Schnaufer L
Surg Clin North Am. 1976 Apr;56(2):349-59. doi: 10.1016/s0039-6109(16)40881-9.
Hirschsprung's disease or aganglionic megacolon is an anomaly caused by the absence of ganglion cells in the myenteric plexus of the distal colon. It produces intestinal obstruction or lethal enterocolitis in the neonatal period and constipation of varying degree in the older child. The diagnosis can be made by history alone and confirmed by physical findings, barium enema, motility studies, and rectal biopsy. Colostomy may be a life saving measure in the newborn, to be followed by a definitive pull-through procedure before the age of 1 year. Operative correction consists of various techniques all of which aim for excision of the aganglionic segment with preservation of the internal anal sphincter. The major pitfall of these procedures is a too perfectly preserved sphincter which remains spastic and still produces obstruction. Some damage to this muscle must be accomplished either during the procedure or postoperatively by bouginage in order to obtain a satisfactory result.
先天性巨结肠症或无神经节细胞性巨结肠是一种由远端结肠肌间神经丛中神经节细胞缺失引起的异常疾病。它在新生儿期会导致肠梗阻或致命性小肠结肠炎，在大龄儿童中则会引起不同程度的便秘。仅凭病史即可做出诊断，并通过体格检查、钡剂灌肠、动力研究和直肠活检来确诊。结肠造口术可能是新生儿的一项挽救生命的措施，随后在1岁前进行确定性的拖出手术。手术矫正包括各种技术，所有这些技术的目的都是切除无神经节段并保留肛门内括约肌。这些手术的主要缺陷是括约肌保留得过于完好，仍然痉挛并仍会导致梗阻。为了获得满意的结果，必须在手术过程中或术后通过探条扩张对该肌肉造成一些损伤。
