Shukla Aseem R, Bellah Richard A, Canning Douglas A, Carr Michael C, Snyder Howard M, Zderic Stephen A
Division of Pediatric Urology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399, USA.
J Urol. 2004 Nov;172(5 Pt 1):1977-9. doi: 10.1097/01.ju.0000140450.50242.50.
Congenital giant diverticulum of the bladder is a consequence of deficiency in the detrusor musculature and has been reported in male infants as a rare cause of bladder outlet obstruction.
A 10-year retrospective review revealed 4 patients (3 boys and 1 girl) with bladder outlet obstruction due to a giant bladder diverticulum. Prenatal and postnatal clinical and imaging records were reviewed.
Prenatal sonography was unremarkable in all patients. Three males (ages 4 months, 10 months and 3 years) had no medical history of voiding dysfunction, and 1 female (11 years) had the Ehlers-Danlos syndrome. While the girl presented with urinary tract infection, all patients presented with progressively decreasing urinary stream and urinary retention. Each patient underwent voiding cystourethrography (VCUG) and ultrasound. In each patient VCUG showed a giant (greater than 7 cm) bladder diverticulum that descended below the bladder neck and compressed the urethra during voiding. Vesicoureteral reflux was seen in 2 patients. Ultrasonography demonstrated moderate unilateral hydronephrosis in 2 patients. Bladder diverticulectomy was successfully performed in all patients, with ureteral reimplantation in 3.
A giant congenital bladder diverticulum, when noted on VCUG to descend below the bladder neck, may lead to bladder outlet obstruction. To our knowledge we report the first case of a female presenting with bladder outlet obstruction due to a giant bladder diverticulum. Children with connective tissue disorders may be predisposed to this disorder, which must be excluded, regardless of gender, in all patients presenting with voiding abnormalities. Surgical diverticulectomy, often with ureteral reimplantation, is the preferred treatment, with excellent long-term results.
先天性巨大膀胱憩室是逼尿肌肌肉组织发育缺陷的结果,在男婴中作为膀胱出口梗阻的罕见原因已有报道。
一项为期10年的回顾性研究发现4例因巨大膀胱憩室导致膀胱出口梗阻的患者(3例男孩和1例女孩)。对产前和产后的临床及影像记录进行了回顾。
所有患者产前超声检查均无异常。3例男性患者(年龄分别为4个月、10个月和3岁)无排尿功能障碍病史,1例女性患者(11岁)患有埃勒斯-当洛综合征。该女孩表现为尿路感染,所有患者均表现为尿流逐渐减少和尿潴留。每位患者均接受了排尿性膀胱尿道造影(VCUG)和超声检查。每位患者的VCUG均显示一个巨大(大于7厘米)的膀胱憩室,其在排尿时降至膀胱颈以下并压迫尿道。2例患者出现膀胱输尿管反流。超声检查显示2例患者有中度单侧肾积水。所有患者均成功进行了膀胱憩室切除术,其中3例进行了输尿管再植术。
当在VCUG上发现巨大先天性膀胱憩室降至膀胱颈以下时,可能导致膀胱出口梗阻。据我们所知,我们报告了首例因巨大膀胱憩室导致膀胱出口梗阻的女性病例。结缔组织疾病患儿可能易患此病,在所有出现排尿异常的患者中,无论性别如何,都必须排除这种疾病。手术切除憩室,通常同时进行输尿管再植术,是首选的治疗方法,长期效果良好。