Proesmans Willem
Department of Pediatrics Renal Unit, University of Leuven, Leuven, Belgium.
Pediatr Nephrol. 2008 Apr;23(4):537-40. doi: 10.1007/s00467-008-0768-3. Epub 2008 Feb 16.
Neurogenic bladder dysfunction in children is frequently seen in patients with meningomyelocele (MMC). The disorder carries a high risk for all kinds of complications, with renal damage being the most important. More than 95% of MMC patients have a neurogenic bladder, the paramount manifestation of which is a disturbed coordination between detrusor and sphincter muscles. This vesicourethral dysfunction leads to defective filling and emptying of the urinary bladder. Voiding at will is almost never possible. According to the location and extent of the neural tube lesion, patients have either an atonic or a hypertonic pelvic floor and either an atonic or a hypertonic detrusor, leading to four classic combinations. Hypertonic sphincter and detrusor hyperactivity lead to the most dangerous form of neurogenic bladder, referred to as the "unsafe" bladder. The presence of residual urine in a high-pressure container causes either decompensation of the detrusor with vesicoureteral reflux or deterioration of the bladder wall with hypertrophy and stiffness resulting in uterovesical obstruction. The subsequent insufficient drainage of the upper urinary tract leads to decompensation of the ureters and finally to chronic renal disease, the process being accelerated by urinary tract infections. The aim of treatment is to restore as much as possible both essential functions: urine storage and timely emptying of the reservoir. What should and can be achieved is a more or less adequate, low-pressure, functional capacity of the bladder that is emptied as completely as possible by clean intermittent catheterization (CIC). MMC leads to the prototype of neurogenic bladder in childhood. What we know and what we do for MMC patients can roughly be applied to all other forms of neurogenic bladder, either congenital or acquired.
小儿神经源性膀胱功能障碍常见于脊髓脊膜膨出(MMC)患者。该疾病引发各类并发症的风险很高,其中肾脏损害最为严重。超过95%的MMC患者存在神经源性膀胱,其最主要的表现是逼尿肌与括约肌之间的协调性紊乱。这种膀胱尿道功能障碍导致膀胱充盈和排空出现问题。几乎不可能自主排尿。根据神经管病变的位置和范围,患者要么盆底张力过低或过高,要么逼尿肌张力过低或过高,从而产生四种典型组合。括约肌张力过高和逼尿肌活动亢进会导致最危险的神经源性膀胱形式,即“不安全”膀胱。高压状态下膀胱内残余尿液的存在,要么导致逼尿肌失代偿并伴有膀胱输尿管反流,要么导致膀胱壁因肥厚和僵硬而恶化,进而造成膀胱颈梗阻。随后上尿路引流不畅会导致输尿管失代偿,最终引发慢性肾病,尿路感染会加速这一进程。治疗的目的是尽可能恢复两项基本功能:尿液储存和及时排空储存器。我们应该且能够实现的是让膀胱具备或多或少足够的、低压的功能容量,并通过清洁间歇性导尿(CIC)尽可能完全地排空膀胱。MMC导致了儿童期神经源性膀胱的典型病例。我们对MMC患者的了解及采取的措施大致可应用于所有其他先天性或后天性神经源性膀胱形式。