Abstract
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. 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. 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). 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 III.
Highlights
Hirschsprung disease (HD) is a congenital disorder characterized by absent or impaired neural innervation of the distal segment of the bowel
Exclusion criteria were: patients receiving preoperative care in another hospital, patients operated in another hospital, patients receiving surgery before the suspicion HD was made, patients in whom no medical report or no histopathological confirmation of HD was available, patients who died preoperatively owing to a cause that was unrelated to HD, patients not receiving surgery for HD because of conservative treatment, patients diagnosed after the age of six months, and patients that did not provide informed consent
We analyzed 200 patients of whom 68 were excluded: In 32 patients preoperative care was performed outside the Amsterdam UMC, in nine patients preoperative documentation was missing, three patients died prior to definitive surgery of a HD unrelated cause, three patients underwent surgery before the suspicion of HD, 16 patients were diagnosed after the age of six months and five caretakers did not provide informed consent to participate in the study
Summary
Hirschsprung disease (HD) is a congenital disorder characterized by absent or impaired neural innervation of the distal segment of the bowel This results in obstructive symptoms, which are often present in the first week after birth [1]. 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%). 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.
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