Diagnosis of aortic coarctation by tardus-parvus renal artery Doppler signal in an infant with multicystic dysplastic kidney: a case report

We report an infant with known unilateral multicystic dysplastic kidney (MCDK) who underwent renal ultrasonography and Doppler spectral waveform analysis for investigation of hypertension. A tardus-parvus waveform was demonstrated in the renal artery on the normal side suggesting either renal artery or more proximal stenosis. Coarctation of the aorta was subsequently demonstrated. Electronic supplementary material Supplementary material is available in the online version of this article at and is accessible to authorized users. An erratum to this article can be found at     

Ambulatory blood pressure monitoring in children with unilateral multicystic dysplastic kidney

Multicystic dysplastic kidney (MCDK) is one of the most common congenital renal anomalies. Arterial hypertension is a potential complication of MCDK. Blood pressure (BP) has so far been measured only casually and the frequency of hypertension has been estimated to be between 0%–8%. Ambulatory blood pressure monitoring (ABPM) provides more precise information on BP than the casual BP measurement. The aim of this study was to investigate the BP profile in children with MCDK using ABPM. A group of 25 children (16 girls), with a mean age of 7.8 years (range 3.8–17.7 years) were investigated. ABPM was performed using the oscillometric SpaceLabs 90207 device. Hypertension was defined as mean systolic and/or diastolic BP during the day and/or in the night exceeding 95th percentile for ABPM. Five (20%) children showed hypertension, two of them had combined daytime and night-time hypertension and three had isolated nocturnal hypertension, although daytime BP was between the 90th–95th percentile in two of them. Children with ultrasonographical and/or laboratory signs of contralateral kidney abnormalities showed a higher incidence of hypertension than those without abnormalities (two of four versus 3 of 21). The mean night-time systolic and diastolic BP of children with MCDK was significantly higher than in healthy children (+0.50 and +0.54 SDS, respectively, P=0.012 and 0.03, respectively). Three of the hypertensive children were already nephrectomised. All five hypertensive children showed ultrasonographical and/or laboratory signs of contralateral kidney abnormalities. Hypertensive children had significantly higher microalbuminuria than normotensive children (6.9 ± 3.2 mg/mmol creatinine versus 1.8 ± 0.7, P=0.03). The nocturnal BP fall (dip) was attenuated in five children, only one of whom was hypertensive. Conclusion Arterial hypertension in children with multicystic dysplastic kidney is seen more often if based on ambulatory blood pressure monitoring than on casual blood pressure recordings. The main risk factor for developing hypertension is contralateral kidney damage. Ambulatory blood pressure monitoring should be performed in children with multicystic dysplastic kidney, especially in those with contralateral kidney abnormalities. Received: 20 July 1999 and in revised form: 27 November 1999, 24 March 2000, 3 May 2000 Accepted: 3 May 2000     

The dilemma of the multicystic dysplastic kidney

Multicystic dysplastic kidney is the most frequent cause of an abdominal mass in the neonate, but controversy continues as to the optimal management of these lesions, since little is known about their natural history. Experience with two complicated cases and a review of reports of retained multicystic dysplastic kidneys suggest that such lesions pose a significant risk to their hosts. Malignancy, reversible hypertension, pain, and mass effect have been associated with retained lesions. Infection is another potential hazard that is frequently cited but poorly documented in the literature. In light of the currently low morbidity and mortality associated with operation and anesthesia in the neonatal period, resection appears to be the treatment of choice for the neonate with a multicystic dysplastic kidney.     

Observations on the use of Doppler ultrasound in multicystic dysplastic kidney

Pulsed Doppler ultrasound was used to assess the renal artery in patients with multicystic kidney. In 7 out of 7 patients there was marked abnormality of the wave-form. Doppler ultrasound may be a useful complementary diagnostic method in patients suspected of having a multicystic dysplastic kidney.     

Segmental multicystic dysplastic kidney: a rare situation

Segmental multicystic dysplastic kidney is a rare subtype, found in only about 4% of children diagnosed with MCDK. To the best of our knowledge, we describe the 36th reported case of segmental multicystic kidney disease.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

小儿先天性发育不良肾的临床特点和治疗

目的 探讨小儿先天性发育不良肾的临床特点、诊断和治疗.方法 总结分析1988年至2009年我科收治的83例单侧先天发育不良肾的临床资料.所有患儿术前均行多普勒超声、静脉肾盂照影和99mTc-DTPA肾脏扫描.35例患儿行增强CT检查,39例行IVP后延迟CT检查三维重建.所有病例均行发育不良肾切除,肾切除的指征包括无功能发育不良肾脏合并临床症状55例,无临床症状的无功能多囊发育不良肾,随访不消退或增大27例,无功能发育不良肾合并肾输尿管积水1例.结果 各项术前检查中IVP后延迟CT三维重建技术阳性诊断率最高,达100%.术后病理均提示为发育不良肾脏.4例因高血压行发育不良肾切除的患儿,术后2例仍然有高血压,其他患儿术后临床症状消失,没有恶变者.结论 儿童先天发育不良肾脏发育不良程度不一,合并畸形不同,临床表现各异.IVP后延迟CT检查三维重建技术诊断发育不良肾脏阳性率高.合并临床症状的无功能发育不良肾、多囊发育不良肾长期随访不消退者可手术治疗,随访过程中有明显消退的多囊发育不良可定期观察,有功能的非多囊发育不良肾可以保留.近19%发育不良肾脏可合并对侧肾脏畸形,应密切监测对侧肾脏功能及血压.     

Outcome of antenatally detected cystic dysplastic kidney disease.

Forty four fetuses with multicystic dysplastic kidney (MCDK) disease recognised on antenatal ultrasound were studied prospectively. In nine aborted fetuses and in five who died in the neonatal period the MCDK disease was bilateral or there were associated lethal abnormalities or syndromes. All surviving infants had unilateral disease and in six (20%) there was significant reflux into the normal contralateral kidney. Since 1988 the management of unilateral MCDK disease has been conservative with no child developing sepsis, hypertension, or malignancy. Serial ultrasound examinations suggest that MCDK lesions involute with time and conservative rather than operative management is favoured.     

Outcome of antenatally detected cystic dysplastic kidney disease

Forty four fetuses with multicystic dysplastic kidney (MCDK) disease recognised on antenatal ultrasound were studied prospectively. In nine aborted fetuses and in five who died in the neonatal period the MCDK disease was bilateral or there were associated lethal abnormalities or syndromes. All surviving infants had unilateral disease and in six (20%) there was significant reflux into the normal contralateral kidney. Since 1988 the management of unilateral MCDK disease has been conservative with no child developing sepsis, hypertension, or malignancy. Serial ultrasound examinations suggest that MCDK lesions involute with time and conservative rather than operative management is favoured.     

儿童肾囊性疾病的诊治

Potter分型将儿童肾囊性疾病分为4型:常染色体隐性遗传性多囊性肾病、多囊性肾发育不良、常染色体显性遗传性多囊性肾病、梗阻性囊性发育不良肾.此外,单纯性肾囊肿、发生在肾肿瘤及其他伴囊性肾病的综合征也可引起肾脏呈囊性改变.这类病由于其发病机制和病理基础不同,临床诊断及治疗方案选择亦不同,要正确诊断这类疾病,需要仔细分类并查明病因.该文就儿童常见的肾囊性疾病进行综述.     

Renovascular hypertension in infant presenting with cardiogenic shock

A 35-day-old infant admitted with cardiogenic shock was hypertensive shortly after initial resuscitation therapy. The hypertension did not respond to increasing doses of sodium nitroprusside. An abnormal blood flow in the right renal artery was detected by pulsed Doppler ultrasonography, and a prompt blood pressure response to oral captopril suggested a diagnosis of renovascular disease. Renal angiography showed right main renal artery stenosis, abnormal intrarenal arteries, and nonfunctional right kidney; the patient subsequently underwent right nephrectomy with good effect. Myocardial dysfunction resolved 2 months after control of the blood pressure.     

Arterial hypertension caused by extrinsic compression of the renal artery of tumor origin in a child

Stenosis of the renal artery secondary to an extrinsic acquired compression of the renal artery because of a tumor is rare. We report two cases. The first case is a boy of 14 months with a large neurogenic tumor from the right renal plexus with compression of the right renal artery. The second case is a girl of 2 years which had a neuroblastoma displacing the right kidney. The right renal vessels were invaded by very calcified solid masses and the removal was very difficult. Arterial hypertension secondary to an acquired unilateral renal stenosis may be healed definitely thanks to surgery.     

Single sysem ectopic ureters and ureteroceles associated with dysplastic kidney

Eight children forming an uncommon subgroup of renal obstructive dysplasia are presented. Each child had a nonfunctioning dysplastic kidney with a single collecting system with ectopic ureteral insertion and/or ureterocele. Five of the children had classic multicystic dysplastic kidneys, one had they hdyronephrotic type of multicystic dysplasia kidney had two had hypoplastic kidneys. Other significant medical problems in 5 of the 8 children (63%Z) included VACTERL association, congenital heart diseaise and other genitourinary malformations. Unlike some children with unilateral multicystic dysplasia kidney, this subgroup of children ahs an increased risk of infection. The must be correctly identified on imaging so that tailored clinical management decisions can be made and associated anomalies detected.Presented at the 1990 meeting o the RSNA     

Renal cysts associated with Turner's syndrome

After noting several occurrences of cystic kidney disease in Turner's syndrome we retrospectively reviewed the renal sonograms of 12 patients with this syndrome. Single renal cysts occurred in two patients and a multicystic dysplastic kidney in another. Our experience suggests that single renal cysts are a relatively frequent finding in Turner's syndrome and should be recognized within the scope of renal abnormalities associated with it.     

Kawasaki disease complicated by renal artery stenosis

We report the case of a child who developed severe renovascular hypertension six months after acute Kawasaki disease. The hypertension was well controlled with enalapril, but there was a gradual decrease in function of the affected kidney. The lesion, an ostial stenosis of the right main renal artery, was not amenable to percutaneous balloon angioplasty, so was treated with bypass surgery. Vasculitis is an important cause of renovascular hypertension in children. This case highlights the importance of regular blood pressure monitoring in children with a history of systemic vasculitis.     

Kawasaki disease complicated by renal artery stenosis.

We report the case of a child who developed severe renovascular hypertension six months after acute Kawasaki disease. The hypertension was well controlled with enalapril, but there was a gradual decrease in function of the affected kidney. The lesion, an ostial stenosis of the right main renal artery, was not amenable to percutaneous balloon angioplasty, so was treated with bypass surgery. Vasculitis is an important cause of renovascular hypertension in children. This case highlights the importance of regular blood pressure monitoring in children with a history of systemic vasculitis.     

The “septation sign” in multicystic dysplastic kidney

The case report is presented of a neonate with a unilateral multicystic dysplastic kidney. An intravenous pyelogram revealed septations throughout this kidney with late pooling of contrast media within the cystic structures. The pathological data stresses the presence of normal appearing glomeruli interspersed between the dysplastic cystic parenchyma. The possible etiologies for the septation sign and pudding phenomenon are discussed.     

Bilateral congenital midureteric strictures associated with multicystic dysplastic kidney and hydronephrosis: evaluation with MR urography

We report a case of bilateral congenital midureteric strictures diagnosed using MR urography. The severity of obstruction differed in the two ureters, resulting in a multicystic dysplastic kidney (MCDK) with an atretic ureter on one side and hydronephrosis that worsened over time due to progressive stenosis on the other. Although midureteric strictures are usually misdiagnosed as ureteropelvic junction (UPJ) or ureterovesical junction (UVJ) obstruction on conventional imaging, MR urography was able to clearly demonstrate both the anatomical and functional abnormalities. Additionally, because of the excellent anatomical resolution, similarities in the underlying pathological lesions could be contrasted with the severity of the pathophysiological impact upon each kidney.     

Case Report of a 22-Week Fetus with 47,XXX Karyotype and Multiple Lower Mesodermal Defects

A 22-week stillborn fetus with 47,XXX karyotype had lower mesodermal defects consisting of irregular fusion of the sacral vertebrae, anal agenesis, multicystic dysplasia of a horseshoe kidney, a single umbilical artery, dysplastic ovaries, and uterine hypoplasia. This case provides additional evidence for an association between trisomy X and genitourinary defects including lower mesodermal defects sequence. Received November 21, 1997; accepted April 2, 1998.