Colorectal Center, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Department of Surgery, Children's Mercy Hospital, Kansas City, MO, USA.
J Pediatr Surg. 2023 Aug;58(8):1578-1581. doi: 10.1016/j.jpedsurg.2023.04.008. Epub 2023 Apr 20.
Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence.
Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated.
We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102).
Further prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation.
Level III.
先天性巨结肠相关性结肠炎（HAEC）是先天性巨结肠（HD）患者发病和死亡的最常见原因；直肠注射肉毒毒素（Botox）已被报道为一种可能的预防策略。我们旨在评估我院 HD 患者的历史队列，首先确定 HAEC 的发病率，其次开始评估 Botox 对 HAEC 发病率的影响。
对我院 2005 年至 2019 年间就诊的 HD 患者进行回顾性研究。统计 HD 的发病率、HAEC 和 Botox 注射的频率。评估初始 Botox 治疗或移行带与 HAEC 发病率之间的关系。
我们共回顾了 221 例患者；其中 200 例纳入分析。113 例（56.5%）患者在中位 24 天（IQR 91）时接受了一期经肛门拖出术。87 例（43.5%）初始造口术患者的肠连续性在中位 318 天（IQR 595）时重建。94 例（49.5%）至少发生过一次 HAEC，62 例（66%）发生过多次 HAEC。19 例（9.6%）患者为全结肠型 HD，HAEC 的总发生率明显高于无全结肠型 HD 的患者（89% vs 44%，p<0.001）。6 例（2.9%）患者在经肛门拖出术或造口关闭术时接受了 Botox 注射；1 例发生 HAEC（与未在手术时接受 Botox 注射的患者的 50.7%相比，p=0.102）。
需要进一步进行前瞻性研究以评估 Botox 对先天性巨结肠相关性结肠炎的影响，这是我们下一步的研究方向。
III 级。
