Pediat. Radiol. 3, 117--119 (1975) 9 by Springer-Verlag 1975
The "Septation Sign" in Multicystic Dysplastic Kidney R a l p h S. L a c h m a n 1, R a b b e R. L i n d s t r o m 2, a n d F r a n k M. H i r o s e a Departments of Radiology, Pediatrics and Pathology UCLA School of Medicine - Harbor General Hospital Torrance, California, U. S. A.
AbsfracI. The case report is presented of a neonate with a unilateral multicystic dysplastic kidney. An intravenous pyelogram revealed septations throughout this kidney with late pooling of contrast media within the cystic structures. The pathological data stresses the presence of normal appear-
ing glomeruli interspersed between the dysplastic cystic parenchyma. The possible etiologies for the septation sign and puddling phenomenon are discussed. Key words : sMulticystic kidney, dysplastic kidney, neonatal abdominal mass, total body opacification.
M u l t i c y s t i c d i s e a s e of t h e k i d n e y is n o t a r a r e p r o b l e m . G r i s c o m i n 1965 r e v e a l e d it t o b e t h e m o s t c o m m o n n e o n a t a l a b d o m i n a l m a s s [6]. T w o of his cases h a d s e p t a t i o n s r e c o g n i z e a b l e at i n t r a v e n o u s p y e l o g r a p h y ( I . V . P . ) w h i c h h e i n t e r p r e t e d as t h e r e s u l t of t h e t o t a l b o d y o p a c i f i c a t i o n effect. N u m e r o u s articles h a v e b e e n p u b l i s h e d c o n c e r n i n g t h e multicystic kidney which consistently mention that n o n v i s u a l i z a t i o n is t h e sine q u a n o n of t h e m u l t i c y s t i c k i d n e y [7]. W e are r e p o r t i n g a case of m u l t i c y s t i c r e n a l d y s p l a s i a w h i c h s h o w e d this p r e v i o u s l y d e s c r i b e d s e p t a t i o n a n d w h i c h also d e m o n s t r a t e d c o n t r a s t m a t e r i a l w i t h i n t h e confines of t h e cysts.
Case Report Case 1. M. J. F. was the product of a 25 year old caucasian, gravida 4, para 3, ab. 0. Three previous children were remarkably premature, but without history of congenital abnormalities. The prenatal history was uneventful except for an abnormal glucose tolerance test in the mother. The infant was full term and weighed 7 pounds 8 ounces. There was no family history of renal anomalies. The physical examination at birth was normal, except for a cystic feeling, abdominal mass which filled most of the right side of the abdomen. At about 30 hours of age, an I. V. P. was obtained. The blood urea nitrogen (BUN) at 10 hours was 20. Electrolytes and urine analysis were normal. The infant was operated on at one week of age. A right multicystic mass was removed from a markedly hypoplastic pelvis and an atretic ureter (Fig. 1). The post operative course was reasonable uneventful. The Radiology. The preliminary film of the intravenous urogram showed a right upper quadrant, soft tissue mass without calcification. 1 Associate Professor of Radiology and Pediatrics 2 Assistant Professor of Radiology a Associate Professor of Pathology
Fig. 1. The right multicystic mass removed at surgery.
5 minutes after the injection of 10 cc's of Renografin-60, septations were evident in the region of the right kidney interspersed between areas of lucency. The liver and spleen appeared quite dense due to total body opacification (Fig. 2). The septations persisted for 45 minutes but had disappeared by 60 minutes at which time there was pooling of contrast material near the previous sites of septation (Fig. 3). The total body opacification effect was evident 5 minutes after injection. It diminished by 30 minutes and was gone by 45 minutes. Films at 3 and 8 hours show no evidence of further puddling on the right. The left kidney appeared normal.
118
R.S. Lachman el a/.: The "Septation Sign" in Mutticystic Dysptastic Kidney The Pathology. The right kidney was represented by a 145 gram (normal value 13.0 gram) multicystic structure measuring 11.0 • 7.0 • 3.5 cm. (Fig. 1). The capsular surface was pink-gray to white and generally smooth. The diameter of the cysts were from 0.2 to 4.0 cm. and the wails were delicate, measuring in the neighborhood of 0.1 cm. in thickness. The cavities contained clear aqueous fluid. The specimen had all the features of a multicystic dysplastic kidney (1). The cysts were lined by low columnar to cuboidal epithelium, Smooth muscle could be identified in the embryonat mesenchyme which was interspersed between the cysts. A rare cartilagenous focus was identified (Fig. 4). In addition, regular renal parenchyma, commensurate with the age of the infant, could be found interspersed between the dysplastic cystic areas (Fig. 4).
Discussion
Fig. 2. The five minute film reveals the appearance of septations on the right side. Contrast media is already in the bladder
Fig. 3. The one hour film reveals pooling of contrast media within the areas of previous lucencies, Septations are not present any more.
Because of the somewhat extended phase of septations in this case and in view of the pathophysiology of the "crescents" in hydronephrosis as described by D u n b a r and N o g r a d y [51 which were pathologically substantiated by Levine et aL [8], we have attempted to deduce the etiology of the "septation sign" in multicystic disease. Some previous pathological data suggest that there is no connection between cysts and tubules, n o r between convoluted and collecting tubules in this f o r m of dysplastic kidney [10]. This, of course, would infer that one cannot have function in a multicystic kidney unless the entire kidney is not involved [31. Pathak and Williams have f o u n d scattered and isolated islands of relatively normal tubules and glomeruli [101. Roentgenographically, it is sometimes difficult to separate the "crescents" f o u n d in grade I V hydronephrosis f r o m septations [2]. T h e classic crescents are usually somewhat thicker and are present frequently in the first twenty to thirty minutes after injection [5]. In slight contrast, the total b o d y opacification phase is an immediate phenomenon, most marked just following the injection and t h e n subsiding gradually [9]. In looking at the liver and spleen in the I . V . P . , it appeared that the total body effect in these organs was gone after 45 minutes when the %eptation sign" was still present within the right kidney. This would suggest that some other mechanism rather than just total body opacification was occurring. Furthermore, some renal function possibly has occurred with the appearance of puddling of contrast media within some of these cysts (Fig. 3). A n o t h e r mechanism for the pooling of contrast media within the cysts is suggested by recent animal experiments utilizing
R. S. Lachman eta]. : The "Septation Sign" in Multicystic Dysplastic Kidney
119
Fig. 4. This portion of the dysplastic kidney shows a focus of cartilage within an area of normal renal parenchyma (240x).
tubular obstruction where the passage of urine or contrast media along the n e p h r o n is blocked, resulting in back diffusion into the interstitial tissues [41. T h e pathologic findings of others and in this case seem to support the fact that some variable potential function is present t h r o u g h o u t in this f o r m of dysplastic kidney [10J. T h e s e data suggest that this type of multicystic kidney m a y be a partially fnnctioning one and that the diagnosis w h e n looked for, m a y at times rest on the "septation sign" rather than nonvisualization to separate it f r o m h y d r o n e phrosis. A case of a multicystic (dysplastic) kidney is presented stressing the diagnostic value of the "septation sign" in this entity.
Acknowledgement.We wish to thank Dr. Thorne Griscom for his help and advice concerning this case. References 1. Arey, J. B.: Cystic lesions of the kidney in infants and children. J. Pediat. 84, 429-445 (1959) 2. Berdon, W. E., Levitt, S. B., Baker, D. H., Becker, J. A., Uson, A. C.: Hydronephrosis in infants and chiid~en -- value of high dosage excretory urography in
3. 4. 5. 6. 7. 8. 9.
10.
predicting renal salvageability. Amer. J. Roentgenol. 109, 380--389 (1970) Becket, J. A., Robinson, T.: Congenital multicystic disease in the adult. J. Canad. Ass. Radiol. 21, 165--168 (1970) Cattell, W. R.: Excretory pathways for contrast media. Invest. Radiol. 5, 485 (1970) Dunbar, J. S., Nogrady, N. B.: The calyceal crescent -A roentgenographic sign of obstructive hydronephrosis. Amer. J. Roentgenol. 110, 520--528 (1970) Griscom, N. T.: The roentgenoIogy of neonatal abdominal masses. Amer. J. Roentgenol. 93, 447--463 (1965) Grossman, H., Winchester, D. H., Chisani, F. V.: Roentgenographic classification of renal cystic disease. Amer. J. Roentgenol. 104, 319--331 (1968) Levine, M., Alien, A., Stein, J. C., Shwartz, S.: Crescent sign. Radiology 81, 971--973 (1963) O'Conner, J. F., Neuhauser, E. B. D.: Total body opacification on conventional and high dose intravenous urography in infancy. Amer. J. Roentgenol. 90, 63-71 (1963) Pathak, I. G., Williams, D. I. : Multicystic and cystic dysplastic kidneys. Brit. J. Urol. 36, 318-331 (1964)
Ralph S. Lachman, M. D. Dept. of Radiology Harbor General Hospital/UCLA 1000 W. Carson Street Torrance, CA 90509 USA
The "Septation Sign" in Multicystic Dysplastic Kidney R a l p h S. L a c h m a n 1, R a b b e R. L i n d s t r o m 2, a n d F r a n k M. H i r o s e a Departments of Radiology, Pediatrics and Pathology UCLA School of Medicine - Harbor General Hospital Torrance, California, U. S. A.
AbsfracI. The case report is presented of a neonate with a unilateral multicystic dysplastic kidney. An intravenous pyelogram revealed septations throughout this kidney with late pooling of contrast media within the cystic structures. The pathological data stresses the presence of normal appear-
ing glomeruli interspersed between the dysplastic cystic parenchyma. The possible etiologies for the septation sign and puddling phenomenon are discussed. Key words : sMulticystic kidney, dysplastic kidney, neonatal abdominal mass, total body opacification.
M u l t i c y s t i c d i s e a s e of t h e k i d n e y is n o t a r a r e p r o b l e m . G r i s c o m i n 1965 r e v e a l e d it t o b e t h e m o s t c o m m o n n e o n a t a l a b d o m i n a l m a s s [6]. T w o of his cases h a d s e p t a t i o n s r e c o g n i z e a b l e at i n t r a v e n o u s p y e l o g r a p h y ( I . V . P . ) w h i c h h e i n t e r p r e t e d as t h e r e s u l t of t h e t o t a l b o d y o p a c i f i c a t i o n effect. N u m e r o u s articles h a v e b e e n p u b l i s h e d c o n c e r n i n g t h e multicystic kidney which consistently mention that n o n v i s u a l i z a t i o n is t h e sine q u a n o n of t h e m u l t i c y s t i c k i d n e y [7]. W e are r e p o r t i n g a case of m u l t i c y s t i c r e n a l d y s p l a s i a w h i c h s h o w e d this p r e v i o u s l y d e s c r i b e d s e p t a t i o n a n d w h i c h also d e m o n s t r a t e d c o n t r a s t m a t e r i a l w i t h i n t h e confines of t h e cysts.
Case Report Case 1. M. J. F. was the product of a 25 year old caucasian, gravida 4, para 3, ab. 0. Three previous children were remarkably premature, but without history of congenital abnormalities. The prenatal history was uneventful except for an abnormal glucose tolerance test in the mother. The infant was full term and weighed 7 pounds 8 ounces. There was no family history of renal anomalies. The physical examination at birth was normal, except for a cystic feeling, abdominal mass which filled most of the right side of the abdomen. At about 30 hours of age, an I. V. P. was obtained. The blood urea nitrogen (BUN) at 10 hours was 20. Electrolytes and urine analysis were normal. The infant was operated on at one week of age. A right multicystic mass was removed from a markedly hypoplastic pelvis and an atretic ureter (Fig. 1). The post operative course was reasonable uneventful. The Radiology. The preliminary film of the intravenous urogram showed a right upper quadrant, soft tissue mass without calcification. 1 Associate Professor of Radiology and Pediatrics 2 Assistant Professor of Radiology a Associate Professor of Pathology
Fig. 1. The right multicystic mass removed at surgery.
5 minutes after the injection of 10 cc's of Renografin-60, septations were evident in the region of the right kidney interspersed between areas of lucency. The liver and spleen appeared quite dense due to total body opacification (Fig. 2). The septations persisted for 45 minutes but had disappeared by 60 minutes at which time there was pooling of contrast material near the previous sites of septation (Fig. 3). The total body opacification effect was evident 5 minutes after injection. It diminished by 30 minutes and was gone by 45 minutes. Films at 3 and 8 hours show no evidence of further puddling on the right. The left kidney appeared normal.
118
R.S. Lachman el a/.: The "Septation Sign" in Mutticystic Dysptastic Kidney The Pathology. The right kidney was represented by a 145 gram (normal value 13.0 gram) multicystic structure measuring 11.0 • 7.0 • 3.5 cm. (Fig. 1). The capsular surface was pink-gray to white and generally smooth. The diameter of the cysts were from 0.2 to 4.0 cm. and the wails were delicate, measuring in the neighborhood of 0.1 cm. in thickness. The cavities contained clear aqueous fluid. The specimen had all the features of a multicystic dysplastic kidney (1). The cysts were lined by low columnar to cuboidal epithelium, Smooth muscle could be identified in the embryonat mesenchyme which was interspersed between the cysts. A rare cartilagenous focus was identified (Fig. 4). In addition, regular renal parenchyma, commensurate with the age of the infant, could be found interspersed between the dysplastic cystic areas (Fig. 4).
Discussion
Fig. 2. The five minute film reveals the appearance of septations on the right side. Contrast media is already in the bladder
Fig. 3. The one hour film reveals pooling of contrast media within the areas of previous lucencies, Septations are not present any more.
Because of the somewhat extended phase of septations in this case and in view of the pathophysiology of the "crescents" in hydronephrosis as described by D u n b a r and N o g r a d y [51 which were pathologically substantiated by Levine et aL [8], we have attempted to deduce the etiology of the "septation sign" in multicystic disease. Some previous pathological data suggest that there is no connection between cysts and tubules, n o r between convoluted and collecting tubules in this f o r m of dysplastic kidney [10]. This, of course, would infer that one cannot have function in a multicystic kidney unless the entire kidney is not involved [31. Pathak and Williams have f o u n d scattered and isolated islands of relatively normal tubules and glomeruli [101. Roentgenographically, it is sometimes difficult to separate the "crescents" f o u n d in grade I V hydronephrosis f r o m septations [2]. T h e classic crescents are usually somewhat thicker and are present frequently in the first twenty to thirty minutes after injection [5]. In slight contrast, the total b o d y opacification phase is an immediate phenomenon, most marked just following the injection and t h e n subsiding gradually [9]. In looking at the liver and spleen in the I . V . P . , it appeared that the total body effect in these organs was gone after 45 minutes when the %eptation sign" was still present within the right kidney. This would suggest that some other mechanism rather than just total body opacification was occurring. Furthermore, some renal function possibly has occurred with the appearance of puddling of contrast media within some of these cysts (Fig. 3). A n o t h e r mechanism for the pooling of contrast media within the cysts is suggested by recent animal experiments utilizing
R. S. Lachman eta]. : The "Septation Sign" in Multicystic Dysplastic Kidney
119
Fig. 4. This portion of the dysplastic kidney shows a focus of cartilage within an area of normal renal parenchyma (240x).
tubular obstruction where the passage of urine or contrast media along the n e p h r o n is blocked, resulting in back diffusion into the interstitial tissues [41. T h e pathologic findings of others and in this case seem to support the fact that some variable potential function is present t h r o u g h o u t in this f o r m of dysplastic kidney [10J. T h e s e data suggest that this type of multicystic kidney m a y be a partially fnnctioning one and that the diagnosis w h e n looked for, m a y at times rest on the "septation sign" rather than nonvisualization to separate it f r o m h y d r o n e phrosis. A case of a multicystic (dysplastic) kidney is presented stressing the diagnostic value of the "septation sign" in this entity.
Acknowledgement.We wish to thank Dr. Thorne Griscom for his help and advice concerning this case. References 1. Arey, J. B.: Cystic lesions of the kidney in infants and children. J. Pediat. 84, 429-445 (1959) 2. Berdon, W. E., Levitt, S. B., Baker, D. H., Becker, J. A., Uson, A. C.: Hydronephrosis in infants and chiid~en -- value of high dosage excretory urography in
3. 4. 5. 6. 7. 8. 9.
10.
predicting renal salvageability. Amer. J. Roentgenol. 109, 380--389 (1970) Becket, J. A., Robinson, T.: Congenital multicystic disease in the adult. J. Canad. Ass. Radiol. 21, 165--168 (1970) Cattell, W. R.: Excretory pathways for contrast media. Invest. Radiol. 5, 485 (1970) Dunbar, J. S., Nogrady, N. B.: The calyceal crescent -A roentgenographic sign of obstructive hydronephrosis. Amer. J. Roentgenol. 110, 520--528 (1970) Griscom, N. T.: The roentgenoIogy of neonatal abdominal masses. Amer. J. Roentgenol. 93, 447--463 (1965) Grossman, H., Winchester, D. H., Chisani, F. V.: Roentgenographic classification of renal cystic disease. Amer. J. Roentgenol. 104, 319--331 (1968) Levine, M., Alien, A., Stein, J. C., Shwartz, S.: Crescent sign. Radiology 81, 971--973 (1963) O'Conner, J. F., Neuhauser, E. B. D.: Total body opacification on conventional and high dose intravenous urography in infancy. Amer. J. Roentgenol. 90, 63-71 (1963) Pathak, I. G., Williams, D. I. : Multicystic and cystic dysplastic kidneys. Brit. J. Urol. 36, 318-331 (1964)
Ralph S. Lachman, M. D. Dept. of Radiology Harbor General Hospital/UCLA 1000 W. Carson Street Torrance, CA 90509 USA
