Siebert Joseph R, Smith Kenneth J, Cox Liza L, Glass Ian A, Cox Timothy C
1 Department of Laboratories, Seattle Children's Hospital, Seattle, WA, USA.
Pediatr Dev Pathol. 2013 Nov-Dec;16(6):405-14. doi: 10.2350/13-08-1359-OA.1. Epub 2013 Aug 26.
Prenatal obstruction of the lower urinary tract may result in megacystis, with subsequent development of hydroureter, hydronephrosis, and renal damage. Oligo- or anhydramnios, pulmonary hypoplasia, and prune belly syndrome are lethal consequences. Causes and mechanisms responsible for obstruction remain unclear but might be clarified by anatomic study at autopsy. To this end, we employed 2 methods of tomographic imaging-optical projection tomography and contrast-enhanced microCT scanning-to elucidate the anatomy of the intact urinary bladder and urethra in 10 male fetuses with lower urinary tract obstruction. Images were compared with those from 9 age-matched controls. Three-dimensional images, rotated and sectioned digitally in multiple planes, permitted thorough examination while preserving specimens for later study. Both external and internal features of the bladder and urethra were demonstrated; small structures (ie, urethral crest, verumontanum, prostatic utricle, ejaculatory ducts) were seen in detail. Types of obstruction consisted of urethral atresia (n = 5), severe urethral stenosis (n = 2), urethral diaphragm (n = 2), or physical kinking (n = 1); classic (Young type I) posterior urethral valves were not encountered. Traditional light microscopy was then used to verify tomographic findings. The prostate gland was hypoplastic or absent in all cases; in 1, prostatic tissue was displaced inferior to the verumontanum. Findings support previous views that dissection may produce valve-like artifacts (eg, bisection of an obstructing diaphragm) and that deformation of an otherwise normal urethra may result in megacystis. The designation "posterior urethral valves" should not be used as a generic expression of urethral obstruction unless actual valves are demonstrated.
下尿路产前梗阻可能导致巨膀胱,随后发展为输尿管积水、肾盂积水和肾损害。羊水过少或无羊水、肺发育不全和梅干腹综合征是致命后果。梗阻的原因和机制尚不清楚,但尸检时的解剖学研究可能会阐明。为此,我们采用了两种断层成像方法——光学投影断层扫描和对比增强显微CT扫描——来阐明10例患有下尿路梗阻的男性胎儿完整膀胱和尿道的解剖结构。将图像与9例年龄匹配的对照组的图像进行比较。三维图像在多个平面上进行数字旋转和切片,以便在保存标本以供后续研究的同时进行全面检查。膀胱和尿道的外部和内部特征均得以显示;小结构(即尿道嵴、精阜、前列腺囊、射精管)也能清晰看到。梗阻类型包括尿道闭锁(n = 5)、严重尿道狭窄(n = 2)、尿道瓣膜(n = 2)或物理扭结(n = 1);未发现典型的(杨氏I型)后尿道瓣膜。然后使用传统光学显微镜来验证断层扫描结果。所有病例中前列腺均发育不全或缺失;其中1例前列腺组织移位至精阜下方。这些发现支持了之前的观点,即解剖可能会产生瓣膜样假象(如梗阻性隔膜的二等分),并且原本正常的尿道变形可能导致巨膀胱。除非实际发现瓣膜,否则“后尿道瓣膜”这一名称不应作为尿道梗阻的通用表述。