Factors influencing the incidence of Hirschsprung associated enterocolitis (HAEC)

Le‐Nguyen, Annie; Righini-Grunder, Franziska; Piché, Nelson; Fauré, Christophe; Aspirot, Ann

doi:10.1016/j.jpedsurg.2019.01.026

Cited by 41 publications

(65 citation statements)

References 23 publications

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“…Noteworthy, a UK survey by Huddard in 1998 suggested that 73% of surgeons would operate on HSCR within a month since diagnosis [17]. Only two papers in our systematic review addressed this issue and suggested to wait an average of 9 weeks (ranging between 6 weeks and 2 to 4 months) when a neonate receive a diagnosis of HSCR [7,9]. Even so we do not know what most surgeons would do in case of delayed diagnoses (those occurring outside neonatal period) as none reported nor indicated the expected delay between diagnosis and surgery in various age groups.…”

Section: Age At Surgery and Time-span Between Diagnosis And Surgerymentioning

confidence: 98%

“…Five documents were excluded as dealing with post-mortem only or as wrongly focused. A total of 10 full-text papers turned out to be eligible to address all concerns related to timing for surgery in patients with HSCR [5][6][7][8][9][10][11][12][13][14]. See Figure 1 for PRISMA details and Table 2 for the list of selected full-text papers.…”

Section: Prismamentioning

confidence: 99%

“…This is the most frequent, severe and potentially fatal preoperative complication of HSCR patients, which can be devastating particularly in newborns or in patients with congenital heart diseases [4]. The selected papers suggested a weighted median incidence of 12% (ranging between 8% to 34%) [8][9][10][12][13][14]. Although preoperative HAEC mortality decreased over time [13], it is still reported and occurs with a median incidence of 6% (ranging between 2.4% and 8% [10,13]) thus posing an important burden on the treatment of the disease.…”

Section: Preoperative Haec Morbidity and Mortalitymentioning

confidence: 99%

“…Although preoperative HAEC mortality decreased over time [13], it is still reported and occurs with a median incidence of 6% (ranging between 2.4% and 8% [10,13]) thus posing an important burden on the treatment of the disease. Some Authors suggested that older age at diagnosis [7], low birth weight, and extended aganglionosis [9,12] could represent predisposing factors for preoperative HAEC occurrences. Preoperative HAEC seem to have significant morbidity, as they can increase early readmission and impact on short-term outcome [5].…”

Section: Preoperative Haec Morbidity and Mortalitymentioning

confidence: 99%

“…Cass in 1986 suggested that the earlier is the better [15], Jiang ZX and coworkers in 1994 suggested surgery to be done in the neonatal period [16]. On the other hand, recent evidences suggested that newborns can safely wait a couple of months before being operated as most reports suggest that surgery should be performed between 2 and 4 months of age [6,7,[9][10][11][12]. Only a minority of surgeons operate at an older age [5,13].…”

Section: Age At Surgery and Time-span Between Diagnosis And Surgerymentioning

confidence: 99%

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Time-dependent management of Hirschsprung disease during Covid-19 pandemic

Prato¹,

Arnoldi²,

Faticato³

et al. 2020

Preprint

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INTRODUCTION – During Covid-19 pandemic, some elective non-deferrable surgical procedures has been maintained on a local basis to avoid complications of time-dependent issues. The aim of this paper is to suggest a possible strategy for the management of patients with Hirschsprung (HSCR) during this dramatic Covid-19 pandemic considering.MATERIALS AND METHODS - A systematic literature search on PubMed and Embase of all available literature addressing “Hirschsprung”, “preoperative enterocolitis”, “preoperative mortality”, “complications”, and “timing” in all possible combination has been performed limiting documents to the last 10 years and excluding non-English papers.RESULTS – Systematic review assessed 170 papers and focused on 10 full-text papers addressing the same issues as above. The incidence of preoperative HAEC proved to be as high as 34% with a mortality between 2.4% and 8%. Age at surgery varied in literature reports but recent evidences suggested that patients should be operated around 3 months of age after effective bowel decompression. Rectal irrigations should not be continued over 2 to 4 months to avoid nutritional issues and subsequent possible increase of surgical complications.CONCLUSIONS – Based on available literature, contingent shortage of health resources and necessity to avoid hospital overcrowding during this pandemic, we suggest as follows: 1) surgery should be delayed for healthy neonates up to 3 months of age, and a diverting enterostomy consider for those with ultralong HSCR, unwell or with ineffective nursing; 2) surgery can be delayed but rectal irrigations cannot be maintained indefinitely (ideally within 2 to 4 months at maximum); 3) Surgery could be postponed in older patients, provided previous bouts of HAEC are excluded. This chosen behaviour of our Center for Digestive Diseases aims at avoiding risk of complication and serve the best for our patients in this delicate setting of Covid-19 pandemic.

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Section: Age At Surgery and Time-span Between Diagnosis And Surgerymentioning

confidence: 98%

Section: Prismamentioning

confidence: 99%

Section: Preoperative Haec Morbidity and Mortalitymentioning

confidence: 99%

Section: Preoperative Haec Morbidity and Mortalitymentioning

confidence: 99%

Section: Age At Surgery and Time-span Between Diagnosis And Surgerymentioning

confidence: 99%

See 3 more Smart Citations

Time-dependent management of Hirschsprung disease during Covid-19 pandemic

Prato¹,

Arnoldi²,

Faticato³

et al. 2020

Preprint

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Screening of undernutrition in children with Hirschsprung disease using preoperative anthropometric parameters: A multicenter cross‐sectional study

Wang

Zhu

et al. 2022

J Parenter Enteral Nutr

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Background The prevalence of malnutrition is unknown in patients with Hirschsprung disease. Undernutrition is associated with poor clinical outcomes. This study aims to describe the nutrition status among patients with Hirschsprung disease at admission. Methods We retrospectively used data from children with Hirschsprung disease admitted to three pediatric surgery centers in China from January 2016 to December 2020. The weight‐for‐age z scores (WAZ), height‐for‐age z scores (HAZ), and body mass index‐for‐age z scores (BAZ) were calculated as the reference for nutrition risk according to the World Health Organization child growth standards. The nutrition status of enrolled children was described and nutrition risk in each clinical characteristic was compared. The association between nutrition status and clinical outcomes was analyzed using univariate and multivariate logistic regression. Results A total of 624 patients were included in this study. The mean WAZ, HAZ, and BAZ of all patients was −0.64 ± 1.40, −0.45 ± 1.78, and −0.43 ± 1.50, respectively. Moderate to severe overall undernutrition was 16.3% (102/624). We found that WAZ and BAZ were significantly reduced with the length of aganglionic segments (P = 0.001). Children who had a definitive surgery at 3 years of age or older had significantly lower HAZ (P = 0.001). A multivariate regression model assessing postoperative Hirschsprung‐associated enterocolitis showed that the WAZ was one of the independent risk factors (P = 0.001). Conclusion Undernutrition is prevalent among children with Hirschsprung disease. Nutrition assessment to identify individuals at risk of undernutrition for further intervention is necessary.

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Clinical relevance of inflammation on rectal biopsy for Hirschsprung disease: An outcomes analysis

Lotakis,

Rubalcava,

Lopyan

et al. 2023

J. pediatr. gastroenterol. nutr.

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ObjectivesInflammation on diagnostic rectal biopsy for children with suspected Hirschsprung disease (HSCR) is reported on pathology, and its significance is unknown. We describe the management and outcomes of a cohort with inflammation on rectal biopsy compared to those without. Specifically, to address the hypothesis that inflammation on diagnostic biopsy is associated with increased complication rates irrespective of intervention type and timing.MethodsA single institution retrospective review of children with HSCR who underwent biopsy and endorectal pull‐through (ERPT) from 2010 to 2020 was performed. The primary outcome was overall complications at 30‐days following ERPT. Secondary outcomes included timing and type of operative intervention as well as postoperative enterocolitis diagnosed within 6‐months of ERPT.ResultsForty‐nine children were identified; inflammation was present on diagnostic biopsy for 17 children. Those with inflammation were more likely to have clinical evidence of enterocolitis at the time of biopsy (p = 0.001) and were more likely to undergo leveling colostomy before ERPT (p = 0.01). Children with inflammation had a higher anastomotic leak rate (p = 0.04). Subgroup analysis of patients with inflammation undergoing primary ERPT versus leveling colostomy demonstrated no significant difference in outcomes following definitive ERPT.ConclusionsOur study suggests inflammation on diagnostic rectal biopsy for HSCR is associated with increased anastomotic leak rates. While additional prospective studies are indicated, attention to methods of mitigating inflammation and confirming its resolution before definitive pull‐through may be of benefit for improving clinical outcomes in patients found with inflammation on diagnostic rectal biopsy.

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Factors influencing the incidence of Hirschsprung associated enterocolitis (HAEC)

Cited by 41 publications

References 23 publications

Time-dependent management of Hirschsprung disease during Covid-19 pandemic

Time-dependent management of Hirschsprung disease during Covid-19 pandemic

Screening of undernutrition in children with Hirschsprung disease using preoperative anthropometric parameters: A multicenter cross‐sectional study

Clinical relevance of inflammation on rectal biopsy for Hirschsprung disease: An outcomes analysis

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