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

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.

Evaluation of Bowel Function on Patient Post Operative Hirschsprung Disease

“…26,27 Commonly used procedures include TAEPT, Soave, and Duhamel. 26,28 Fourteen patients who received definitive surgery received treatment with the TAEPT procedure, and Duhamel treated 7 patients each (Table 2).…”

“…Patients with definitive treatment affected the outcome of postoperative patients. 28 If the patient is compared with healthy subjects, the patient will have poorer quality of defecation. The quality of defecation in question is rectal sensation, frequency of defecation, frequent stool incontinence, holding defecation, frequent soiling, and impaired social problems.…”

Screening of undernutrition in children with Hirschsprung disease using preoperative anthropometric parameters: A multicenter cross‐sectional study

“…18 However, few studies exist that evaluate undernutrition in patients with HSCR. One study assessed the nutrition status as an end point indicating growth outcome in the HSCR population, 23 but limited information was available on preoperative nutrition risk screening.…”

“… 18 However, few studies exist that evaluate undernutrition in patients with HSCR. One study assessed the nutrition status as an end point indicating growth outcome in the HSCR population, 23 but limited information was available on preoperative nutrition risk screening. To our knowledge, this study is the first to describe the nutrition risk prior to operation using anthropometric measurements in a large sample HSCR population based on three major HSCR treatment centers in China.…”

Assessment of Functional Outcome Following Duhamel Retro-Rectal Pull-Through Surgery for Hirschsprung's Disease - A Follow-up Study

“…HD is a common cause of intestinal obstruction in newborn, requiring surgery. It presents with functional obstruction caused by the aganglionosis of the colon, which is due to the failure of the migration of the neural crest cells in the bowel, 1,2 mainly the distal bowel, beginning at the internal sphincter and extending proximally for varying distances. The incidence of HD ranges from 1 in 5000 to 1 in 12000 live births.…”