Renal Dysplasia: A 6 Year Retrospective Study with Clinicopathological Correlation
DOI:
https://doi.org/10.21276/apalm.1880Keywords:
Multicystic dysplasia, renal dysplasia, urinary tract anomalies.Abstract
Bakground: Renal dysplasia (RD) is rare congenital disease resulting from anomalous development of metanephric elements. The objective of the study was to evaluate the clinicopathologic features of RD with emphasis on the morphological changes.
Methods: A retrospective study of RD cases diagnosed on nephrectomy specimens over a duration of six years at a south Indian tertiary health care centre. The clinical, radiological and macroscopic features were retrieved and microscopic morphological features were reviewed.
Results: A total of 13 cases of RD were obtained, all of which were unilateral, with age range of 20 weeks of gestation to 54 years. Male predominance (61%) was noted and left kidney was preponderantly involved (69%). Associated genito-urinary tract anomalies were present in 54% of cases. Multicystic, hypodysplastic, hydronephrotic and segmental macroscopic types of RD were present in 39%, 23%, 23% and 15% of the cases respectively. The most consistent microscopic feature was primitive ducts surrounded by fibromuscular collarets.
Conclusion: A diagnosis of RD should prompt the search of other associated urinary tract anomalies. Histopathologic examination is required for the definite diagnosis of RD and differentiation from other conditions like polycystic kidney disease, renal hypoplasia, renal atrophy and pure hydronephrosis. This differentiation is important from prognostic and genetic counselling point of view.
References
2. Jain S, Liapis H. Dysplasia/Hypoplasia/Agenesis. Chapter 6. In: Colvin RB, editor. Diagnostic Pathology. Kidney diseases. 1st ed. Canada: Amirys Publishing Inc; 2011.p. 6-11.
3. Rukmangadha N, Chowhan AK, Patnayak R, Radhika K, Phaneendra BV, Lakshmi AY, et al. Multicystic dysplastic kidney- a retrospective study with clinicopathological correlation. J Clin Sci Res. 2013;2:88-93.
4. Onal B, Kogan BA. Natural History of Patients With Multicystic Dysplastic Kidney- What Follow up is Needed? The Journal of Urology. 2006;176:1607-11.
5. Woolf AS, Price KL, Scambler PJ, Winyard PJD. Evolving Concepts in Human Renal Dysplasia. JSAN. 2004;15:998-1007.
6. Liaps H, Winyard PJD. Cystic Diseases and Developmental Kidney Defects. Chapter 4. In: Jennette JC, Olson JL, Silva FG, D’Agati VD, editors. Hepinstall’s Pathology of the Kidney,7th ed. Lippincort Williams & Wilkins, Philadelphia.2015;1:p.119-171.
7. Bonsib SM. Non-neoplastic diseases of the Kidney. In: Bostwick DG, Cheng L, editors. Urologic Surgical Pathology. 2nd ed. Mosby Elsevier; 2008.p. 11-29.
8. Lin CC, Tsai JD, Sheu JC, Lu HJ, Chang BPH. Segmental multicystic dysplastic kidney in children: clinical presentation, imaging finding, management, and outcome. Journal of Pediatric Surgery.2010;45:1856-62.
9. Han JH, Lee YS, Kim MJ, Lee MJ, Im YJ, Kim SW, et al. Conservative Management of Segmental Multicystic Dysplastic Kidney in Children. Urology.2015;86:1013-18.
10. Singh JK, Kanojia RP, Narasimhan KL. Multicystic dysplastic kidney in children- a need for conservative and long term approach. Indian J Pediatr. 2009;78:809-12.
11. Schreuder MF, Westland R, van Wilk JA. Unilateral multicystic dysplastic kidney: a meta-analysis of observational studies on the incidence associated urinary tract malformations and the contralateral kidney. Nephrol Dial Transplant. 2009;24:1810-8.
12. Chen RY, Chang H. Renal Dysplasia. Arch Pathol Lab Med. 2015;139:547-51.
13. Lim FF, Tsao TF, Chang HM, Sheu JN. Multicystic Dysplastic Kidney Disease Presenting With a Single Large Cyst in a Fetus- Anatomical Basis and Radiological Aspects. Pediatrics and Neonatology. 2011;52:227-31.
14. Aytac B, Sehitoglu I, Vuruskan H. Multicystic Dysplastic Kidney: Four-Year Evaluation. Turkish Journal of Pathology. 2011; 27:210-4.
15. Carmack AJK, Castellan M, Brayfield MP, Gosalbez R. Segmental multicystic dysplasia and ureteropelvic junction obstruction in a nonduplicated kidney. Journal of Pediatric Surgery. 2006;41: E1-E3.
16. Psooy K. Multicystic dysplastic kidney in the neonate: the role of the urologist. Can Urol Assoc J. 2010;4:95-97.
17. Nishio H, Kojima Y, Mizuno K, Kamisawa H, Kohri K, Hayashi Y. Laparoscopic Nephrectomy for Pelvic Multicystic Dysplastic Kidney.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2018 Clement Wilfred Devadass, Madhu Aramane Basavaraj, Prasanna Shetty Badila, Mangala Gouri
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access at http://opcit.eprints.org/oacitation-biblio.html).
