Barnes P R, Lennard-Jones J E, Hawley P R, Todd I P
Gut. 1986 May;27(5):534-41. doi: 10.1136/gut.27.5.534.
The distinction between Hirschsprung's disease and idiopathic megacolon in childhood dates from the classic clinical, radiological, and histological studies of Bodian, Stephens, and Ward. This article describes clinical experience over 15 years of 94 patients in whom megacolon of these two types was recognised for the first time after the age of 10, to illustrate the problems of diagnosis and treatment in later years. Just as it is now recognised that patients with the clinical characteristics of Hirschsprung's disease may have one of several abnormalities of the myenteric plexus, including not only absence of ganglion cells, but also patchy or zonal loss, abnormal neurones or neuronal dysplasia, so idiopathic megacolon may also be a heterogeneous group of cases. This paper suggests on clinical grounds that those patients with idiopathic megacolon whose symptoms start in childhood differ from those whose symptoms develop in later years.
先天性巨结肠与儿童特发性巨结肠的区别可追溯到博迪安、斯蒂芬斯和沃德的经典临床、放射学及组织学研究。本文描述了15年间94例患者的临床经验，这些患者均在10岁之后首次被诊断为这两种类型的巨结肠，以说明大龄患者的诊断和治疗问题。正如现在人们认识到，具有先天性巨结肠临床特征的患者可能存在肠肌丛的多种异常情况之一，不仅包括神经节细胞缺失，还包括散在或带状缺失、异常神经元或神经元发育异常，特发性巨结肠也可能是一组异质性病例。本文基于临床情况表明，儿童期起病的特发性巨结肠患者与成年后起病的患者有所不同。
