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.
Department of Child Health, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/UGM Academic Hospital, Yogyakarta, 55291, Indonesia.
BMC Pediatr. 2022 May 3;22(1):246. doi: 10.1186/s12887-022-03301-6.
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.
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.
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).
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.
先天性巨结肠（HSCR）是一种常见的先天性疾病，表现为结肠无神经节细胞导致的功能梗阻。HSCR 有多种拖出手术方式，包括经腹直肠内（Soave）、Swenson、Duhamel、经肛直肠内拖出术（TEPT）和腹腔镜（Georgeson）方法。本研究旨在描述我院行经腹 Soave、Duhamel 和 TEPT 治疗的 HSCR 患者的长期结局。
我们对 2012 年 1 月至 2015 年 12 月在我院行 Duhamel、Soave 和 TEPT 的患者进行了病例系列分析。采用肠功能评分（BFS）确定长期功能结局。通过对至少完成 3 年随访的患者进行访谈获得 BFS。
本研究纳入了 25 例患者，其中经腹 Soave 手术 8 例（n=8），Duhamel 手术 4 例（n=4），TEPT 手术 13 例（n=13）。24 例患者为短段先天性巨结肠。HSCR 诊断时的中位年龄为 10 个月（IQR=1-39），而拖出手术时的中位年龄为 17 个月（IQR=7-47）。HSCR 患者拖出术后的 BFS 水平中位随访时间为 72 个月（IQR，54-99）。11 例患者 BFS 水平良好，10 例患者 BFS 水平正常。此外，Duhamel 患者 50%的 BFS 水平较差，Soave 患者 50%的 BFS 水平良好，TEPT 患者 54%的 BFS 水平正常（p=0.027）。多达 50%的 Duhamel 患者存在每日污粪，需要使用保护装置，而 38.5%的 TEPT 患者每周污粪少于 1 次，不需要更换内衣，50%的 Soave 患者无污粪（p=0.030）。此外，75%的 Duhamel 患者有意外发生，而 75%的 Soave 和 46.2%的 TEPT 患者无意外（p=0.035）。
我们的研究表明，确定性手术类型可能会影响长期肠功能结局，特别是 TEPT 方法可能优于经腹 Soave 和 Duhamel 手术。
