 May 33(5):517-21.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5395325
http://www.ncbi.nlm.nih.gov/pubmed/28154902?tool=bestpractice.com
The pathogenesis of HAEC is thought to be due in part to dysbiosis, but there is no good evidence for or against probiotics to prevent HAEC.[12]Lewit RA, Kuruvilla KP, Fu M, et al. Current understanding of Hirschsprung-associated enterocolitis: pathogenesis, diagnosis and treatment. Semin Pediatr Surg. 2022 Apr;31(2):151162.
https://pmc.ncbi.nlm.nih.gov/articles/PMC9523686
http://www.ncbi.nlm.nih.gov/pubmed/35690459?tool=bestpractice.com
[104]Mei F, Wu M, Zhao L, et al. Probiotics for the prevention of Hirschsprung-associated enterocolitis. Cochrane Database Syst Rev. 2022 Apr 26;4(4):CD013714.
https://pmc.ncbi.nlm.nih.gov/articles/PMC9039968
http://www.ncbi.nlm.nih.gov/pubmed/35470864?tool=bestpractice.com