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.     

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     

Multicystic kidney dysplasia

The clinical course of 48 children (27 boys and 21 girls) with multicystic kidney dysplasia was analysed retrospectively. The patients were seen and treated at the Children's Hospital of Medical School Hannover between 1976 to 1989. There was no familial occurrence of the disease, yet in eight families various other renal diseases could be observed. In 20 patients the diagnosis of multicystic renal dysplasia was presumed by prenatal sonographic examination, in the other patients the diagnosis was established at the age between 1 day to 12 years. The first manifestations were palpable abdominal mass (n = 16), urinary tract infection (n = 4), casually because of a sonographic evaluation for other reasons (n = 4) and vomiting (n = 2). Associated malformations were found in 18 patients: cardiac malformations (n = 6), dysplasia of the other kidney (n = 5), ureter obstruction of the other kidney (n = 3), horseshoe kidney (n = 1) and others (n = 3). There was no hypertension and serum creatinine levels were normal in those children, who did not suffer from associated malformations of the other kidney. The multicystic kidney was removed operatively in 42 patients at the age of 3 days to 9.5 years (median 4 weeks). The prognosis of multicystic kidney dysplasia depends on associated renal and other malformations.     

Multicystic dysplasia in the upper component of the complete duplex kidney

Four cases of neonatal multicystic dysplasia involving the upper part of a complete duplex kidney are presented. This malformation, suspected on antenatal ultrasound examination, was confirmed by postnatal IVP and ultrasound. This emphasizes the need to perform an IVP when the diagnosis of multicystic kidney is suggested by ultrasound. Conservative surgery may then be achieved, as in three of the four cases.     

Fatal hypertensive encephalopathy in a child with association of multicystic kidney and renal artery stenosis

At the age of 5 years, a boy with known multicystic dysplastic kidney disease showed signs of arterial hypertension with progress to fatal hypertensive encephalopathy. Arterial hypertension was refractory to antihypertensive therapy and the child lost consciousness. Computed tomography of the brain revealed multiple cerebral infarctions. Doppler ultrasound showed an elevation of blood flow in the main artery of the functioning kidney consistent with stenosis as a cause of hypertension. CONCLUSION: Arterial hypertension is a known complication of kidney disease. Multicystic dysplastic kidney and renal artery stenosis is a potentially fatal association. Careful evaluation and monitoring, with special emphasis on blood pressure, should be performed in children with multicystic dysplastic kidney disease.     

Are therapeutic stem cells justified in bilateral multicystic kidney disease? A review of literature with insights into the embryology

Aim was to describe the challenges faced in the management of bilateral multicystic kidney disease (MCKD). A case of antenatally detected bilateral polycystic disease was referred at 28 weeks of gestation. The patient was advised to continue pregnancy till term and be in regular follow-up. Postnatally, the male baby passed urine in normal stream and was diagnosed as bilateral multicystic kidney disease on ultrasonography. He developed symptoms of renal failure. The baby was operated with right pyeloplasty and left pyelostomy, as the left ureter was atretic. The histopathology was consistent with bilateral multicystic kidney disease. Postoperatively, the baby was stable with intermittent episodes of metabolic acidosis that were managed medically and with peritoneal dialysis. Autologous stem cells were injected at the age of 1 year into the aorta at the level of the renal arteries clamping the aorta below. Repeat biopsy at time of stem cell injection showed 5/10 glomeruli showing global sclerosis on right side and 5/15 glomeruli showing global sclerosis on left side. The only improvement seen was in decreased doses of medicines to keep the child metabolically stable. The baby kept struggling but succumbed at the age of 17 months and 15 days. Post mortem bilateral renal biopsies demonstrated presence of primitive renal tubules and blastemal cells that were not demonstrated earlier. Survival for few months in bilateral multicystic kidney disease is thus possible with adequate treatment, the novel use of stem cells in these cases may prove beneficial in future though it is too early to comment further.     

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.     

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.     

Multicystic kidney dysplasia: a prospective study on the natural history of the affected and the contralateral kidney

In a 6-year period, 41 young infants with multicystic kidney dysplasia were seen in our department. In 30 cases, the diagnosis had already been suspected by prenatal ultrasonography. A prospective protocol was proposed to the parents which comprised ultrasound evaluation every 3 months until the age of 24 months and renal function assessment at the age of 18 months. In 33 patients, the study was completed as scheduled. At the start of the study, the maximal diameter of the multicystic kidney was above the mean length of normal kidneys in all cases where precise measurement was possible. Within 24 months, 7 of the dysplastic kidneys disappeared, 20 regressed in size, 1 remained unchanged and only 5 increased in size. Between the age of 0 to 3 months, renal length of the contralateral kidney was within the normal range in 19 infants and above +2SD in 14 cases. At the age of 18 to 24 months, renal length was, with few exceptions, between 0 and +4SD. Inulin clearance was normal in all 33 individuals with a median value of 112 ml/min per 1.73 m². Conclusion As a rule, multicystic kidneys shrink in the first 2 years of life. In most cases the contralateral kidney maintains a normal renal function as a consequence of progressive compensatory hypertrophy. Received: 19 November 1997 / Accepted in revised form: 31 January 1998     

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.     

Cystic dysplasia of the testis associated with multicystic dysplastic kidney

Cystic dysplasia of the testis (CDT) is a rare congenital defect characterized by the formation of multiple irregular cystic spaces in the mediastinum testis. Co-existent genitourinary lesions have commonly been associated with this lesion and have included absence of the ipsilateral kidney, duplication anomalies, and cryptorchidism. We describe the first case in which multicystic dysplastic kidney (MCDK) is associated with CDT.     

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.     

Intrauterine diagnosis of renal anomalies by ultrasonography.

The diagnosis of renal disease was made antenatally in three patients by ultrasonographic examination. In two cases a multicystic kidney was detected in utero and removed shortly after delivery. In another case, a hydronephrotic kidney was detected in utero; repair was then performed early, before symptoms developed in the infant. Antenatal diagnosis of renal disease by ultrasonography may help reduce morbidity and mortality in some cases.     

Collecting duct carcinoma arising from multicystic dysplastic kidney disease

Malignancy arising from a multicystic dysplastic kidney (MCDK) is rare. Most reports are of Wilms' tumor and clear-cell renal cell carcinoma arising from a previously unrecognized MCDK. To our knowledge, no reports have described collecting duct carcinoma arising from MCDK.     

Benign multilocular cystic nephroma

This article reports a case of cystic nephroma to bring awareness about the benign nature of this condition. The patient presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the superolateral portion of the left kidney with thin septa without solid parts. Histology confirmed the diagnosis of cystic nephroma.     

Integrated imaging of neonatal renal masses

Thirty-three neonatal renal masses were evaluated during a 2-year interval. The final diagnoses in these 33 patients were hydronephrosis [14], multicystic dysplastic kidney [10], renal vein thrombosis [3], obstructed upper pole duplication [2], polycystic kidney disease [2], nephroblastomatosis [1], and mesoblastic nephroma [1]. We recommend an integrated imaging approach that utilizes sonography to clarify anatomy and renal scintigraphy or excretory urography to determine renal function.     

Multicystic renal dysplasia. Spontaneous remission of a hypertrophic form

A case of spontaneous regression of the tumor type of multicystic dysplastic kidney (MDK) is reported. This case is consistent with recent data on the natural history of MDK. The classical therapeutic approach which involves surgery is discussed in the context of this case and of others with a similar course.     

Anorectal malformation associated with a perineal protrusion of the rectal mucosa: case presentation

We present a rare case of a female neonate with an imperforate anus associated with a perineal mass which may correspond to an extrophied rectal duplication. Associated anomalies were thoracic hemivertebrae and a multicystic dysplastic kidney. Excision of the perineal lesion followed by anal transplantation and perineal reconstruction corrected the anomaly.