Martínez-Almoyna C, Monereo J
An Esp Pediatr. 1976 Nov-Dec;9(6):574-83.
35 cases of megarrectum associated with lumbo-sacro-coccygeal dysplasis are reviewed. 32.7% of the dysplasia were sacral. Nine cases were ultrashort aganglionic segments. Symptoms were variable, of early onset (82.7% before one year) and mild (57.1% required medical advise after three). Medical management was useful in the non aganglionic group whereas it was ineffective in the aganglionic group. Sphincteric myotomy was the more common operation used. Physiopathology and modalities of treatment are discussed.
回顾了35例与腰骶尾发育异常相关的巨直肠病例。32.7%的发育异常发生在骶骨。9例为超短无神经节段。症状多样,起病早(82.7%在1岁前)且症状较轻(57.1%在3岁后需要就医建议)。非无神经节组采用内科治疗有效,而无神经节组则无效。括约肌切开术是最常用的手术方式。本文讨论了其病理生理学和治疗方式。