Fondelli P, Martucciello G, Taccone A, Dodero P
Servizio di Radiologia, Istituto G. Gaslini, Genova-Quarto.
Radiol Med. 1989 Mar;77(3):207-10.
The radiological findings pathognomonic of Hirschsprung's disease have been known for quite a long time. However, in addition to those diseases characterized by rectum or rectum-sigmoid aganglionosis (as in the classic form of Hirschsprung's disease), in pediatric pathology of surgical interest severe affections exist characterized by aganglionosis extending from the rectum to the entire colon, and exceptionally as far as the small bowel (ultra-long Hirschsprung's disease). The authors report on the results they obtained in radiological studies of 6 cases of total colonic aganglionosis by means of the current radiological procedures, i.e. plain abdominal films and barium enema. As for total colonic aganglionosis, no diagnostic radiological parameters have been assessed yet. The authors compare their results with those taken from international literature: microcolon with reflux into mega-ileum (2 out of 6 cases) appears to be the most suggestive X-ray finding for total colonic aganglionosis. Finally, the role is discussed of radiological studies in the diagnostic approach to total colonic aganglionosis. The authors conclude that, even though radiological investigations are indispensable, they are often not conclusive. Therefore, an accurate evaluation of the proximal extension of aganglionosis can be made only at surgery.
先天性巨结肠的典型放射学表现已为人所知甚久。然而，除了那些以直肠或直肠乙状结肠无神经节症为特征的疾病（如先天性巨结肠的经典形式）外，在小儿外科病理学中，还存在一些严重病变，其特征是无神经节症从直肠延伸至整个结肠，极少数情况下甚至延伸至小肠（超长型先天性巨结肠）。作者报告了他们通过目前的放射学检查方法，即腹部平片和钡剂灌肠，对6例全结肠无神经节症进行放射学研究的结果。至于全结肠无神经节症，尚未评估出诊断性放射学参数。作者将他们的结果与国际文献中的结果进行了比较：结肠细小并伴有回肠扩张反流（6例中有2例）似乎是全结肠无神经节症最具提示性的X线表现。最后，讨论了放射学研究在全结肠无神经节症诊断方法中的作用。作者得出结论，尽管放射学检查不可或缺，但往往无法得出确定性结论。因此，只有在手术中才能准确评估无神经节症的近端延伸情况。
