Hutson J M, McNamara J, Gibb S, Shin Y M
Department of General Surgery and General Paediatrics, Royal Children's Hospital and, Murdoch Children's Research Institute, Parkville, Victoria, Australia.
J Paediatr Child Health. 2001 Oct;37(5):426-30. doi: 10.1046/j.1440-1754.2001.00692.x.
Patients with chronic constipation that fails to respond to treatment remain a challenge for paediatricians and surgeons. Ongoing work in our institution suggests that a number of children with intractable symptoms have slow transit constipation, which has only been described recently in paediatrics. Common features of slow transit are: delayed passage of the first meconium stool beyond 24 h of age, symptoms of severe constipation within a year, or treatment-resistant 'encopresis' at 2-3 years, soft stools despite infrequent bowel actions, and delay in colonic transit on a transit study. A proportion of children with slow transit constipation have an abnormality of intestinal innervation associated with the dysfunctional colonic motility, recognized as intestinal neuronal dysplasia (IND). Intestinal neuronal dysplasia type B, the most common variant of IND, is defined on rectal biopsy by hyperplasia of the submucosal plexus. On laparoscopic colon muscle biopsy, many specimens show reduced numbers of excitatory substance P-immunoreactive nerve fibres in the circular muscle. Functional markers of the nerves allow new diagnostic criteria to be developed which may also allow a more rational approach to treatment. The aetiology remains obscure and the optimal management poorly defined, although subtotal colectomy, proximal colostomy or appendicostomy (for antegrade enemas) have been tried. Once the anatomy and physiology of the colon in children with slow colonic transit is better understood, we will have defined not only a new form of constipation, but also will be able to consider new therapies.
对治疗无反应的慢性便秘患儿仍然是儿科医生和外科医生面临的挑战。我们机构正在进行的研究表明，许多有顽固性症状的儿童患有慢传输型便秘，这在儿科领域直到最近才被描述。慢传输型便秘的常见特征包括：出生后24小时后首次胎粪排出延迟、一岁内出现严重便秘症状、2至3岁时出现难治性“遗粪症”、尽管排便次数少但大便柔软、以及在传输研究中结肠传输延迟。一部分慢传输型便秘儿童存在与结肠动力功能障碍相关的肠道神经支配异常，即肠道神经元发育异常（IND）。B型肠道神经元发育异常是IND最常见的类型，通过直肠活检发现黏膜下神经丛增生来定义。在腹腔镜结肠肌肉活检中，许多标本显示环行肌中兴奋性P物质免疫反应性神经纤维数量减少。神经的功能标志物有助于制定新的诊断标准，这也可能使治疗方法更加合理。病因仍然不明，最佳治疗方案也不明确，尽管已经尝试了次全结肠切除术、近端结肠造口术或阑尾造口术（用于顺行灌肠）。一旦更好地了解了结肠传输缓慢儿童的结肠解剖和生理，我们不仅将定义一种新的便秘形式，还将能够考虑新的治疗方法。
