Unilateral multicystic dysplastic kidney: long term outcomes

Aims

To report the long term follow up of children with antenatally detected unilateral multicystic dysplastic kidney (MCDK) with documentation of complications, involution rate with time, and renal function at 10 years.

Methods

Data were retrieved from a prospective regional registry of patients with MCDK between 1985 and 2004. Children were followed using a common protocol of investigation with follow up ultrasound scans (USS) at 2 (165 patients), 5 (117 patients), and 10 years (43 patients).

Results

Serial USS showed that 33% of the MCDK kidneys had completely involuted at 2 years of age, 47% at 5 years, and 59% at 10 years. No patients developed hypertension, significant proteinuria, or malignancy, but two developed pelviureteric junction obstruction in the contralateral kidney. Twenty seven of 143 children (19%) had vesicoureteric reflux (VUR) (96% mild to moderate VUR) into the contralateral kidney with no difference in the incidence of urinary tract infections or renal scarring between those with or without VUR. The mean estimated glomerular filtration rate (GFR) was 86.4 ml/min/1.73 m² (range 48–125) in 31 of 43 patients followed to 10 years.

Conclusions

Conservative management of unilateral MCDK is justified with clinical review and infrequent USS but longer term follow up continues in the 41% still with renal remnants at 10 years and those with impaired GFR. It is suggested that the initial micturating cystogram is deferred unless abnormal USS features are present in the contralateral kidney or ureter.     

Unilateral multicystic dysplastic kidney: long term outcomes.

AIMS: To report the long term follow up of children with antenatally detected unilateral multicystic dysplastic kidney (MCDK) with documentation of complications, involution rate with time, and renal function at 10 years. METHODS: Data were retrieved from a prospective regional registry of patients with MCDK between 1985 and 2004. Children were followed using a common protocol of investigation with follow up ultrasound scans (USS) at 2 (165 patients), 5 (117 patients), and 10 years (43 patients). RESULTS: Serial USS showed that 33% of the MCDK kidneys had completely involuted at 2 years of age, 47% at 5 years, and 59% at 10 years. No patients developed hypertension, significant proteinuria, or malignancy, but two developed pelviureteric junction obstruction in the contralateral kidney. Twenty seven of 143 children (19%) had vesicoureteric reflux (VUR) (96% mild to moderate VUR) into the contralateral kidney with no difference in the incidence of urinary tract infections or renal scarring between those with or without VUR. The mean estimated glomerular filtration rate (GFR) was 86.4 ml/min/1.73 m2 (range 48-125) in 31 of 43 patients followed to 10 years. CONCLUSIONS: Conservative management of unilateral MCDK is justified with clinical review and infrequent USS but longer term follow up continues in the 41% still with renal remnants at 10 years and those with impaired GFR. It is suggested that the initial micturating cystogram is deferred unless abnormal USS features are present in the contralateral kidney or ureter.     

Unilateral multicystic dysplastic kidney: the case for nephrectomy

Management of unilateral multicystic dysplastic kidneys (MCDK) presents physicians and surgeons with a significant dilemma. Recent studies have indicated that the incidence of short term complications of MCDK is low and many authors have recommended conservative non-operative treatment. Surgery has been proposed by some because of the potential complications of hypertension, infection, and malignant change. Three children with hypertension secondary to MCDK seen at this institution in the past four years, one of whom had been discharged from follow up as a result of 'disappearance' of the cystic kidney on ultrasound examination, are reported. We believe that the risks of hypertension secondary to MCDK have been understated, and that based on the conclusions of these studies, many children may be receiving suboptimal follow up. We currently favour elective nephrectomy as the treatment of choice for this lesion.     

Unilateral multicystic dysplastic kidney: the case for nephrectomy

Accepted 28 August 1996Management of unilateral multicystic dysplastic kidneys (MCDK) presents physicians and surgeons with a significant dilemma. Recent studies have indicated that the incidence of short term complications of MCDK is low and many authors have recommended conservative non-operative treatment. Surgery has been proposed by some because of the potential complications of hypertension, infection, and malignant change. Three children with hypertension secondary to MCDK seen at this institution in the past four years, one of whom had been discharged from follow up as a result of `disappearance'' of the cystic kidney on ultrasound examination, are reported. We believe that the risks of hypertension secondary to MCDK have been understated, and that based on the conclusions of these studies, many children may be receiving suboptimal follow up. We currently favour elective nephrectomy as the treatment of choice for this lesion.     

Unilateral multicystic dysplastic kidney and contralateral megacalycosis. An unusual association

Dilation of the genitourinary tract is not always synonymous with obstruction. It is well known that malformations with apparent hydronephrosis and normal urinary flow can exist. In addition, two or more distinct malformations of the genitourinary tract can coexist in the same patient. We present a case of coexistence of a unilateral multicystic dysplastic kidney and contralateral megacalycosis associated with ipsilateral distal segmental megaureter. This association is unusual in the literature. Glomerular renal function was normal, with only a slight defect of renal concentration capacity.     

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.     

Abnormal contralateral kidney in unilateral multicystic dysplastic kidney disease

We performed a retrospective study of infants with unilateral multicystic dysplastic kidney (MCDK) in order to investigate the associated urological abnormalities. We examined the records of seven patients, in five of whom a diagnosis had been made prenatally using ultrasonography. Our investigation focused on the associated urological abnormalities, particularly on the contralateral side, and the results of voiding cystourethrography (VCUG). Four of the seven patients (57%) had urological abnormalities other than MCDK: three exhibited vesicoureteral reflux (VUR) of the contralateral side including one patient who also had an ipsilateral ectopic ureter, and the fourth patient had a ureterocele of the ipsilateral side. The results indicate that contralateral VUR, was the most common abnormality associated with MCDK. Two infants had high-grade VUR and underwent anti-reflux surgery soon after the diagnosis. Both have remained free of recurrent urinary tract infection.     

Unilateral multicystic dysplastic kidney disease: defining the natural history. Anglia Paediatric Nephrourology Group

Controversy exists as to what is the optimal management of antenatally detected multicystic dysplastic kidney disease (MCDK): conservative or operative. We report 70 (31M, 39F) children with unilateral MCDK in whom the initial micturating cystourethrography revealed vesicoureteric reflux into the contralateral kidney in 16/63 (25%). Involution of the MCDK kidney was noted at birth in 4 and occurred by 2 y of age in 11/46 (24%), with 16/46 (35%) showing a significant reduction in size. Of 21 children followed to 5 y, the MCDK had disappeared in 3 (14%) and 8 (38%) had shown a further reduction in size. No child has developed hypertension or proteinuria. Four children had urinary tract infections with lower tract symptoms, but no scarring of the normal hypertrophied kidney. We conclude that conservative management of unilateral MCDK is justified with long-term follow-up consisting of annual clinical review and ultrasound evaluation at 2, 5 and 10 y.     

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.     

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.     

Pediatric single-port-access nephrectomy for a multicystic,dysplastic kidney

Major urologic surgery via a single port has emerged as the latest progression in laparoscopy and robotics. While current literature highlights the single-port approach to the surgical treatment of cholecystitis, appendicitis and varicoceles, this technique has never been employed to perform a nephrectomy on a child. We herein report a case of a pediatric patient who underwent nephrectomy via single-port-access.     

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.