Management of Hirschsprung’s disease in mainland China

Objective To survey the current status of surgical management for Hirschsprung’s disease (HSCR) in mainland China. Methods Firstly the questionnaire was designed and divided into two parts. Part I was to collect the basic information of respondents while Part II focused upon the current surgical management of HSCR. The questionnaire was distributed through social platform WeChat. Firstly a WeChat group was established and the link of questionnaire sent to respondents. The data was automatically uploaded to "wenjuan.com" after completion. Data analysis was performed with SPSS13.0 statistical software. The maximal margin of error was calculated based on a 95% confidence interval, sample size, sample rate and total population surveyed. When the sampling rate surpassed 5%, the maximal margin of error was corrected by a finite population correction. Results A total of 234 questionnaires were distributed to 148 respondents with a response rate of 63.2%. The questionnaires of 40 attending physicians were not included for data analysis while another 108 questionnaires included. For diagnosing HSCR, 25.9% respondents underwent routine rectal biopsy preoperatively. The maximum margin of error was 6.1%. Rectal suction biopsy accounted for 13.0% and rectal total biopsy 13.0%. And 51.9% received anorectal manometry preoperatively. The maximal margin of error was 7.0%. All respondents received contrast enema preoperatively. Rectal biopsy stains included hematoxylin/eosin (95.3%), acetylcholinesterase (42.6%) and calretinin (21.7%). For surgical management of classical segment HSCR, Soave approach was preferred by 76.9% respondents, 17.6% favored Swenson’s approach and 0.9% adopted Duhamel’s approach and the maximal margin of error was 1.3%. Respondents opting for open surgery were only 0.9% (1/108) and the maximal margin of error was 1.3%. Laparoscopic-assisted and transanal procedures accounted for 45.4% (49/108) and 46.3% (50/108) respectively. Another 6.6% respondents had a choice depending on age. Radical surgery was performed neonatally by 35.2% or delayed by 64.8% and the maximal margin of error was 6.7%. Conclusions Currently the diagnostic strategy of HSCR has remained inconsistent and irregular in mainland China. The application rate of preoperative rectal biopsy and AchE stain is low. Furthermore, professional pediatric pathologists are too few. This directly affects the accuracy of HSCR diagnosis. Especially for classical segment and long-segment HSCR, surgical treatment of HSCR is based on mini-invasive surgery (laparoscopy and transanal technique). However, timing of surgery is not uniform. In 35.2% respondents, radical surgery during neonatal period was associated with a relatively high incidence of postoperative complications. Therefore it is imperative to develop HSCR guidelines, improve the referral system and optimize the management of HSCR. Key words: Hirschsprung disease; Diagnosis; Surgical treatment