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Risk factors for complications in patients with Hirschsprung disease while awaiting surgery: Beware of bowel
Lieke Beltman1, Hosnieya Labib2, Jaap Oosterlaan3
1Department of Pediatric Surgery, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Gastroenterology and Metabolism Research Institute and Amsterdam Reproduction and Development Research Institute, Meibergdreef 9, Amsterdam 1105 AZ, the Netherland; Department of Pediatrics, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Emma Children's Hospital Amsterdam UMC Follow-Me program and Emma Neuroscience Group, Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherland.
Insights
A 17% complication rate was observed in Hirschsprung disease (HD) patients awaiting surgery, with bowel perforation being the most frequent severe complication. Total colon aganglionosis (TCA) significantly increases complication risk, necessitating timely surgical intervention.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Research
Background:
- Hirschsprung disease (HD) management involves surgical correction typically at 3-6 months of age.
- Preoperative management includes therapies like transanal irrigation (TAI) or laxatives to alleviate obstruction.
- Complications arising during the preoperative period require thorough investigation for improved patient outcomes.
Purpose of the Study:
- To determine the incidence and severity of complications in HD patients awaiting surgery.
- To identify patient-specific factors that predict the development of these preoperative complications.
Main Methods:
- Retrospective analysis of HD patients operated between 2000-2021.
- Complications were graded using the Clavien-Dindo classification system.
- Logistic regression analysis identified predictors of complications.
Main Results:
- 17% of patients (22/132) experienced 45 complications preoperatively; 91% were major.
- Bowel perforation occurred in 7% of patients, with TAI implicated in 5% and associated with total colon aganglionosis (TCA).
- TCA was a significant predictor of complications (OR 9.905, p < 0.001).
Conclusions:
- A 17% preoperative complication rate highlights the risks in HD patients awaiting surgery.
- Bowel perforation, particularly in TCA patients, poses life-threatening risks.
- Early surgical intervention is recommended for patients with suspected or confirmed TCA.
Background:
Patients with Hirschsprung disease (HD) mostly undergo surgery around the age of three to six months. While awaiting surgery, therapy to treat the obstruction such as transanal irrigation (TAI) or laxatives is applied. The aim of this study was to gain insight in the prevalence and severity of complications occurring while awaiting surgery and to identify patient characteristics associated with the development of these complications.
Methods:
This study retrospectively analyzed data of patients with HD operated in our center between 2000 and 2021. Complications emerging while awaiting surgery were graded using Clavien-Dindo (CD). Patient characteristics as predictor of a complication were tested using logistic regression analysis.
Results:
Twenty-two of 132 (17%) included patients (preoperative treatment: 94% TAI; 2% laxatives; 2% other therapy) developed 45 complications while awaiting surgery, including predominantly major complications (91%). Bowel perforation occurred most frequently (n = 9, 7%) wherefrom six caused by TAI (5%), including three patients with total colon aganglionosis (TCA) (2%) counting one life-threatening and one lethal perforation. The other perforations were caused by meconium ileus (n = 2) and Hirschsprung associated enterocolitis (HAEC) (n = 1). Other frequent complications were: sepsis (5%), ileus (4%) and persistent obstruction (4%). Predictive factor for developing complication was TCA (OR 9.905, CI 2.994-32.772, p < 0.001).
Conclusion:
We found a complication rate of 17% in patients while awaiting surgery, reporting bowel perforation most frequently. We found this complication in patients with TCA being highly dangerous causing one life-threatening and one lethal perforation. Therefore, we advise in patients with (suspected) TCA to limit the time awaiting surgery.
Level Of Evidence:
level III.
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