Hirschsprung's Disease.
Hirschsprung's Disease.
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DOI：
10.1007/s11938-003-0006-9
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发表时间：
2003-06-01
期刊：
影响因子：
--
通讯作者：
Belknap, William M.
中科院分区：
文献类型：
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作者：
Belknap, William M.
先天性巨结肠（HSCR）是肠神经系统最常见的先天性畸形，需要早期诊断和手术修复以获得最佳的综合结果。这种疾病的早期诊断允许使用原发性直肠内拉通术 (PERPT)，目前这是 HSCR 的最终手术治疗方法。 PERPT已成为HSCR的首选治疗方法，最近的医学文献中描述了大量成功治疗的患者。术后并发症的发生率通常与两阶段手术修复后的并发症发生率相似，但 PERPT 患者患先天性巨结肠相关小肠结肠炎的风险可能稍高。尽管最近在 HSCR 治疗方面取得了外科进展，但多达三分之一的患者可能仍然需要进行涉及临时改道结肠造口术的两阶段手术修复。分阶段修复的候选者包括那些患有长节段或全结肠疾病的 HSCR 患者，或者诊断延迟导致近端结肠明显扩张或患者临床不稳定的患者。肛门内括约肌张力过高，无论是作为孤立的原发性肛门失弛缓症还是术后并发症，都可以通过肉毒毒素注射或肛门肌切除术成功治疗。对术后长期存在肠功能异常的手术修复患者的结肠动力进行测量，可以识别出几种不同的动力障碍，这些障碍适合单独和个体化的治疗。 HSCR 管理中最重要的一个因素仍然是记录外科医生的临床判断，他利用所有可辨别的临床数据来选择特定患者的手术修复方式。
Hirschsprung's disease (HSCR) is the most common congenital malformation of the enteric nervous system and requires early diagnosis and surgical repair for the best comprehensive outcome. The early diagnosis of this disorder permits the use of primary endorectal pull-through (PERPT), which is now the definitive surgical therapy for HSCR. PERPT has become the preferred method of treatment for HSCR, and large numbers of successfully treated patients have been described in the recent medical literature. The rate of postoperative complications is generally similar to that following a two-stage surgical repair, but PERPT patients may be at a slightly higher risk for Hirschsprung's-associated enterocolitis. Despite recent surgical advances in the treatment of HSCR, a two-stage surgical repair involving a temporary diverting colostomy may still be necessary in up to one third of patients. Candidates for a staged repair include those HSCR patients with long-segment or total colonic disease or when there has been a delay in diagnosis that results in a markedly dilated proximal colon or patient clinical instability. Internal anal sphincter hypertonicity, occurring either as isolated primary anal achalasia or as a postoperative complication, can be successfully managed by either botulinum toxin injections or anal myectomy. The measurement of colonic motility in surgically repaired patients with a long-standing postoperative abnormality of bowel function can identify several distinct motility disorders that are amenable to separate and individualized therapies. The single most important element in the management of HSCR remains the clinical judgement of the surgeon of record, who utilizes all discernible clinical data to elect the manner of surgical repair in a given patient.