Alexacos L, Skouteli H, Sofatzis J, Nacopoulou L
Z Kinderchir. 1985 Feb;40(1):58-9. doi: 10.1055/s-2008-1059714.
A fatal case of the megacystis-microcolon syndrome in a newborn girl is presented and two previously unreported findings are discussed. The umbilical hernia is thought to represent a mechanically induced embryological defect, secondary to the expanding nonfunctional megacystis which appears to be the dominant component from an early stage. Hypertrophic changes of the large intestinal wall musculature, adjacent to dilated segments, may indicate an impaired propulsive activity in utero, as a result of defective neurotransmission. The latter is proposed as the common pathophysiological mechanism for both urinary and intestinal malfunction.
本文报告了一例新生儿女孩的致死性巨膀胱-小结肠综合征病例，并讨论了两个此前未报告的发现。脐疝被认为是一种机械性诱导的胚胎学缺陷，继发于不断扩张的无功能巨膀胱，而巨膀胱似乎从早期就是主要成分。与扩张段相邻的大肠壁肌肉组织的肥厚性改变，可能表明由于神经传递缺陷，子宫内推进活动受损。后者被认为是泌尿和肠道功能障碍的共同病理生理机制。
