Menon Prema, Rao Katragadda L N, Sodhi Kushaljit S, Bhattacharya A, Saxena Akshay K, Mittal Bhagwant R
Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
J Pediatr Urol. 2015 Apr;11(2):80.e1-6. doi: 10.1016/j.jpurol.2014.10.014. Epub 2015 Mar 4.
Pediatric ureteropelvic junction obstruction (UPJO) due to an extrinsic crossing vessel (CV) is rare and often remains undiagnosed preoperatively. Vascular hitch procedures are often performed as associated intrinsic obstruction is not expected. We compared data and intravenous urography (IVU) findings of patients with aberrant CV versus those with intrinsic UPJO, all undergoing open dismembered pyeloplasty.
Is accurate pre-operative diagnosis of aberrant CV causing extrinsic UPJO possible?
To assess differences in the demographic, clinical, radiological, intra-operative features and postoperative improvement after pyeloplasty between patients with a CV and those with only intrinsic UPJO.
Prospective study of all children below 12 years with UPJO presenting to a tertiary referral centre and who underwent open Anderson - Hynes dismembered pyeloplasty between 2003 and 2013 was conducted. Pre-operative investigations included serial ultrasonography, renal dynamic [ethylene di-cysteine (EC)] scan and IVU. These were repeated 3 months after pyeloplasty. Pre-operative IVUs of children with CV were compared with the IVUs of an equal number of similar aged children, randomly selected from the intrinsic obstruction group.
Pyeloplasty was performed in 643 children during the study period. Data of 33 children with aberrant CVs (mean age 6.99 years) were compared with the remaining 610 children (mean age 3.27 years) with only intrinsic obstruction. Highly significant associations of those with CV included age above 2 years, female gender, associated anomalies, abdominal pain in those above 2 years and poor preoperative function on IVU. Specific IVU features which were statistically highly significant in favor of presence of CV were small, intrarenal and globular flat bottomed pelvis. (Figure) Calyceal dilatation was also more prominent in the CV group. A funnel shaped, extrarenal pelvis was highly significant in favor of intrinsic obstruction. There was associated intrinsic obstruction in addition to CV obstruction in 8 children. All children symptomatically improved after pyeloplasty and did well on long term follow up. The majority showed improvement or stabilization of function on EC scan.
With the advent of antenatal ultrasonography, most children with UPJO are detected early. Children with CV tend to present later. This is often detected during surgery. Color Doppler is useful but is operator dependant and not performed routinely. In this study, IVU showed the presence of obstruction and loss of function unlike color Doppler, but also revealed specific diagnostic features not previously reported in literature. This can help in accurate preoperative prediction and avoid endopyelotomy, or a dorsal lumbotomy/retroperitoneal approach. Renal function in CVs is expected to be good as the obstruction is thought to be intermittent. However, we noted delayed contrast uptake on IVU in 60.6% and differential renal function on EC scan below 40% in 17 patients (56.6%). These indicate the effect of the obstruction on the renal parenchyma and the importance of early detection. Higher association with other anomalies and higher incidence in females has also not been emphasized in the literature so far. We noted associated intrinsic obstruction in 24.24% patients which is highly significant. This category of patients is likely to be missed and inappropriately treated if a "vascular hitch procedure" is performed. None of our patients had postoperative complications.
Characteristic features were seen on IVU helping in preoperative diagnosis which can be extrapolated to magnetic resonance urography. There is a higher association of CV in age above 2 years, females, associated congenital anomalies, delayed uptake on IVU and differential renal function below 40% compared to intrinsic obstruction. Associated intrinsic obstruction in 24% with no postoperative complications indicates the superiority of dismembered pyeloplasty over vasculopexy procedures.
由外在交叉血管(CV)导致的小儿肾盂输尿管连接部梗阻(UPJO)较为罕见,术前常难以诊断。由于预计不存在相关的内在梗阻,因此常施行血管固定术。我们比较了患有异常CV的患者与患有内在UPJO的患者的数据及静脉肾盂造影(IVU)结果,所有患者均接受开放性离断性肾盂成形术。
对于由异常CV导致外在UPJO能否进行准确的术前诊断?
评估患有CV的患者与仅患有内在UPJO的患者在人口统计学、临床、放射学、术中特征以及肾盂成形术后的改善情况方面的差异。
对一家三级转诊中心收治的所有12岁以下患有UPJO且在2003年至2013年间接受开放性安德森-海因斯离断性肾盂成形术的儿童进行前瞻性研究。术前检查包括系列超声检查、肾脏动态[二巯基丁二酸(EC)]扫描和IVU。肾盂成形术后3个月重复这些检查。将患有CV的儿童的术前IVU与从内在梗阻组中随机选取的同等数量的同龄儿童的IVU进行比较。
在研究期间,643名儿童接受了肾盂成形术。将33名患有异常CV的儿童(平均年龄6.99岁)的数据与其余610名仅患有内在梗阻的儿童(平均年龄3.27岁)的数据进行比较。患有CV的儿童与年龄大于2岁、女性、相关畸形、2岁以上儿童的腹痛以及IVU显示的术前功能较差高度相关。在统计学上高度支持CV存在的特定IVU特征为小的、肾内的和球形平底肾盂。(图)CV组的肾盏扩张也更明显。漏斗形、肾外肾盂高度支持内在梗阻。8名儿童除了CV梗阻外还存在相关的内在梗阻。所有儿童在肾盂成形术后症状均有改善,长期随访情况良好。大多数儿童在EC扫描中显示功能改善或稳定。
随着产前超声检查的出现,大多数患有UPJO的儿童得以早期发现。患有CV的儿童往往就诊较晚。这通常在手术中被发现。彩色多普勒超声有用,但依赖操作者且并非常规进行。在本研究中,IVU显示出梗阻的存在和功能丧失,这与彩色多普勒超声不同,但也揭示了文献中此前未报道的特定诊断特征。这有助于进行准确的术前预测,避免内镜肾盂切开术或背侧腰椎切开术/腹膜后入路。由于认为CV导致的梗阻是间歇性的,因此预计其肾功能良好。然而,我们注意到60.6%的患者在IVU上有造影剂摄取延迟,17名患者(56.6%)在EC扫描中显示分肾功能低于40%。这些表明梗阻对肾实质的影响以及早期检测的重要性。文献中迄今也未强调与其他畸形的更高相关性以及女性中的更高发病率。我们注意到24.24%的患者存在相关的内在梗阻,这具有高度显著性。如果施行“血管固定术”,这类患者很可能被漏诊并接受不恰当的治疗。我们的患者均未出现术后并发症。
IVU上可见特征性表现,有助于术前诊断,这可外推至磁共振尿路造影。与内在梗阻相比,CV在年龄大于2岁、女性、相关先天性畸形、IVU造影剂摄取延迟以及分肾功能低于40%的患者中更为常见。24%的患者存在相关的内在梗阻且无术后并发症,这表明离断性肾盂成形术优于血管固定术。