Hirschsprung’s disease: a systematic review

Abstract

Background: Hirschsprung’s disease (HD) is a congenital disorder characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional intestinal obstruction and chronic constipation. Surgical management remains the cornerstone of treatment, with the Swenson and Soave procedures being two of the most widely utilized techniques. The Swenson procedure involves complete resection of the aganglionic segment with a primary anastomosis, while the Soave procedure uses an endorectal pull-through technique, preserving the outer muscular layer of the rectum. However, there is ongoing debate regarding which technique provides superior short- and long-term outcomes. This systematic review aims to compare the postoperative complications and functional results associated with the Swenson and Soave procedures to guide surgical decision-making and improve patient care. Methods: A systematic literature search was conducted in PubMed and ScienceDirect from January 2000 to December 2022. Five comparative studies involving 332 pediatric patients met the inclusion criteria. Data regarding operative time, blood loss, enterocolitis, anastomotic complications, and long-term functional outcomes (continence and constipation) were extracted and analyzed. Results: The Soave procedure was associated with higher rates of postoperative enterocolitis (30.4% vs 15.2%) but better long-term fecal continence (85.2% vs 74.1%). In contrast, the Swenson procedure demonstrated shorter operative times (128±25 min vs. 152±30 min), reduced blood loss (45±12 mL vs. 68±15 mL), and lower rates of constipation (4.8% vs. 11.1%). Anastomotic strictures were more frequent in the Swenson group (6.1% vs 2.0%). Conclusion: The Soave procedure may be preferable for short-segment disease when continence is prioritized, despite its higher enterocolitis risk. Conversely, the Swenson procedure offers advantages in operative efficiency and constipation outcomes, particularly for extensive aganglionosis.