Are all patients with short segment Hirschsprung’s disease equal? A retrospective multicenter study

• Published in: Pediatric surgery international 2018, Vol.34 (1), p.47-53
• Main Authors: Chung, Patrick Ho Yu, Wong, Kenneth Kak Yuen, Tam, Paul Kwong Hang, Leung, Michael Wai Yip, Chao, Nicholas Sih Yin, Liu, Kelvin Kam Wing, Chan, Edwin Kin Wai, Tam, Yuk Him, Lee, Kim Hung
• Format: Article
• Language: English
• Subjects: Child; Child, Preschool; Colon; Colon, Sigmoid - surgery; Digestive System Surgical Procedures; Female; Hirschsprung Disease - surgery; Humans; Male; Manometry; Medicine; Medicine & Public Health; Original Article; Patient Outcome Assessment; Pediatric Surgery; Pediatrics; Prognosis; Rectum - surgery; Retrospective Studies; Surgery
• ISSN: 0179-0358; 1437-9813
• DOI: 10.1007/s00383-017-4202-4

Background/purpose Short segment Hirschsprung’s disease (HSCR) carries a better prognosis than long segment disease, but the definition of short is controversial. The objective of this study is to determine anatomically the extent of disease involvement that would be associated with a better functional outcome. Methods This is a retrospective multicenter ( n  = 3) study with patients (≥ 3 years) who had transanal pullthrough operation done for aganglionosis limited to the recto-sigmoid colon were reviewed. The extent of disease involvement and bowel resection was retrieved by reviewing the operative records as well as histopathological reports of the resected specimens. Clinical assessment was performed according to the criteria of a seven-itemed bowel function score (BFS) (maximum score = 20). Manometric assessment was performed with anorectal manometry. Results The study period started from 2003 to 45 patients were studied with median age at assessment = 52.0 months and operation = 3.0 months. The disease involvement was categorized into upper sigmoid-descending colon (DC) ( n  = 8), sigmoid colon (SC) ( n  = 12), upper rectum (UR) ( n  = 14) and lower rectum (LR) ( n  = 11) according to the level of normal biopsy result. There was no significant difference in the age of assessment between the four groups. The median BFSs in the DC, SC, UR and LR were 13, 15, 17 and 17, respectively ( p  = 0.01). Nine patients from the DC and SC groups reported soiling for more than twice per week. Sub-group analysis comparing patients with and without the entire sigmoid colon resected revealed worse functional outcomes in terms of the incidence of soiling (40.7 vs 22.2%, p  = 0.05) and the BFS (14 vs 18, p  = 0.04) in the former group. Anorectal manometry did not reveal any significant difference between the four groups, but a higher proportion of patients in the UR and LR groups appeared to have a normal sphincter resting pressure (DC vs SC vs UR vs LR = 62.5 vs 75.0 vs 85.7 vs 80.0%, p  = 0.10). Conclusion Patients with short segment HSCR are not equal at all. HSCR patients with aganglionosis limited to the rectum without the need of removing the entire sigmoid colon have a better bowel control and overall functional score. Less bowel loss and colonic dissection maybe the underlying reasons. Although future studies with a larger sample size and a longer follow-up period are required to validate the results of this study, it has provided a new insight to the current un