Controversies concerning diagnostic guidelines for Hirschsprung's disease and allied disorders

Abstract

Objective To study the agreement and disagreement in definition, standard or method of diagnosis of Hirschsprung's disease (HD) and Allied disorders of Hirschsprung disease (HAD). Methods Recorded the as discussed at the workshop of the International Summit Forum of Hirschsprung's disease and its Allied Diseases, Wuhan, China. Results AchE stain of rectal suction biopsy has been recognized as the gold standard for preoperative diagnosis of HD. Adequate full-thickness intraoperative biopsy was considered to be necessary during surgery of IDs, but the choice of histologic staining method was not uniform. As a traditional dyeing, the reliability of HE has been questioned, it is generally believed that it is necessary to carry out other dyeing to be diagnosed at the same time, and the specific method is different from each other. At present, there is a lack of data on intestinal development of normal children in large samples. Therefore, there is an argument about the diagnostic criteria of Hypoganglionosis and Intestinal Neuronal Dysplasia. Whether IND is a disease entity, is the primary or secondary disease are also the hot spots of controversy. Pathological findings of various types of non ganglion lesions are not yet know how to define and classify. Therefore, we need do a more detailed description for all that undefined pathological variation. Conclusions There are some consensus about definition, standard or method of diagnosis of HD and Allied disorders of Hirschsprung disease (HAD) domestic and overseas, while part of hot debates which are lack of correct summary of experiences and theoretical data support have no unified conclusion, and these hot spots will become the new direction of the future research. Key words: Congenital megacolon; Intestinal neuronal dysplasia; Diagnosis