TY - JOUR
T1 - Risk factors for complications in patients with Hirschsprung disease while awaiting surgery
T2 - Beware of bowel perforation
AU - Beltman, L.
AU - Labib, H.
AU - Oosterlaan, J.
AU - van Heurn, E.
AU - Derikx, J.
N1 - Funding Information: This study was supported with a grant from the Dutch patient association for Hirschsprung disease. Publisher Copyright: © 2022 The Author(s)
PY - 2022/11/1
Y1 - 2022/11/1
N2 - © 2022 The Author(s)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.
AB - © 2022 The Author(s)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.
KW - Clavien-Dindo
KW - Complications
KW - Hirschsprung disease
KW - Preoperative obstructive therapy
KW - Transanal irrigation
UR - http://www.scopus.com/inward/record.url?scp=85127319287&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.jpedsurg.2022.02.022
DO - https://doi.org/10.1016/j.jpedsurg.2022.02.022
M3 - Article
C2 - 35354528
SN - 0022-3468
VL - 57
SP - 561
EP - 568
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
IS - 11
ER -