Non-surgical management of multicystic dysplastic kidney

Department of Urology, University of California, Davis School of Medicine, Sacramento, CA 95817, USA.
BJU International (Impact Factor: 3.53). 04/2008; 101(7):804-8. DOI: 10.1111/j.1464-410X.2007.07328.x
Source: PubMed


To better define the outcome and association of multicystic dysplastic kidney (MCDK) with hypertension, vesico-ureteric reflux (VUR), infection and cancer, as there is no consensus on the management of patients born with MCDK. The risk of cancer has dictated the surgical management of the disease in the past.
The Medline database was searched for articles published between 1965 and 2006 and written in the English language, and containing the keywords 'multicystic dysplastic kidney'.
The inclusion criteria were met by 105 reports that were subsequently analysed. Of MCDK, 60% regress or involute within 3 years. About 25% of patients will have VUR into the contralateral kidney, of which 90% is grade <or=3. The risk of urinary tract infection appears to be associated with VUR or coexistent abnormalities rather than the MCDK. The risk of hypertension is no greater than that in the general population and nephrectomy is usually not curative. The overall risk of Wilms' tumour developing in a MCDK is <1 in 2000. All reported Wilms' tumours were identified before 4 years of age and 70% presented as a palpable mass.
Published reports support the non-surgical management of MCDK. Common practice has been to remove palpable or growing MCDKs, although these represent a very small fraction of MCDKs. In theory, ultrasonographic surveillance until 4 years old might allow the earlier detection of a Wilms' tumour, and decrease the intensity of chemotherapy and improve prognosis. Previous reports do not prove or disprove this concept, and the appropriate frequency of surveillance is not evident.

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    ABSTRACT: In children, unilateral multicystic dysplastic kidney (MCDK) is one of the most frequently identified urinary tract abnormalities. A variety of proposed etiologies has been associated with the underlying pathogenesis of MCDK. These include genetic disturbances, teratogens, in utero infections, and urinary outflow tract obstruction. From 5-43% of the time, MCDK has associated genito-urinary anomalies, both structural and functional in nature. A review of the literature reveals that involution rates are reported to be 19-73%, compensatory hypertrophy of the contralateral kidney occurs from 24-81% of the time, and estimated glomerular filtration rates (GFRs) (by the Schwartz formula) range from 86-122 ml/min per 1.73 m(2) body surface area. Most authors suggest serial ultrasonography to monitor contralateral growth, routine blood pressure monitoring, and a serum creatinine monitoring algorithm. The risk of hypertension in those with MCDKs does not appear to be greater than that of the general population, and the rates of malignant transformation of MCDK are small, if at all increased, in comparison with those in the general population. If the patient develops a urinary tract infection or has abnormalities of the contralateral kidney, shown on ultrasound, a voiding cystourethrogram is recommended. Finally, the body of literature does not support the routine surgical removal of MCDKs.
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    ABSTRACT: We retrospectively studied our cases of Multicystic Dysplastic Kidney (MCDK). The review was aimed at identifying the pattern of the disease in Indian Scenario and the required management thereof. We studied the clinical, radiological and nuclear scan findings of 22 patients with unilateral MCDK. They were diagnosed and/or treated in our unit from 1999 to 2007. The diagnosis was achieved by Ultrasound and further confirmed by DMSA scans. Other ancillary investigations like Micturating cystourethrogram were done if indicated. These patients were followed and followup investigations consisted of renal ultrasound, blood pressure measurement, and urinalysis and blood biochemistry A total of 22 patients (18 boys and 4 girls) with unilateral MCDK were investigated and followed for a mean period of 41 months. MCDK was detected on antenatal ultrasound only in 12(55%) and postnatally in 10(45%) babies. Mean age for postnatal diagnosis was 20 months. Follow up ultrasound revealed complete involution of MCDK in 3 patients and partial regression in 11 patients. The size of dysplastic kidney was unchanged in 4 patients and a further 4 patients underwent nephrectomy. Indications of nephrectomy were parental anxiety in 2, hypertension in 1 and palpable mass in 1. Large proportion (45%) of patients in presented series are diagnosed post natally contrary to western world where more than 80% are diagnosed antenatally. Uncomplicated isolated MCDK carry good prognosis with nephrectomy required in only a few patients. Association with other urological anomalies in ipsilateral/contralateral genitourinary tract is important to identify as they have worse outcome in terms of ultimate renal function. All patients with simple/complex unilateral MCDK should be advised long term follow up for the possible development of hypertension and/or hyper infiltration injury.
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