Patel Chetan D, Chawla Madhavi, Nadig Murali R, Mahapatra Ashok K, Bal Chandrasekhar
Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.
Hell J Nucl Med. 2007 May-Aug;10(2):102-4.
Meningomyelocele (MMC) is the most common form of neural crest closure defect and may be associated with dysfunction and malformations of multiple organs including urinary tract. It affects as many as 2-4 in 1000 live births, however there are no epidemiological studies available from India. Anatomical and neurogenic abnormalities of the urinary tract are known to occur in these patients. Scanty literature is available regarding the utility of radionuclide procedures in the evaluation of urinary tract in patients of MMC. The aim of this study was to evaluate urinary tract dysfunction and malformations in patients operated of MMC by diuretic renal dynamic scintigraphy (RDS) and direct radionuclide cystography (DRCG). In this retrospective analysis, 140 operated patients for MMC, mean age: 4.46, range: 1-18 years; 83 male and 57 female, referred to our department for evaluation of the upper renal tract, were studied. Neurogenic bladder (NB) was associated in 69 patients. The diagnosis of NB was based on clinical features like incontinence, expressibility of urine bladder and on urodynamic studies. RDS was performed after the intravenous administration of 10.36 MBq/kg of technetium-99m diethylene triamine penta-acetate or 99mTc-L, L-ethylenedicysteine. All patients with hydronephrosis (HDN) or hydroureteronephrosis (HDUN) on RDS underwent DRCG using 11.1-18.5 MBq of 99mTc-sulphur colloid. Our results showed that: out of the 140 patients, 78 (56%) including 33 with NB, had normal renal scintigraphy. Six patients (4%) had congenital renal anomalies. Twenty patients (14%) revealed non-obstructive HDN and had no vesico-ureteric reflux (VUR). Thirty-six patients (26%) with bilateral HDUN also had NB of which 19 (53%) had VUR. Fourteen (74%) of these patients with VUR had impaired renal function. In discussion, patients with MMC may present with disordered innervation of the detrussor muscle and external sphincter, which adversely affects the bladder function and potentially threatens the upper urinary tract. Patients of MMC with or without NB can be evaluated with RDS for congenital anomalies and initial assessment of renal function. Patients with NB and particularly those with HDUN can be evaluated with DRCG for the presence of VUR to prevent renal scarring and subsequent impairment of renal function. Long-term surveillance of the urinary tract is important in the management of these patients and this can be accomplished with RDS and DRCG. In conclusion, in our patients with MMC, the tests of RDS, and DRCG could differentiate those with normal or our abnormal renal anatomy and function while the major group of patients had bilateral HDUN and NB, and/or VUR or impaired renal function.
脊髓脊膜膨出（MMC）是最常见的神经嵴闭合缺陷形式，可能与包括泌尿系统在内的多个器官的功能障碍和畸形有关。每1000例活产中就有2 - 4例受其影响，然而印度尚无相关流行病学研究。已知这些患者会出现泌尿系统的解剖和神经源性异常。关于放射性核素检查在MMC患者泌尿系统评估中的应用，相关文献较少。本研究的目的是通过利尿肾动态闪烁显像（RDS）和直接放射性核素膀胱造影（DRCG）评估接受MMC手术患者的泌尿系统功能障碍和畸形。在这项回顾性分析中，研究了140例接受MMC手术的患者，平均年龄4.46岁，范围为1 - 18岁；其中男性83例，女性57例，他们因上尿路评估转诊至我院。69例患者伴有神经源性膀胱（NB）。NB的诊断基于诸如尿失禁、膀胱可挤压性等临床特征以及尿动力学研究。静脉注射10.36 MBq/kg的锝 - 99m二乙三胺五醋酸或99mTc - L，L - 乙二巯基丁二酸后进行RDS检查。所有在RDS检查中发现肾积水（HDN）或输尿管肾积水（HDUN）的患者均使用11.1 - 18.5 MBq的99mTc - 硫胶体进行DRCG检查。我们的结果显示：在140例患者中，78例（56%）包括33例伴有NB的患者，肾闪烁显像正常。6例患者（4%）有先天性肾脏异常。20例患者（14%）显示为非梗阻性HDN且无膀胱输尿管反流（VUR）。36例双侧HDUN患者也伴有NB，其中19例（53%）有VUR。这些有VUR的患者中有14例（74%）肾功能受损。在讨论中，MMC患者可能存在逼尿肌和外括约肌的神经支配紊乱，这会对膀胱功能产生不利影响，并可能威胁上尿路。有或无NB的MMC患者均可通过RDS评估先天性异常和肾功能的初步情况。伴有NB尤其是伴有HDUN的患者可通过DRCG评估是否存在VUR，以预防肾瘢痕形成及随后的肾功能损害。对这些患者进行泌尿系统的长期监测在其管理中很重要，这可通过RDS和DRCG来实现。总之，在我们的MMC患者中，RDS和DRCG检查能够区分肾脏解剖和功能正常或异常的患者，而主要患者群体存在双侧HDUN和NB，和/或VUR或肾功能受损。
