International Urology and Nephrology 10 (2), pp. 89--92 (1978)
Bilateral Renal Dysplasia (Case Report)* P. AHUJA, K. K. KAPOOR, T. SINGH, R. R. DAROGA
Department of Pathology, Maulana Azad Medical College, New Delhi, India (Received June 1, 1977)
Bilateral renal dysplasia is a very rare condition, unlike unilateral renal dysplasia. We came across three cases. In one of them there were other multiple congenital anomalies. In all the cases the ureters and renal blood vessels were rudimentary. The condition is incompatible with life. The different theories explaining this congenital anomaly and the incidence of the disease are being discussed. Cystic disease o f the kidney has aroused interest a m o n g all branches o f medicine and a lot of controversy has arisen a b o u t the nomenclature of other similar renal conditions, n o w satisfactorily settled by Parkkulainen et al. [1]. Multicystic kidney was first described by Schwartz in 1963 (cit. [1, 2]), and went under several different names during the years. In our review of the literature, we have come across many cases of unilateral renal dysplasia and rare cases of bilateral renal dysplasia. In the autopsy studies over a p e r i o d of 5 years from 1971 to 1976, at the D e p a r t m e n t of Pathology, M a u l a n a Azad Medical College, we came across 3 cases of bilateral renal dysplasia and 1 case of unilateral renal dysplasia, a m o n g neonatal cases and none a m o n g s t adults.
Case reports Case 1. P. M. (2/74). A three weeks postmature stillborn female child. Each kidney weighed 5 g and showed small cysts of varying sizes on the surface filled with clear fluid. The renal blood vessels and ureter were rudimentary. N o other congenital anomaly was seen. On the cut surface the whole kidney was found to be converted into several cysts of different sizes, filled with clear watery fluid. Microscopic examination showed several groups of tubular structures of varying sizes, lined by tall columnar to flattened epithelial cells. A m o n g some groups of tubules abhortive glomeruli were seen. Isolated and small groups of tubules were surrounded by thick mesenchymal tissue arranged concentrically. Areas of cartilage formation were also seen (Figs 1 - 3 ) . Case 2. P. M. (6/75). A male child, full-term breech delivery, who lived for * Paper presented at the 27th Annual Conference of the Indian Association of Pathologists and Microbiologists, December 19, 1976, Manipal, India. International Urology and Nephrology 10, 1978
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Ahuja et al.: Bilateral renal dysplasia
14 hours. The right kidney weighed 15 g, tee left kidney 10 g. Both showed multiple cysts, of varying sizes, on the outer and cut surface filled with clear fluid. The ureter and renal vessels were rudimentary. There was no ott,_er congenital anomaly. The microscopic appearance of tke kidneys was tke same as in Case 1. Case 3. P. M. (9/76). A stillborn full-term female ckild, delivered by m/ncavity forceps. There were multiple copgerital abnormalities. The ears were large and low-set. The eyes had a mongoloid slant. T~ere was bilateral cataract, hyper-
Fig. 1. Group of tubules lined by columnar epithelium surrounded by concentrically arranged mesenchymal fibres. H & E, • 66
Fig. 2. Group of abhortive glomeruli with primitive tubular structures. H & E, • International Urology and Nephrology 10, 1978
Ahuja et at.: Bilateral renal dysplasia
91
extension of hands and adduction of arms, bilateral talepes and interventricular septal defect. The kidneys weighed l0 g each and showed m a n y cysts of different sizes on their outer and cut surface. The microscopic findings were similar to the above two cases, except that there was no cartilage formation.
Fig. 3. Two areas of cartilage (arrow), with abhortive glomeruli, a group of tubules and two
blood vessels surrounded by concentrically arranged mesenchymal fibres. H & E, x 66 One case of unilateral renal dysplasia was also come across, in a full-term male stillborn child. The right kidney was affected and showed multiple cysts o f varying sizes filled with clear fluid. The left kidney was normal. The microscopic appearance of the affected kidney was similar to that of Case 3. There were no other congenital anomalies. Incidence
The word renal dysplasia is used synonymously with multicystic kidney in several reports by different workers. Several cases of unilateral renal dysplasia are reported, as compared with bilateral renal dysplasia. The sex incidence as reported in renal dysplasia has no significance, and there is no special preponderance of any sex. Parkkulainen et ah [ 1] reported on 7 females and 12 males; Staubitz et al. [5] on 4 females and 3 males; Pathak et al. [3] on 2 females and 10 males, and in our series we found 2 males and 2 females. Unilateral cases can present at any age and are compatible with life. Bilateral renal dysplasia is usually incompatible with life. Discussion
Schwartz was the first to describe in 1936 unilateral multicystic kidney disease. Baggentosis in 1951 suggested the term dysplasia to disclose the presence 2
International Urology and Nephrology 10, 1978
92
Ahuja et al.: Bilateral renal dysplasia
of primitive renal element (cit. [1 ]) (primitive connective tissue with smooth muscle, primitive glomeruli, primitive tubules, capillary network, etc.). The term multicystic is preserved because it is descriptive of the macroscopic appearance of the deformity [1 ]. Spence [4] further differentiated this condition from polycystic kidney. It is now accepted that renal dysplasia is a disturbed differentiation of nephron tissue with persistence of structures inappropriate to the gestational age of the patient [2]. Many theories have been put forward to explain the etiopathogenesis of the cysts of the kidney, but none have been universally accepted and there is no agreement on the mechanism. The various theories are as follows: 1. Hildurbrandt (cit. [1-4]) thinks that failure of union of the nephrons arising from the nephrogenic blastema to unite with the collecting tubules of ureteric bud origin [3] results in this condition. This was the explanation given by him in 1894. 2. Ossthanonth and Potter in 1964 ascribed it to be due to premature cessation of branching by the ampullae of collecting tubules which are devoid of their capacity to induce the formation of nephrons from cysts (cit. [2]). 3. Heptinstall [2] reported in 1966 that (a) disorganized differentiation of metanephric tissue is an expression of the intrinsic potency of that tissue, deprived of the organizing influence of the ureteric bud, either because of the failure of the latter or some of its branches to develop, or because the capacity of the ampullae to induce nephrons has become somehow impaired by distal obstruction; and (b) the morphological consequence in the developing kidney, of obstruction itself, causes cystically dilated ampullae and cystic dilatation of the immature nephrons already induced. As can be seen, many attempts have been made to clarify this condition, but there is no satisfactory explanation as yet.
Acknowledgements We are grateful to the Dean and the Professor of Pathology, Maulana Azad Medical College, New Delhi, India, for permission to publish this report. We are also grateful to Mr. K. S. Jolly, for his help rendered by the photographs,
References 1. Parkkulainen, K. V., Itjelt, L., Sirola, K.: Congenital multicystic dysplasia of the kidney. Acta Chir. Scand., 244, Suppl. 1 (1959). 2. Heptinstall, R. N.: Pathology of the Kidney. First edition. J. A. Churchill, London 1966. 3. Pathak, I. G., William, D. L.: Multicystic and cystic dysplastic kidneys. Brit. J. Urol., 36, 318 (1964). 4. Spence, H. M.: Congenital unilateral multicystic kidney: an entity to be distinguished from polycystic kidney disease and other cystic disorders. J. Urol., 74, 693 (1955). 5. Staubitz, W. J., Jewett, T. C. Jr., Pletman, R. J. : Renal cystic disease in childhood. J. UroL, 90, 8 (1963). International Urology and Nephrology 10, 1978
Bilateral Renal Dysplasia (Case Report)* P. AHUJA, K. K. KAPOOR, T. SINGH, R. R. DAROGA
Department of Pathology, Maulana Azad Medical College, New Delhi, India (Received June 1, 1977)
Bilateral renal dysplasia is a very rare condition, unlike unilateral renal dysplasia. We came across three cases. In one of them there were other multiple congenital anomalies. In all the cases the ureters and renal blood vessels were rudimentary. The condition is incompatible with life. The different theories explaining this congenital anomaly and the incidence of the disease are being discussed. Cystic disease o f the kidney has aroused interest a m o n g all branches o f medicine and a lot of controversy has arisen a b o u t the nomenclature of other similar renal conditions, n o w satisfactorily settled by Parkkulainen et al. [1]. Multicystic kidney was first described by Schwartz in 1963 (cit. [1, 2]), and went under several different names during the years. In our review of the literature, we have come across many cases of unilateral renal dysplasia and rare cases of bilateral renal dysplasia. In the autopsy studies over a p e r i o d of 5 years from 1971 to 1976, at the D e p a r t m e n t of Pathology, M a u l a n a Azad Medical College, we came across 3 cases of bilateral renal dysplasia and 1 case of unilateral renal dysplasia, a m o n g neonatal cases and none a m o n g s t adults.
Case reports Case 1. P. M. (2/74). A three weeks postmature stillborn female child. Each kidney weighed 5 g and showed small cysts of varying sizes on the surface filled with clear fluid. The renal blood vessels and ureter were rudimentary. N o other congenital anomaly was seen. On the cut surface the whole kidney was found to be converted into several cysts of different sizes, filled with clear watery fluid. Microscopic examination showed several groups of tubular structures of varying sizes, lined by tall columnar to flattened epithelial cells. A m o n g some groups of tubules abhortive glomeruli were seen. Isolated and small groups of tubules were surrounded by thick mesenchymal tissue arranged concentrically. Areas of cartilage formation were also seen (Figs 1 - 3 ) . Case 2. P. M. (6/75). A male child, full-term breech delivery, who lived for * Paper presented at the 27th Annual Conference of the Indian Association of Pathologists and Microbiologists, December 19, 1976, Manipal, India. International Urology and Nephrology 10, 1978
90
Ahuja et al.: Bilateral renal dysplasia
14 hours. The right kidney weighed 15 g, tee left kidney 10 g. Both showed multiple cysts, of varying sizes, on the outer and cut surface filled with clear fluid. The ureter and renal vessels were rudimentary. There was no ott,_er congenital anomaly. The microscopic appearance of tke kidneys was tke same as in Case 1. Case 3. P. M. (9/76). A stillborn full-term female ckild, delivered by m/ncavity forceps. There were multiple copgerital abnormalities. The ears were large and low-set. The eyes had a mongoloid slant. T~ere was bilateral cataract, hyper-
Fig. 1. Group of tubules lined by columnar epithelium surrounded by concentrically arranged mesenchymal fibres. H & E, • 66
Fig. 2. Group of abhortive glomeruli with primitive tubular structures. H & E, • International Urology and Nephrology 10, 1978
Ahuja et at.: Bilateral renal dysplasia
91
extension of hands and adduction of arms, bilateral talepes and interventricular septal defect. The kidneys weighed l0 g each and showed m a n y cysts of different sizes on their outer and cut surface. The microscopic findings were similar to the above two cases, except that there was no cartilage formation.
Fig. 3. Two areas of cartilage (arrow), with abhortive glomeruli, a group of tubules and two
blood vessels surrounded by concentrically arranged mesenchymal fibres. H & E, x 66 One case of unilateral renal dysplasia was also come across, in a full-term male stillborn child. The right kidney was affected and showed multiple cysts o f varying sizes filled with clear fluid. The left kidney was normal. The microscopic appearance of the affected kidney was similar to that of Case 3. There were no other congenital anomalies. Incidence
The word renal dysplasia is used synonymously with multicystic kidney in several reports by different workers. Several cases of unilateral renal dysplasia are reported, as compared with bilateral renal dysplasia. The sex incidence as reported in renal dysplasia has no significance, and there is no special preponderance of any sex. Parkkulainen et ah [ 1] reported on 7 females and 12 males; Staubitz et al. [5] on 4 females and 3 males; Pathak et al. [3] on 2 females and 10 males, and in our series we found 2 males and 2 females. Unilateral cases can present at any age and are compatible with life. Bilateral renal dysplasia is usually incompatible with life. Discussion
Schwartz was the first to describe in 1936 unilateral multicystic kidney disease. Baggentosis in 1951 suggested the term dysplasia to disclose the presence 2
International Urology and Nephrology 10, 1978
92
Ahuja et al.: Bilateral renal dysplasia
of primitive renal element (cit. [1 ]) (primitive connective tissue with smooth muscle, primitive glomeruli, primitive tubules, capillary network, etc.). The term multicystic is preserved because it is descriptive of the macroscopic appearance of the deformity [1 ]. Spence [4] further differentiated this condition from polycystic kidney. It is now accepted that renal dysplasia is a disturbed differentiation of nephron tissue with persistence of structures inappropriate to the gestational age of the patient [2]. Many theories have been put forward to explain the etiopathogenesis of the cysts of the kidney, but none have been universally accepted and there is no agreement on the mechanism. The various theories are as follows: 1. Hildurbrandt (cit. [1-4]) thinks that failure of union of the nephrons arising from the nephrogenic blastema to unite with the collecting tubules of ureteric bud origin [3] results in this condition. This was the explanation given by him in 1894. 2. Ossthanonth and Potter in 1964 ascribed it to be due to premature cessation of branching by the ampullae of collecting tubules which are devoid of their capacity to induce the formation of nephrons from cysts (cit. [2]). 3. Heptinstall [2] reported in 1966 that (a) disorganized differentiation of metanephric tissue is an expression of the intrinsic potency of that tissue, deprived of the organizing influence of the ureteric bud, either because of the failure of the latter or some of its branches to develop, or because the capacity of the ampullae to induce nephrons has become somehow impaired by distal obstruction; and (b) the morphological consequence in the developing kidney, of obstruction itself, causes cystically dilated ampullae and cystic dilatation of the immature nephrons already induced. As can be seen, many attempts have been made to clarify this condition, but there is no satisfactory explanation as yet.
Acknowledgements We are grateful to the Dean and the Professor of Pathology, Maulana Azad Medical College, New Delhi, India, for permission to publish this report. We are also grateful to Mr. K. S. Jolly, for his help rendered by the photographs,
References 1. Parkkulainen, K. V., Itjelt, L., Sirola, K.: Congenital multicystic dysplasia of the kidney. Acta Chir. Scand., 244, Suppl. 1 (1959). 2. Heptinstall, R. N.: Pathology of the Kidney. First edition. J. A. Churchill, London 1966. 3. Pathak, I. G., William, D. L.: Multicystic and cystic dysplastic kidneys. Brit. J. Urol., 36, 318 (1964). 4. Spence, H. M.: Congenital unilateral multicystic kidney: an entity to be distinguished from polycystic kidney disease and other cystic disorders. J. Urol., 74, 693 (1955). 5. Staubitz, W. J., Jewett, T. C. Jr., Pletman, R. J. : Renal cystic disease in childhood. J. UroL, 90, 8 (1963). International Urology and Nephrology 10, 1978
