Abstract
Purpose
This study was aiming to explore the risk factors contributing to enterostomy in neonates with Hirschsprung disease (HD) to provide a reference for clinicians to make treatment decisions.
Methods
Medical records of 284 patients diagnosed with HD during the neonatal period were retrospectively analyzed. The patients were divided into 2 groups based on operative intervention (one stage transanal pull-through, versus enterotomy and staged transanal pull-through). Univariate and multivariable logistic regression analysis was performed to identify risk factors contributing to enterostomy.
Results
The incidence of enterostomy was 12.0% (34/284) in neonates with HD. Univariate and multivariate logistic regression analysis showed that serum albumin < 25.4 g/L, radiographic results as subphrenic free air, and level of aganglionosis with long-segment or total colonic aganglionosis (TCA) were independent risk factors of enterostomy in neonates, with OR of 42.045 (6.131, 288.319), 285.558 (26.651, 3059.694) and 15.573 (4.319, 56.157), respectively.
Conclusions
The low serum albumin level, bowel perforation, and level of aganglionosis with long-segment or TCA could influence the occurrence of enterostomy in neonates with HD.
Similar content being viewed by others
References
Langer J (2013) Hirschsprung disease Current opinion in pediatrics 25(3):368–374. https://doi.org/10.1097/MOP.0b013e328360c2a0
Ambartsumyan L, Smith C, Kapur R (2020) Diagnosis of Hirschsprung Disease. Pediatr Dev Pathol 23(1):8–22. https://doi.org/10.1177/1093526619892351
de Lorijn F, Boeckxstaens G, Benninga M (2007) Symptomatology, pathophysiology, diagnostic work-up, and treatment of Hirschsprung disease in infancy and childhood. Curr Gastroenterol Rep 9(3):245–253. https://doi.org/10.1007/s11894-007-0026-z
Sulkowski J, Cooper J, Congeni A, Pearson E, Nwomeh B, Doolin E et al (2014) Single-stage versus multi-stage pull-through for Hirschsprung’s disease: practice trends and outcomes in infants. J Pediatr Surg 49(11):1619–1625. https://doi.org/10.1016/j.jpedsurg.2014.06.002
Zani A, Eaton S, Morini F, Puri P, Rintala R, Van Heurn E et al (2017) European paediatric surgeons' association survey on the management of Hirschsprung disease. Eur J Pediatr Surg 27(1):96–101. https://doi.org/10.1055/s-0036-1593991
Kyrklund K, Sloots CE, de Blaauw I, Bjornland K, Rolle U, Cavalieri D et al (2020) ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease. Orphanet J Rare Dis 15(1):164. https://doi.org/10.1186/s13023-020-01362-3
Das K, Mohanty S (2017) Hirschsprung Disease - current diagnosis and management. Indian J Pediatr 84(8):618–623. https://doi.org/10.1007/s12098-017-2371-8
Bradnock T, Knight M, Kenny S, Nair M, Walker G (2017) The use of stomas in the early management of Hirschsprung disease: findings of a national, prospective cohort study. J Pediatr Surg 52(9):1451–1457. https://doi.org/10.1016/j.jpedsurg.2017.05.008
Bradnock T, Walker G (2011) Evolution in the management of Hirschsprung’s disease in the UK and Ireland: a national survey of practice revisited. Ann R Coll Surg Engl 93(1):34–38. https://doi.org/10.1308/003588410x12771863936846
Singh S, Croaker G, Manglick P, Wong C, Athanasakos H, Elliott E et al (2003) Hirschsprung’s disease: the Australian Paediatric Surveillance Unit’s experience. Pediatr Surg Int 19(4):247–250. https://doi.org/10.1007/s00383-002-0842-z
Ieiri S, Higashi M, Teshiba R, Saeki I, Esumi G, Akiyoshi J et al (2009) Clinical features of Hirschsprung’s disease associated with Down syndrome: a 30-year retrospective nationwide survey in Japan. J Pediatr Surg 44(12):2347–2351. https://doi.org/10.1016/j.jpedsurg.2009.07.055
Fuhrman MP, Charney P, Mueller CM (2004) Hepatic proteins and nutrition assessment. J Am Diet Assoc 104(8):1258–1264. https://doi.org/10.1016/j.jada.2004.05.213
Wessner S, Burjonrappa S (2014) Review of nutritional assessment and clinical outcomes in pediatric surgical patients: does preoperative nutritional assessment impact clinical outcomes? J Pediatr Surg 49(5):823–830. https://doi.org/10.1016/j.jpedsurg.2014.01.006
Keckler SJ, Yang JC, Fraser JD, Aguayo P, Ostlie DJ, Holcomb G et al (2009) Contemporary practice patterns in the surgical management of Hirschsprung's disease. J Pediatric Surg 44(6):1257–60; discussion 1260. https://doi.org/10.1016/j.jpedsurg.2009.02.050
Suita S, Taguchi T, Ieiri S, Nakatsuji T (2005) Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J Pediatric Surg 40(1):197–201; discussion 201–2. https://doi.org/10.1016/j.jpedsurg.2004.09.052
Pastor A, Osman F, Teitelbaum D, Caty M, Langer J (2009) Development of a standardized definition for Hirschsprung’s-associated enterocolitis: a Delphi analysis. J Pediatr Surg 44(1):251–256. https://doi.org/10.1016/j.jpedsurg.2008.10.052
Zhu T, Zhang G, Meng X, Yang J, Niu Y, He Y et al (2022) Enterocolitis is a risk factor for bowel perforation in neonates with Hirschsprung’s Disease: a retrospective multicenter study. Front Pediatr 10:807607. https://doi.org/10.3389/fped.2022.807607
Langer J, Rollins M, Levitt M, Gosain A, Torre L, Kapur R et al (2017) Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung disease. Pediatr Surg Int 33(5):523–526. https://doi.org/10.1007/s00383-017-4066-7
Hukkinen M, Koivusalo A, Rintala R, Pakarinen M (2014) Restorative proctocolectomy with J-pouch ileoanal anastomosis for total colonic aganglionosis among neonates and infants. J Pediatr Surg 49(4):570–574. https://doi.org/10.1016/j.jpedsurg.2013.07.021
Gosain A, Frykman P, Cowles R, Horton J, Levitt M, Rothstein D et al (2017) Guidelines for the diagnosis and management of Hirschsprung-associated enterocolitis. Pediatr Surg Int 33(5):517–521. https://doi.org/10.1007/s00383-017-4065-8
Author information
Ethics declarations
Competing interests
The authors declare no competing interests.
Conflict of interest
The authors declare no competing interest.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Liu, Z., Zhang, Y., Li, S. et al. Risk factors of enterostomy in neonates with Hirschsprung disease. Int J Colorectal Dis 37, 1127–1132 (2022). https://doi.org/10.1007/s00384-022-04151-5
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00384-022-04151-5