Renal Dysplasia: A 6 Year Retrospective Study with Clinicopathological Correlation

  • Clement Wilfred Devadass MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
  • Madhu Aramane Basavaraj MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
  • Prasanna Shetty Badila MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
  • Mangala Gouri MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
Keywords: 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.

Author Biographies

Clement Wilfred Devadass, MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
MD(Patho), DNB ( Patho), MNAMS, Associate professor, Dep’t of Pathology
Madhu Aramane Basavaraj, MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
Final year Postgraduate student (MD), Department of Pathology
Prasanna Shetty Badila, MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
Senior Professor and Head of the Department, Dpt of Pathology
Mangala Gouri, MS Ramaiah Medical College and Hospitals, MSRIT Post, MSR Nagar, Bangalore (India)
Professor and Laboratory Director, Department of Pathology

References

1. Sing S, Gupta R, Nigam S, Khurana N, Aggarwal SK, Chaturvedi KU, et al. Clinic-pathological profile of 22 cases of cystic renal dysplasia. Indian J Pathol Microbiol. 2007;50:6-10.
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.
Published
"20-07-2018"
Section
Original Article