Pediatric Surgery Department, Hôpital La Timone-Enfants, Marseille, France.
J Pediatr Gastroenterol Nutr. 2019 Apr;68(4):527-532. doi: 10.1097/MPG.0000000000002204.
In Hirschsprung disease (HD), despite successful surgical treatment, 50% of children experience long-term functional gastrointestinal problems, particularly chronic functional obstructive symptoms. We report our experience regarding clinical effects of neurostimulation-guided anal intrasphincteric botulinum toxin (BT) injections on postoperative obstructive symptoms attributed to a nonrelaxing anal sphincter complex in HD patients.
In this monocenter cohort study, 15 HD patients with postoperative functional intestinal obstructive symptoms received neurostimulation-guided anal intrasphincteric BT injections. Short-, medium-, and long-term effects were evaluated. The Bristol stool form scale was used to assess stool consistency, and the Jorge-Wexner (JW) score to assess fecal continence.
The median age at first injection was 4 years. In the short-term, a significant improvement in stool consistency was noted in 12 of 14 patients (P = 0.0001) and JW score decreased for 14 of 15 patients (P = 0.001). In the medium-term, JW score significantly decreased for all patients (P = 0.0001), with an improvement of 50% or more for 10 patients (66.7%). In the long term, 83.3% of patients had normal stool consistency and JW score was <3 for all. Recurrent enterocolitis decreased from 86.7% to 8.3%. A complete resolution of all symptoms without further medication was observed in 66.7% of patients in the long term.
Intrasphincteric BT injection was a safe, effective, and durable option for the management of postoperative functional intestinal obstructive symptoms in HD. The use of neurostimulator guidance for specific delivery of BT to muscular fibers of nonrelaxing anal sphincter complex takes into consideration the variability of patient's anatomy secondary to curative surgery.
在先天性巨结肠（HD）中，尽管手术治疗成功，但仍有 50%的患儿存在长期的功能性胃肠道问题，特别是慢性功能性梗阻症状。我们报告了我们在神经刺激引导下肛门内括约肌肉毒毒素（BT）注射治疗术后非弛缓性肛门括约肌复合体相关梗阻症状方面的经验，这些症状归因于 HD 患者。
在这项单中心队列研究中，15 例 HD 患者术后出现功能性肠道梗阻症状，接受了神经刺激引导下肛门内括约肌 BT 注射治疗。评估了短期、中期和长期疗效。采用布里斯托粪便形态量表评估粪便稠度，采用 Jorge-Wexner（JW）评分评估粪便控制情况。
首次注射的中位年龄为 4 岁。在短期，14 例患者中有 12 例（P=0.0001）粪便稠度显著改善，15 例患者中有 14 例（P=0.001）JW 评分降低。在中期，所有患者的 JW 评分均显著降低（P=0.0001），10 例患者（66.7%）改善 50%或更多。在长期，83.3%的患者粪便稠度正常，JW 评分均<3。复发性肠炎从 86.7%降至 8.3%。在长期，66.7%的患者所有症状完全缓解，无需进一步药物治疗。
内括约肌 BT 注射是治疗 HD 术后功能性肠道梗阻症状的一种安全、有效且持久的选择。神经刺激器引导下将 BT 精确递送至非弛缓性肛门括约肌复合体的肌纤维，考虑到了根治性手术后患者解剖结构的可变性。
