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Multicenter Study
. 2014 Nov;49(11):1619-25.
doi: 10.1016/j.jpedsurg.2014.06.002. Epub 2014 Jul 10.

Single-stage versus multi-stage pull-through for Hirschsprung's disease: practice trends and outcomes in infants

Jason P Sulkowski  1 , Jennifer N Cooper  1 , Anthony Congeni  1 , Erik G Pearson  2 , Benedict C Nwomeh  1 , Edward J Doolin  2 , Martin L Blakely  3 , Peter C Minneci  1 , Katherine J Deans  4
Affiliations
Multicenter Study

Single-stage versus multi-stage pull-through for Hirschsprung's disease: practice trends and outcomes in infants

Jason P Sulkowski et al. J Pediatr Surg. 2014 Nov.

Abstract

Purpose: The aim of this study was to evaluate surgical treatments and outcomes in a multi-institutional cohort of neonates with Hirschsprung's disease (HD).

Methods: Using the Pediatric Health Information System (PHIS) from 1999 to 2009, neonates diagnosed with HD were identified and classified as having a single stage pull-through (SSPT) or multi-stage pull-through (MSPT). Diagnosis and classification algorithms and clinical variables and outcomes were validated by multi-institutional chart review. Groups were compared using logistic regression modeling and propensity-score matched analysis to account for baseline differences between groups.

Results: 1555 neonates with HD were identified; 77.2% underwent SSPT and 22.8% underwent MSPT. Misclassification of disease or surgical treatment was <2%. Rates of SSPT increased over time (p=0.03). Compared to SSPT, patients undergoing MSPT had significantly lower birth weights and higher rates of prematurity, non-HD gastrointestinal anomalies, enterocolitis, and preoperative mechanical ventilation. Patients undergoing MSPT had significantly higher rates of readmissions (58.5 vs. 37.9%) and additional operations (38.7 vs. 26%). Results were consistent in the propensity-score matched analysis.

Conclusion: Most neonates with HD undergo SSPT. In patients with similar observed baseline characteristics, MSPT was associated with worse outcomes suggesting that some infants currently selected to undergo MSPT may have better outcomes with SSPT. However, there remains a subgroup of MSPT patients who were too ill to be adequately compared to SSPT patients; for this subgroup of severely ill infants with HD, MSPT may be the best option.

Keywords: Hirschsprung's disease; Multi-stage pull-through; Outcomes; PHIS; Pediatric Health Information System; Primary pull-through; Single stage pull-through.

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Figures

Figure 1
Figure 1
Identification of study cohort of neonatal Hirschsprung’s Disease patients. *Includes patients who had an ostomy procedure by age 60 days but who died at that admission (N=2) or died at a later admission (N=22) before age 1, without ever having a pull-through procedure.
Figure 2
Figure 2
Variability across PHIS hospitals in the percent of patients who undergo multi-stage pull-through procedures. Unadjusted estimates and 95% CIs from a mixed effects logistic regression model with hospital specific intercepts are represented by the open circles and solid error bars. The asterisks denote hospitals that were significantly higher or lower than average (as shown by the reference line).
See this image and copyright information in PMC

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