Long-term functional outcomes of patients with Hirschsprung disease following pull-through

Gunadi; Theodora Monica Carissa; Stevie; Ezzah Fatmala Daulay; Dicky Yulianda; Kristy Iskandar; Andi Dwihantoro

doi:10.1186/s12887-022-03301-6

Long-term functional outcomes of patients with Hirschsprung disease following pull-through

BMC Pediatr. 2022 May 3;22(1):246. doi: 10.1186/s12887-022-03301-6.

Authors

Gunadi¹, Theodora Monica Carissa², Stevie², Ezzah Fatmala Daulay², Dicky Yulianda², Kristy Iskandar³, Andi Dwihantoro²

Affiliations

¹ Pediatric Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Jl. Kesehatan No. 1, Yogyakarta, 55281, Indonesia. drgunadi@ugm.ac.id.
² Pediatric Surgery Division, Department of Surgery, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito Hospital, Jl. Kesehatan No. 1, Yogyakarta, 55281, Indonesia.
³ Department of Child Health, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/UGM Academic Hospital, Yogyakarta, 55291, Indonesia.

Abstract

Background: Hirschsprung disease (HSCR) is a common congenital disorder presenting with functional obstruction due to aganglionosis of the colon. There are numerous types of pull-through surgery for managing HSCR, such as transabdominal endorectal (Soave), Swenson, Duhamel, transanal endorectal pull-through (TEPT), and laparoscopic (Georgeson) approach. Here, we aimed to describe the long-term outcome of patients with HSCR who underwent transabdominal Soave, Duhamel, and TEPT in our institution.

Methods: We performed a cross-sectional analysis for patients who underwent Duhamel, Soave, and TEPT at our institution from January 2012 to December 2015. Long-term functional outcome was determined by bowel function score (BFS). The BFS was obtained by interviewing patients who had completed at least three years of follow-up.

Results: Twenty-five patients were included in this study who underwent transabdominal Soave (n = 8), Duhamel (n = 4), and TEPT (n = 13). There were 24 patients with short aganglionosis type. The median age of HSCR diagnosis was 10 (IQR = 1-39) months, while the median age of pull-through surgery was 17 (IQR = 7-47) months. The median follow-up of BFS level for HSCR patients after pull-through was 72 (IQR, 54-99) months. There were 11 patients with good BFS level and 10 patients with normal BFS level. Additionally, 50% of Duhamel patients had poor BFS level, while 50% of Soave patients had good BFS level, and 54% of TEPT patients had normal BFS level (p = 0.027). As many as 50% of Duhamel patients showed daily soiling and required protective aids, while 38.5% of TEPT had staining less than 1/week and no change of underwear required, and 50% of Soave patients revealed no soiling, respectively (p = 0.030). Furthermore, 75% of Duhamel patients had accidents, while 75% of Soave and 46.2% of TEPT patients had no accidents (p = 0.035).

Conclusion: Our study shows that the type of definitive surgery might affect the long-term bowel functional outcome; particularly, the TEPT approach might have some advantages over the transabdominal Soave and Duhamel procedures.

Keywords: Bowel function score; Definitive surgery; Hirschsprung disease; Long-term functional outcome; Transabdominal- and transanal pull-through.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Child, Preschool
Cross-Sectional Studies
Defecation
Digestive System Surgical Procedures* / adverse effects
Digestive System Surgical Procedures* / methods
Hirschsprung Disease* / surgery
Humans
Infant
Postoperative Complications / etiology