Rigas A, Karamanolakis D, Bogdanos I, Stefanidis A, Androulakakis P A
Department of Paediatric Urology, Aghia Sophia Children's Hospital, Athens, Greece.
BJU Int. 2003 Jul;92(1):101-3. doi: 10.1046/j.1464-410x.2003.04265.x.
To present the characteristic clinical and imaging findings of pelvi-ureteric junction (PUJ) obstruction caused by crossing renal vessels (CRV), as it presents particular features within the spectrum of congenital hydronephrosis.
Between April 1982 and December 2000, 384 children underwent surgery for PUJ obstruction. In 71 (18.5%; mean age 8.5 years, range 2 months to 14 years; 49 aged> 5 years), the obstruction was caused by CRV. The data collected from the medical records of these patients were analysed for their clinical presentation and imaging findings, i.e. ultrasonography (US), intravenous urography (IVU) and diuretic renography.
The main presenting symptom was recurrent renal colic (pain, nausea, vomiting) in 59%, followed by urinary infection (UTI) in 20%, gross haematuria in 11% and an incidental diagnosis in 10%. By contrast, in the 313 children with intrinsic PUJ obstruction, renal colic was present in only 10.5%. Moreover, from 1991 to 2000, when the use of prenatal US became widespread, hydronephrosis was detected prenatally in 42 of 212 children (20%) with intrinsic PUJ obstruction, but in only two of 31 (6%) with obstruction by CRV. However, in 10 children with CRV operated on during this period, prenatal US had shown mild hydronephrosis (< 15 mm), which during the follow-up decreased until the children became symptomatic after 5-9 years (eight renal colic, two UTI). US during acute symptoms showed significant hydronephrosis (> 25 mm), and colour Doppler US of two patients directly showed the CRV. In all 71 children with CRV obstruction diuretic IVU and renography during the acute symptoms had an obstructive pattern, and in 24 renal colic was reproduced during the examination. The differential kidney function was < 40% in 11 children who presented with UTI; two required nephrectomy and in the remaining 69 an Anderson-Hynes pyeloplasty, after which none had an episode of renal colic or UTI during a mean (range) follow-up of 10.2 (2-20) years.
PUJ obstruction by CRV should be suspected in older children presenting with recurrent renal colic and hydronephrosis. Good kidney function is expected in most of these children, despite their age, because the vascular obstruction is intermittent. Mild prenatal hydronephrosis that could decrease postnatally does not exclude the possibility of vascular obstruction, which may later become symptomatic. Imaging (US, diuretic IVU and renography) during an episode of pain is essential and colour Doppler US could help to establish the diagnosis in these cases. Knowing that a child has a CRV is important for choosing an open surgical approach rather than endoscopic pyelotomy, to avoid potential complications
阐述由交叉肾血管(CRV)导致的肾盂输尿管连接处(PUJ)梗阻的特征性临床及影像学表现,因为其在先天性肾积水范围内具有独特特征。
1982年4月至2000年12月期间,384例儿童因PUJ梗阻接受手术。其中71例(18.5%;平均年龄8.5岁,范围2个月至14岁;49例年龄>5岁)梗阻由CRV引起。分析从这些患者病历中收集的数据,以了解其临床表现及影像学表现,即超声检查(US)、静脉肾盂造影(IVU)和利尿肾图。
主要表现症状为反复肾绞痛(疼痛、恶心、呕吐)的占59%,其次是泌尿系统感染(UTI)占20%,肉眼血尿占11%,偶然诊断出的占10%。相比之下,在313例原发性PUJ梗阻的儿童中,肾绞痛仅占10.5%。此外,从1991年到2000年,随着产前超声的广泛应用,在212例原发性PUJ梗阻儿童中有42例(20%)产前检测到肾积水,但在31例CRV梗阻儿童中仅2例(6%)。然而,在此期间接受手术的10例CRV梗阻儿童中,产前超声显示轻度肾积水(<15mm),随访期间积水逐渐减轻,直到5 - 9年后儿童出现症状(8例肾绞痛,2例UTI)。急性症状期超声显示明显肾积水(>25mm),2例患者的彩色多普勒超声直接显示了CRV。在所有71例CRV梗阻儿童中,急性症状期利尿IVU和肾图呈梗阻型,24例在检查期间再现肾绞痛。出现UTI的11例儿童患侧肾功能<40%;2例需要肾切除,其余69例行安德森 - 海恩斯肾盂成形术,术后平均(范围)10.2(2 - 20)年随访期间均未出现肾绞痛或UTI发作。
对于出现反复肾绞痛和肾积水的大龄儿童,应怀疑CRV导致的PUJ梗阻。尽管这些儿童年龄较大,但由于血管梗阻是间歇性的,大多数患儿肾功能良好。产前轻度肾积水出生后可能减轻,但不排除血管梗阻的可能性,后期可能出现症状。疼痛发作时进行影像学检查(US、利尿IVU和肾图)至关重要,彩色多普勒超声有助于这些病例的诊断。了解患儿存在CRV对于选择开放手术方式而非内镜肾盂切开术很重要,以避免潜在并发症。