本文报告先天性巨结肠类缘病33例;肠神经元性发育异常症(NID)20例;神经节细胞减少症9例;神经元性发育不良症1例;神经节细胞未成熟症3例.对各类缘病的病理特征和临床特点作了描述,诊断需作大肠多处全层活检,治疗需完全切除病变肠段.%Reviewing the pathological and clinical records of 339 patients with the diagnosis of HD,the author found 33 cases Of Hirschsprung's disease (HD) allied disease.The patients were classified into four types according to their pathology:(1) neuronal intestinal dysplasia (NID) 20 cases;(2) hypoganglionosis 9 cases;(3) ganglion hypogenesis 1 case;(4) immaturity of ganglion cells 3 cases.It is very dificult to make a diagnosis of HD allied disease.The author suggests the following guide lines:(1) constipation is better or worse;(2) anorectal reflex is normal but constipation is resistant to dilatation treatment;(3) the constipation is remarkable while the narrow segment ts very short in x-ray;(4) segment of severe dilatation and hypertrophy is extrordinarily long;(5) constipation recurred after an uneventful operation.However,the confirmative diagnosis of HD allied disease is made by multiple biopsies,and the treatment ought to be the total resection of the diseased bowel.
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