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Congenital Unilateral Multicystic Kidney in Infancy
THE JO'CRNAL OF 1.}ROLOGY
Vol. 81, No. 5, May 1959 Printed in U.S.A.
CO:NGENITAL UNILATERAL MULTICYSTIC KIDNEY IN INFAXCY JUA:i' C. BELTRAX From the Division of Surqery (Urology), Homer G. Phillips Hospital, St. Louis, 1vlo.
The purpose of this paper is to report a case of congenital, unilateral multicystic kidney in an infant, and to re-emphasize the concept that unilateral multicystic kidney is an anomaly unrelated to polycystic disease of the kidney and from which it should be differentiated. Recently, Moore' reviewed the literature and found 20 cases of congenital unilateral multicystic kidney, and he reported another case of his own (twentyfirst case); this report is the twenty-second known case of congenital unilateral multicystic kidney. Disagreement still exists among urologists and pathologists in regard to the nomenclature, classification and significance of cystic disease of the kidney. The reasons for this disagreement are multiple: a) Terminology. The prefixes poly, multi and multiple, having identical meaning, have bern used indiscriminately to designate different entities of cystic disease of the kidney whenever more than one cyst was encountered. By so doing, the same type of cystic disorder was differently nominated, or two separate cystic diseasrs were equally designated by different authors. b) Etiology. Since the etiology of this particular group of cystic diseases is to a great extent unknown, an accurate classification with markrd delineation of the different entities is difficult. It is true that most of them are of congenital origin, but the intimate mechanism is still unknown. Hildebrandt's2 theory of an abnormal union between the nephron and the collecting system, Kampmein's 3 and JYicKenna's 4 finding of a failure of embryonal generations of tubules to atrophy, and Hepler's 5 and Hinman's 6 idea of Accepted for publication September 15, 1958. 1 lVIoore, G. W. and Buchert, W. I.: Unilateral multicystic kidney in an infant. J. Urol., 78: 721, 1957.
2 Hildebrandt, A.: Weiterer Beitrag zi_\r pathologischen Anatomie der Nierengeschwiilste. Arch. f. ldin. Chir., 48: 343, 1894. 3 Kampmeier, 0. F.: A hitherto unrecognized mode of origin of congenital renal cysts. Surg., Gynec. & Obst., 36: 208, 1923. 4 ~le Kenna, C. M. and Kampmeier, 0. F.: Consideration of development of polycystic kidney. J. Urol., 32: 37, 1934. 5 Hepler, A. B.: Etiology of multilocular cysts of the kidney. J. Urol., 44: 206, 1940. 6 Hinman, F. and Morrison, D. M.: Com para-
urinary obstruction with secondary vascular impairment, are the usual accepted explanations. But, do the different congenital cystic diseases of the kidney really have a common etiology, or is there a different mechanism for each one of the srparate entities? This question still remains unanswered. In the case of unilateral multicystic kidney, Hildebrandt's theory seems to be the most logical one. In spite of the difficulties mentioned above, the following classification of congenital cystic diseases of the kidney is recommended in an attempt to help in the understanding of the pathologic, clinical and prognostic characteristics of each. CLASSIFICATION
Simple cyst. a) Single. The cyst, averaging in size about 8.5 cm. in diameter, involves only part of the kidney; macroscopically and microscopically the remainder of the renal parenchyma is normal and the cortex and medulla are well demarcated. There, is no communication of the cyst with the calyces or pelvis. Christeson,7 Chalkley and Sutton8 have reviewed the literature on simple cyst of the kidney in the infant. In their series the cyst was always unilateral. b) Multilocular. Part of the kidney, usually one of the poles, is replaced by a cystic mass consisting of a capsule and many septa which subdivide the mass into many cavities. On microscopic examination renal tissue cannot be identified between the walls of the cysts, but normal renal parenchyma is present in the unaffected part of the kidney. Frazier' reported two such cases, analyzed those previously recorded and clearly outlined these typical characteristics. All the known cases rcporkd have been unilateral.
tive study of circulatory changes in hydronephrosis, casco-cavernous tuberculous and polycystic kidney. J. Urol., 11: 131, l\l24. 7 Christeson, W.W.: Simple renal cysts in the newborn. Report of two cases. J. Urol., 72: 1137, 1954. 8 Chalkley, T. S. and Sutton, L. E.: Infected solitary cyst of the kidney in a child with review of the literature. J. Urol., 50: 414, 1U43. 9 Frazier, T. H.: Multilocular cysts of the kid, ney. J. Urol., 65: 351, Hl51.
602
UNJLA'l'ERAL l\IULTICYSTIC KIDNEY
Unilateral 111.ulticystic Spc11c:c10 clcarJy described this entity and pointed out thG charactc-~ristic differern·es h<'twcC'n unilateral multic::rntic kidney ancl polycyRtic disease. The affected kiclrn:y loses its normal contour ancl is replaced by an irregularly lobulated mass composed of ten or twelve cysts of varying size and averaging from one to fivp centimeters in diameter. Grossly, no renal parenchyma can be identified, but on microscopic examination <'mbryonal nephrons which have failed to develop normally arc present between the walls of the cysts. Tbe disease is always unilateral, nonhereditary and not associatrd with other congenital anomalies. Pol!}cystic kidney disease. In infancy the kidney is of normal size or slightly enlarged, retains its normal configuration, and uniform pea-size cysts are present over its eutire surface. Grossly, normal renal parcnchynrn can be observed between the cysts and microscopically well clevelopccl, functioning nephrons are present. Bagg11 conclusively demonstrated that polycystic disease of the kidnc:v is inherited as a mendclian recessive. Rall and Odel1 2 found a positive family history in 34 per cent of their rascs and a questionable history in 27 pcr cent. Associated congenital urological or nonurological anomalies are frequently encountered in renal polycy,;tic disease; they were found in 81 per cent of 46 cases studied at necropsy by Rall and Odel. Does unilateral congenital polycystic kidney really exist? Oppenheimer13 reviewed 60 cases and found all of them to be bilateral. Lowsley and Curtis, 14 in their series of 53 cases, were nnablr to find any instances of unilateral polycystic kidney. Campbell15 performed autopsies in 72 children with polycystic disease. The condition was bilateral in 46 and "grossly" limited to one side in but he states: "Histologic study of the opposite kidney will almost always disclose anomalous development, 10 Spence, H. M.: Congenital unilateral multicystic kidney . .J. Urol., 74: 693-706, 1955. 11 Bagg, H. S.: Hereditary structural defects in the descendants of mice exposed to x-ray radiation. Am . .J. AnaL 36: 275, Hl2S. 12 Rall, .J.E. and Ode!, H. M.: Congenital poly cvstic disease of the kiclnev. Review of the literati'ire rmd data on 207 cases·. Am . .J. l\Ied. Sc., 218: 399, 1949. 13 Oppenheimer, G. D. · Polycystic disease of (be kidney. Ann. Surg., 100: 1136, rn:14. u Lowsley, 0. S. and Curtis, :\I. S.: The surgical ,1speds of c,-stic disease of tlw kidney . .J. A. J\I. A., 127: 1112, Hl45. 15 Campbell, J\I.: Crology. Philadelphia: W. B. Satmclern Co., HJ54, vol. 1, p. 258.
(j(l;<
characteristically tlrn t of earl~, n'nal Ill poly cystic dist'asr·." Some of the rc·pmts in U1e literature of unilateral poly cystic kiclm·Y 11 ere demonstrated by Spence to lw in realit\' nni lateral nrnlticystic kidm·)-. The practical consideration of the above fact~ lead.~ one to conclude that if during an cxploratorr m an infant, one kidney is fonrnl to pre.sent. tlw
Fm. 1. Thirty minute excretory urogram ~hows left ureter displaced medially hy rnul1icystic kiJ-ney.
FIG. 2. Section of right kidney reveals 1wnnal architect.urn.
604
JUAN C. BELTRAN
typical characteristies of polycystic disease while the other one looks grossly normal, conservative treatment should be instituted. In all probability this normal-appearing kidney will develop cystic changes later in life. On the other hand, if the affected kidney presents the gross features of unilateral multicystic kidney as described above, a nephrectomy is indicated. CASE REPORT
No. 404880. R. L. was a newborn male infant. On the third day of life routine physical examination revealed a large, smooth, movable, compressible mass in the left upper quadrant of the abdomen. The size of this mass was approximately 8 cm. in its greatest diameter. An x-ray confirmed the presence of a soft tissue mass occupying the left half of the abdominal cavity. An excretory urogram showed the right kidney and ureter to
FIG. 3. A, left multicystic kidney. B, normal right kidney. ·
be normal throughout. The left kidney and calyceal system were not visualized. At the end of thirty minutes a small amount of dye was noted in the left renal pelvis and upper third of the ureter (fig. 1). The latter was displaced medially by an ill-defined mass occupying the left upper quadrant of the abdomen and apparently forming a part of the kidney itself. In the differential diagnosis Wilms tumor, unilateral multicystic kidney, congenital hydronephrosis, neuroblastoma and retroperitoneal teratoma were considered. An exploratory laparotomy was recommended. On the day of surgery the patient's respirations became depressed and irregular. The operation was canceled, the patient continued to do poorly and he died the following day. At autopsy bronchopneumonia was found to be the immediate cause of death. The right kidney was grossly normal. I ts surface was smooth and the capsule stripped with ease. On section normal architecture was noted. Microscopically no signs of any anomalous development were found (fig. 2). The left kidney was completely replaced by an irregularly lobulated mass composed of many cysts varying in size from one to five centimeters in diameter. The size of this mass was 8 by 4 by 2 cm. and weighed 200 gm. (fig. 3). On cut surface there was no demarcation between the cortex and medulla and there was no demonstrable renal parenchyma. The left ureter entered the cystic mass on its medial aspect and its insertion into the bladder was not unusual. On microscopic examination the cyst walls were
FIG. 4. Section of left kidney shows cyst wall composed of fibrous tissue lined bv layer of cuboidal epithelial cells, 11. Islands of embryonal glomeruli and tubules are also present, B. ·
UNILA'l'EUAL Ml:LTICYSTIC KIDNEY
composc:cl of fibrous tissue lined by a single layer of cuboiclal epithelial cells. The connective tissue betwc·en the ,rnlls of the ,ms of the embryonal, rncscnchymal t.vpc, and embedded in this tissue were several islands of cmbryonal glomeruli ancl tubuks (fig. 4). Some of these tubules communicate-cl with the while iu other areas blind-ending collecting tubules could be seen. DJSCUSSJ ON
Up to the present all of the rPportecl cases of unilateral multicystic kidney have been found on surgical exploration, and the opposite kidney has been described as grossly normal. Because of the lack of microscopic examination in this otherwise normal-appearing kidney, some urologists and pathologists have questioned the term unilateral and object to it on this basis. The)' contend that if a microscopic examination of the opposite, grossly nornml-appearing kidney had been made, congenital anomalous changes would have been found, as happens in polycystic disease. Lnfortunately, in our case tlw baby died of bronchopncumonia; however, this µermitted a detailed study of the disease as well as of the opposite kidney. As stated above, the right kidney was found macroscopically and microscopically to be completely normal. This proves without controversy the unilateral characteristic of this case. On the other hand, the other findings concur with those previously d(·scribed as typical of unilateral multicystic kidney. Another point of interest is the microscopic appearance of this kidney: the finding in some areas of embryonal
glomeruli and tubules, some of the latter commtmicating with the cysts, while in other only blindly ending collecting tub1.iles wcrc present, seems to corroboratu HildC'brnndt':o etiologic theory of rnalunion betwC'cu the nephni-genic substance and the collecting system. The main differential diagnostic between unilateral rnulticystic kidne:v and cystic kidney disease are: Unilateral lvlvlticystic Kidney 1) Unilateral occurrence 2) Loss of normal renal configuration 3) Wide variation m size of the cysts 4) No demorrntrable renal parenchyma
grossly 5) Embryonal nephrons 6) ~ ot hereditary 7) No other associated congenital anomalies
Polycyslic Kidney Disease l) Bilateral renc:e
ocru1
2) Retains normal 1'1'· na! configuration :)) Small si.ze, 1mifonn cysts 4) Easily identifiable renal parenchynw gro.ssly 5) Adult nephrons
6) Hereditary 7) Associated congeni-· tal anomalies
SUJ\HIAHY
The twenty-second known case of tmilateral multicystic kidney has b0en presented. It is believed that this congenital anomaly ic' a separate entity and should be differentiat.i-d from polyeystic disease of the kidney. The differential diagnosis between the different congenital cystic diseases of the kidney lrns been outlined and the literature reviewed,
Vol. 81, No. 5, May 1959 Printed in U.S.A.
CO:NGENITAL UNILATERAL MULTICYSTIC KIDNEY IN INFAXCY JUA:i' C. BELTRAX From the Division of Surqery (Urology), Homer G. Phillips Hospital, St. Louis, 1vlo.
The purpose of this paper is to report a case of congenital, unilateral multicystic kidney in an infant, and to re-emphasize the concept that unilateral multicystic kidney is an anomaly unrelated to polycystic disease of the kidney and from which it should be differentiated. Recently, Moore' reviewed the literature and found 20 cases of congenital unilateral multicystic kidney, and he reported another case of his own (twentyfirst case); this report is the twenty-second known case of congenital unilateral multicystic kidney. Disagreement still exists among urologists and pathologists in regard to the nomenclature, classification and significance of cystic disease of the kidney. The reasons for this disagreement are multiple: a) Terminology. The prefixes poly, multi and multiple, having identical meaning, have bern used indiscriminately to designate different entities of cystic disease of the kidney whenever more than one cyst was encountered. By so doing, the same type of cystic disorder was differently nominated, or two separate cystic diseasrs were equally designated by different authors. b) Etiology. Since the etiology of this particular group of cystic diseases is to a great extent unknown, an accurate classification with markrd delineation of the different entities is difficult. It is true that most of them are of congenital origin, but the intimate mechanism is still unknown. Hildebrandt's2 theory of an abnormal union between the nephron and the collecting system, Kampmein's 3 and JYicKenna's 4 finding of a failure of embryonal generations of tubules to atrophy, and Hepler's 5 and Hinman's 6 idea of Accepted for publication September 15, 1958. 1 lVIoore, G. W. and Buchert, W. I.: Unilateral multicystic kidney in an infant. J. Urol., 78: 721, 1957.
2 Hildebrandt, A.: Weiterer Beitrag zi_\r pathologischen Anatomie der Nierengeschwiilste. Arch. f. ldin. Chir., 48: 343, 1894. 3 Kampmeier, 0. F.: A hitherto unrecognized mode of origin of congenital renal cysts. Surg., Gynec. & Obst., 36: 208, 1923. 4 ~le Kenna, C. M. and Kampmeier, 0. F.: Consideration of development of polycystic kidney. J. Urol., 32: 37, 1934. 5 Hepler, A. B.: Etiology of multilocular cysts of the kidney. J. Urol., 44: 206, 1940. 6 Hinman, F. and Morrison, D. M.: Com para-
urinary obstruction with secondary vascular impairment, are the usual accepted explanations. But, do the different congenital cystic diseases of the kidney really have a common etiology, or is there a different mechanism for each one of the srparate entities? This question still remains unanswered. In the case of unilateral multicystic kidney, Hildebrandt's theory seems to be the most logical one. In spite of the difficulties mentioned above, the following classification of congenital cystic diseases of the kidney is recommended in an attempt to help in the understanding of the pathologic, clinical and prognostic characteristics of each. CLASSIFICATION
Simple cyst. a) Single. The cyst, averaging in size about 8.5 cm. in diameter, involves only part of the kidney; macroscopically and microscopically the remainder of the renal parenchyma is normal and the cortex and medulla are well demarcated. There, is no communication of the cyst with the calyces or pelvis. Christeson,7 Chalkley and Sutton8 have reviewed the literature on simple cyst of the kidney in the infant. In their series the cyst was always unilateral. b) Multilocular. Part of the kidney, usually one of the poles, is replaced by a cystic mass consisting of a capsule and many septa which subdivide the mass into many cavities. On microscopic examination renal tissue cannot be identified between the walls of the cysts, but normal renal parenchyma is present in the unaffected part of the kidney. Frazier' reported two such cases, analyzed those previously recorded and clearly outlined these typical characteristics. All the known cases rcporkd have been unilateral.
tive study of circulatory changes in hydronephrosis, casco-cavernous tuberculous and polycystic kidney. J. Urol., 11: 131, l\l24. 7 Christeson, W.W.: Simple renal cysts in the newborn. Report of two cases. J. Urol., 72: 1137, 1954. 8 Chalkley, T. S. and Sutton, L. E.: Infected solitary cyst of the kidney in a child with review of the literature. J. Urol., 50: 414, 1U43. 9 Frazier, T. H.: Multilocular cysts of the kid, ney. J. Urol., 65: 351, Hl51.
602
UNJLA'l'ERAL l\IULTICYSTIC KIDNEY
Unilateral 111.ulticystic Spc11c:c10 clcarJy described this entity and pointed out thG charactc-~ristic differern·es h<'twcC'n unilateral multic::rntic kidney ancl polycyRtic disease. The affected kiclrn:y loses its normal contour ancl is replaced by an irregularly lobulated mass composed of ten or twelve cysts of varying size and averaging from one to fivp centimeters in diameter. Grossly, no renal parenchyma can be identified, but on microscopic examination <'mbryonal nephrons which have failed to develop normally arc present between the walls of the cysts. Tbe disease is always unilateral, nonhereditary and not associatrd with other congenital anomalies. Pol!}cystic kidney disease. In infancy the kidney is of normal size or slightly enlarged, retains its normal configuration, and uniform pea-size cysts are present over its eutire surface. Grossly, normal renal parcnchynrn can be observed between the cysts and microscopically well clevelopccl, functioning nephrons are present. Bagg11 conclusively demonstrated that polycystic disease of the kidnc:v is inherited as a mendclian recessive. Rall and Odel1 2 found a positive family history in 34 per cent of their rascs and a questionable history in 27 pcr cent. Associated congenital urological or nonurological anomalies are frequently encountered in renal polycy,;tic disease; they were found in 81 per cent of 46 cases studied at necropsy by Rall and Odel. Does unilateral congenital polycystic kidney really exist? Oppenheimer13 reviewed 60 cases and found all of them to be bilateral. Lowsley and Curtis, 14 in their series of 53 cases, were nnablr to find any instances of unilateral polycystic kidney. Campbell15 performed autopsies in 72 children with polycystic disease. The condition was bilateral in 46 and "grossly" limited to one side in but he states: "Histologic study of the opposite kidney will almost always disclose anomalous development, 10 Spence, H. M.: Congenital unilateral multicystic kidney . .J. Urol., 74: 693-706, 1955. 11 Bagg, H. S.: Hereditary structural defects in the descendants of mice exposed to x-ray radiation. Am . .J. AnaL 36: 275, Hl2S. 12 Rall, .J.E. and Ode!, H. M.: Congenital poly cvstic disease of the kiclnev. Review of the literati'ire rmd data on 207 cases·. Am . .J. l\Ied. Sc., 218: 399, 1949. 13 Oppenheimer, G. D. · Polycystic disease of (be kidney. Ann. Surg., 100: 1136, rn:14. u Lowsley, 0. S. and Curtis, :\I. S.: The surgical ,1speds of c,-stic disease of tlw kidney . .J. A. J\I. A., 127: 1112, Hl45. 15 Campbell, J\I.: Crology. Philadelphia: W. B. Satmclern Co., HJ54, vol. 1, p. 258.
(j(l;<
characteristically tlrn t of earl~, n'nal Ill poly cystic dist'asr·." Some of the rc·pmts in U1e literature of unilateral poly cystic kiclm·Y 11 ere demonstrated by Spence to lw in realit\' nni lateral nrnlticystic kidm·)-. The practical consideration of the above fact~ lead.~ one to conclude that if during an cxploratorr m an infant, one kidney is fonrnl to pre.sent. tlw
Fm. 1. Thirty minute excretory urogram ~hows left ureter displaced medially hy rnul1icystic kiJ-ney.
FIG. 2. Section of right kidney reveals 1wnnal architect.urn.
604
JUAN C. BELTRAN
typical characteristies of polycystic disease while the other one looks grossly normal, conservative treatment should be instituted. In all probability this normal-appearing kidney will develop cystic changes later in life. On the other hand, if the affected kidney presents the gross features of unilateral multicystic kidney as described above, a nephrectomy is indicated. CASE REPORT
No. 404880. R. L. was a newborn male infant. On the third day of life routine physical examination revealed a large, smooth, movable, compressible mass in the left upper quadrant of the abdomen. The size of this mass was approximately 8 cm. in its greatest diameter. An x-ray confirmed the presence of a soft tissue mass occupying the left half of the abdominal cavity. An excretory urogram showed the right kidney and ureter to
FIG. 3. A, left multicystic kidney. B, normal right kidney. ·
be normal throughout. The left kidney and calyceal system were not visualized. At the end of thirty minutes a small amount of dye was noted in the left renal pelvis and upper third of the ureter (fig. 1). The latter was displaced medially by an ill-defined mass occupying the left upper quadrant of the abdomen and apparently forming a part of the kidney itself. In the differential diagnosis Wilms tumor, unilateral multicystic kidney, congenital hydronephrosis, neuroblastoma and retroperitoneal teratoma were considered. An exploratory laparotomy was recommended. On the day of surgery the patient's respirations became depressed and irregular. The operation was canceled, the patient continued to do poorly and he died the following day. At autopsy bronchopneumonia was found to be the immediate cause of death. The right kidney was grossly normal. I ts surface was smooth and the capsule stripped with ease. On section normal architecture was noted. Microscopically no signs of any anomalous development were found (fig. 2). The left kidney was completely replaced by an irregularly lobulated mass composed of many cysts varying in size from one to five centimeters in diameter. The size of this mass was 8 by 4 by 2 cm. and weighed 200 gm. (fig. 3). On cut surface there was no demarcation between the cortex and medulla and there was no demonstrable renal parenchyma. The left ureter entered the cystic mass on its medial aspect and its insertion into the bladder was not unusual. On microscopic examination the cyst walls were
FIG. 4. Section of left kidney shows cyst wall composed of fibrous tissue lined bv layer of cuboidal epithelial cells, 11. Islands of embryonal glomeruli and tubules are also present, B. ·
UNILA'l'EUAL Ml:LTICYSTIC KIDNEY
composc:cl of fibrous tissue lined by a single layer of cuboiclal epithelial cells. The connective tissue betwc·en the ,rnlls of the ,ms of the embryonal, rncscnchymal t.vpc, and embedded in this tissue were several islands of cmbryonal glomeruli ancl tubuks (fig. 4). Some of these tubules communicate-cl with the while iu other areas blind-ending collecting tubules could be seen. DJSCUSSJ ON
Up to the present all of the rPportecl cases of unilateral multicystic kidney have been found on surgical exploration, and the opposite kidney has been described as grossly normal. Because of the lack of microscopic examination in this otherwise normal-appearing kidney, some urologists and pathologists have questioned the term unilateral and object to it on this basis. The)' contend that if a microscopic examination of the opposite, grossly nornml-appearing kidney had been made, congenital anomalous changes would have been found, as happens in polycystic disease. Lnfortunately, in our case tlw baby died of bronchopncumonia; however, this µermitted a detailed study of the disease as well as of the opposite kidney. As stated above, the right kidney was found macroscopically and microscopically to be completely normal. This proves without controversy the unilateral characteristic of this case. On the other hand, the other findings concur with those previously d(·scribed as typical of unilateral multicystic kidney. Another point of interest is the microscopic appearance of this kidney: the finding in some areas of embryonal
glomeruli and tubules, some of the latter commtmicating with the cysts, while in other only blindly ending collecting tub1.iles wcrc present, seems to corroboratu HildC'brnndt':o etiologic theory of rnalunion betwC'cu the nephni-genic substance and the collecting system. The main differential diagnostic between unilateral rnulticystic kidne:v and cystic kidney disease are: Unilateral lvlvlticystic Kidney 1) Unilateral occurrence 2) Loss of normal renal configuration 3) Wide variation m size of the cysts 4) No demorrntrable renal parenchyma
grossly 5) Embryonal nephrons 6) ~ ot hereditary 7) No other associated congenital anomalies
Polycyslic Kidney Disease l) Bilateral renc:e
ocru1
2) Retains normal 1'1'· na! configuration :)) Small si.ze, 1mifonn cysts 4) Easily identifiable renal parenchynw gro.ssly 5) Adult nephrons
6) Hereditary 7) Associated congeni-· tal anomalies
SUJ\HIAHY
The twenty-second known case of tmilateral multicystic kidney has b0en presented. It is believed that this congenital anomaly ic' a separate entity and should be differentiat.i-d from polyeystic disease of the kidney. The differential diagnosis between the different congenital cystic diseases of the kidney lrns been outlined and the literature reviewed,