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我对先天性巨结肠症的早期经历。

My early experience with Hirschsprung's disease.

作者信息

Swenson O

出版信息

J Pediatr Surg. 1989 Aug;24(8):839-44; discussion 844-5. doi: 10.1016/s0022-3468(89)80549-4.

Abstract

Prior to 1948, when the first distal colonic resection with preservation of continence was performed for Hirschsprung's disease, there was no clear understanding of the etiology of the disease. Early explanations for the cause of the disease included mechanical obstruction, "neurologic imbalance," and congenital malformation of the entire colon. No medical or surgical treatment had proved successful in treating the disease. It is generally assumed today that the absence of ganglion cells was widely known and accepted as the cause of Hirschsprung's disease prior to 1948 and that this led to the concept of distal colon dysfunction and formed the basis for the distal colonic resection. As a matter of fact, it played no role whatsoever in devising the successful operation. The rare absence of ganglion cells was widely held to be produced by the massive distention and chronic stasis in the megacolon and was considered of no pathologic significance. This report reviews the early work in developing the concept of a distal physiologic obstruction that led to a successful surgical treatment.

摘要

1948年首例保留控便功能的远端结肠切除术用于治疗先天性巨结肠之前,人们对该病的病因尚无清晰认识。对该病病因的早期解释包括机械性梗阻、“神经失衡”以及整个结肠的先天性畸形。尚无任何医学或手术治疗方法被证明对该病有效。如今人们普遍认为,1948年之前,神经节细胞缺失作为先天性巨结肠的病因已广为人知并被接受,这进而引出了远端结肠功能障碍的概念,并为远端结肠切除术奠定了基础。事实上,在设计这一成功手术时,神经节细胞缺失并未起到任何作用。人们普遍认为,巨结肠中大量扩张和慢性淤滞导致神经节细胞罕见缺失,且认为这没有病理意义。本报告回顾了早期在形成远端生理性梗阻概念方面的工作,该概念促成了成功的手术治疗。

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