Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Multicystic Renal Dysplasia

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4854-1

Definition

A nonheritable developmental disease resulting of abnormal metanephric differentiation.

Clinical Features

  • Incidence

    It occurs in 1 in 4300 live births. Multicystic dysplastic kidneys are also a finding of genetic disorders (Sanna-Cherchi et al. 2007).

  • Age

    It is a congenital urinary tract anomaly (Bernstein 1971).

  • Sex

    Multicystic renal dysplasia affects predominantly males.

  • Site

    The condition is usually unilateral.

  • Treatment

    Although previously, nephrectomy was the treatment of choice to avoid potential complications such as infection, pain, hypertension, and malignancy, to date the management is primarily conservative.

  • Outcome

    Most patients have a favorable prognosis.

Multicystic renal dysplasia is usually sporadic but may rarely be associated with obstruction, multi-malformation syndromes, chromosomal anomalies, or hereditary malformation syndromes. Unilateral multicystic renal dysplasia is the most common cause of renal mass in childhood. Bilateral lesions may be present...

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References and Further Reading

  1. Bernstein, J. (1971). The morphogenesis of renal parenchymal maldevelopment (renal dysplasia). Pediatric Clinics of North America, 18, 395–407.CrossRefGoogle Scholar
  2. Sanna-Cherchi, S., Caridi, G., Weng, P. L., et al. (2007). Genetic approaches to human renal agenesis/hypoplasia and dysplasia. Pediatric Nephrology, 22, 1675–1684.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly