Children's Hospital of Fudan University, Shanghai 200032, People's Republic of China.
J Pediatr Surg. 2009 Dec;44(12):2355-8. doi: 10.1016/j.jpedsurg.2009.07.056.
Total colonic aganglionosis (TCA) is a relatively uncommon and severe condition managed by pediatric surgeons. Several procedures exist for the treatment of TCA. However, there is no current consensus on a superior operative procedure. The objective of this article is to evaluate the comparative effectiveness of the Soave procedure and the Martin procedure in the treatment of TCA with respect to preoperative data and postoperative outcome.
In the period from January 2001 to June 2008, we recruited 29 patients with TCA who were treated with the Martin procedure (14) or the Soave procedure (15). Relevant data were collected from the medical charts kept in the hospital library. The follow-up study used a detailed questionnaire that was answered by patients either by telephone or directly in the clinic. In the clinic, data, including age, body weight before operation, intraoperative blood transfusion, anastomotic leakage, postoperative enterocolitis, durations of any postoperative fever, days in hospital, and a range of functional outcomes, were particularly recorded to enable a comparison of the effectiveness of the Soave procedure and the Martin procedure.
A male predominance has been described for patients with TCA, with a male-to-female ratio of approximately 6.25:1. In the Soave group, time between ileostomy and the definitive procedure, body weight before the definitive procedure, and the operative age were less than those in the Martin group (P < .05). In patients who underwent the Martin procedure, the average duration of postoperative fever and days in hospital were significantly more than those in the Soave group (P < .05). In the Soave group, the incidence of intraoperative blood transfusion (46.7%), abdominal wound infection (6.67%), and anastomotic leakage (0) was significantly less (P < .05) than those in the Martin group (92.8%, 28.5%, and 14.3%, respectively). In the follow-up study, 7 patients in the Martin group had enterocolitis, and 4 of them had severe enterocolitis leading to multiple hospitalizations. In contrast, in the Soave group, only 2 patients had enterocolitis, and 1 was ameliorated after the therapy of anus dilation in the clinic. During the 4 weeks after the operation, all patients had an increased frequency of defecation, with soiling in the perianal area. At 3 months after the operation, there were significantly more patients with normal defecation in the Martin group compared to the Soave group (P < .05). Six months later, 11 (78.6%) patients in the Martin group and 8 (61.5%) patients in the Soave group had normal defecation and the difference was still significant (P < .05).
Patients with TCA managed with the Soave procedure had fewer operative complications compared with those of patients who underwent the Martin procedure. However, the patients managed with the Soave procedure took longer to establish normal defecation. Although each procedure has advantages and disadvantages, the Soave procedure is promising for the treatment of TCA, especially with regard to postoperative complications. A long-term follow-up study is needed.
全结肠无神经节细胞症(TCA)是一种相对罕见且严重的疾病,由小儿外科医生进行治疗。有几种手术方法可用于治疗 TCA。然而,目前对于哪种手术方法更有效还没有共识。本文旨在比较 Soave 手术和 Martin 手术治疗 TCA 的疗效,分析其在术前数据和术后结果方面的差异。
在 2001 年 1 月至 2008 年 6 月期间,我们招募了 29 例 TCA 患者,分别接受了 Martin 手术(14 例)或 Soave 手术(15 例)治疗。从医院图书馆的病历中收集了相关数据。通过电话或直接在诊所进行问卷调查的方式对患者进行了随访研究。在诊所中,详细记录了年龄、手术前体重、术中输血、吻合口漏、术后肠炎、任何术后发热持续时间、住院时间以及一系列功能结果等数据,以便比较 Soave 手术和 Martin 手术的效果。
TCA 患者以男性为主,男女比例约为 6.25:1。Soave 组患者的造口回纳时间、术前体重和手术年龄均小于 Martin 组(P<0.05)。在接受 Martin 手术的患者中,术后发热时间和住院时间明显长于 Soave 组(P<0.05)。Soave 组术中输血(46.7%)、腹部伤口感染(6.67%)和吻合口漏(0)的发生率明显低于 Martin 组(92.8%、28.5%和 14.3%)(P<0.05)。在随访研究中,Martin 组有 7 例发生肠炎,其中 4 例发生严重肠炎,导致多次住院。相比之下,Soave 组只有 2 例发生肠炎,1 例在诊所进行肛门扩张治疗后得到缓解。术后 4 周内,所有患者排便次数增加,肛周区域有粪便污染。术后 3 个月,Martin 组有更多的患者正常排便,与 Soave 组相比差异有统计学意义(P<0.05)。6 个月后,Martin 组有 11 例(78.6%)和 Soave 组有 8 例(61.5%)患者正常排便,差异仍有统计学意义(P<0.05)。
与接受 Martin 手术的患者相比,接受 Soave 手术治疗的 TCA 患者手术并发症更少。然而,接受 Soave 手术的患者需要更长时间才能恢复正常排便。尽管每种手术都有其优缺点,但 Soave 手术治疗 TCA 具有很大的潜力,尤其是在术后并发症方面。需要进行长期的随访研究。