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Sponge Kidney
THE JOURNAL OF UROLOGY
Vol. 84, No. 2, August 1960 Printed in U.S.A.
SPONGE KIDNEY SALVATORE A. PENNISI*
AND
R. CARL BUNTS
From the Urological Section, S-u.rgical Service, McGuire Veterans Administration Hospital, Richmond, Va.
Sponge kidney or, more descriptively, cystic disease of the medullary portion of the renal pyramids was apparently first reported in the American literature by Smith and Graham in 1945. 1 This was a single case report. The authors did not attempt to delineate this as a disease entity. In the United States Vermooten, 2 Rogness and Burnell, 3 Mulvaney and Collins, 4 have reported similar cases. In all a total of 7 cases have been reported. In 1939 Lenarduzzi first reported the radiographic appearance of this lesion. 5 However, the credit for the complete delineation of sponge kidney as a disease entity must go to Cacchi and Ricci. 6 In 1949 these authors, in reporting 5 cases, correlated the complete clinical description of this disease entity with its basic pathology. CASE REPORTS
Five additional case reports of this lesion follow: Case 1. E. G., 16-107, a 36-year-old white man, was admitted to the urology service on April 22, 1947 with a chief complaint of a chronic dull backache and a past history of intermittent hematuria and dysuria dating back to 1944. Physical examination showed moderate tenderness in the right upper quadrant but was otherwise unremarkable. Routine laboratory studies, including the blood urea nitrogen, were within normal limits except for the findin;g of albuminuria, pyuria, and inability to concentrate urine above 1.008. Intravenous pyelography revealed a rather normal pelvic and calyceal structure with a small ureterocele (fig. 1, A). Retrograde pyelograms with overfilling revealed bilateral Accepted for publication July 31, 1959. * Present address: 612 Benson St., Camden, N. J. 1 Smith, C. H. and Graham, J. B.: Am. J. Dis. Child., 69: 369, 1945. 2 Vermooten, V.: Yale J. Biol. and Med., 23: 450, 1951.
3 Rogness, J. R. and Burnell, J. M.: Arch. Int. Med., 93: 355, 1954. 4 Mulvaney, W. P. and Collins, W. T.: J. Urol.,
75: 776, 1956. 5 6
Lenarduzzi, G.: Radio!. med., 26: 346, 1939. Cacchi, R. and Ricci, V.: J. d'urol., 55: 498,
1949. 246
dilated calyces with moderate retention, pyeloureterectasis, and numerous small sacculations at the proximal end of each of the minor calyces bilaterally (fig. 1, B). Cystoscopic examination showed chronic cystitis and a mild ureterocele on the right. This was treated by transurethral incision of the ureteral orifice. Both ureters were then dilated transurethrally and sulfa therapy was instituted. There was no objective or subjective improvement. The patient left the hospital against medical advice at this time. On September 17, 1948 the patient was readmitted to the urology service because of pain in the left side, left hip, and thigh. Physical examination revealed a chronically ill white man with puffy eyelids, bilateral tenderness in the costovertebral angle, and suprapubic tenderness. Laboratory work again showed a normal blood urea nitrogen and pyuria with albuminuria. Intravenous pyelography showed some improvement over the previous examination but the bilateral calyceal sacculations were still present. Cystoscopic examination again showed chronic cystitis. Approximately 75 cc purulent urine was aspirated from the left side on catheterization. One-half per cent silver nitrate lavage was carried out on this side. There was no residual urine on the right side. Improvement was marked and the patient was discharged. He was lost to further followup. Case 2. C. S., 89-571, a 38-year-old white man, was admitted to the urology service of Roanoke V. A. Hospital on March 13 1957 complaining of pain in the low back 6 months in duration. Dysuria and intermittent hematuria of a few weeks' duration had also been noted. Studies at that installation showed bilateral Pseudomonas infection resistant to all antibiotics. The patient was then transferred to the McGuire V. A. Hospital for further urologic study. Laboratory examination showed pyuria and 1 + albuminuria. Complete blood chemistry, blood urea nitrogen and serology were within normal limits. Urine cultures were negative for tuberculosis. A chest x-ray was unremarkable. Urine culture which was positive for Pseudomonas was sensitive to
SPONGE KlD2'EY
FrG. 1. A, retrogrnde pyelogram with overfilling revealed dilated calyceP and mtmer011s snw11 s,H·c,1latio1rn ai end of calyces. n, normal appearing 15 minute excretory urograrn.
Frn. 2. A, retrograde pyelogrnm demonstrates slight caliectasis, but only an oecasional papillniy B, excretor~' urogra.rn (1.5 minute) shows multiple sacculations at proximal ends of minor Le rally.
248
S. A. PENNISI AND R. C. BUNTS
mandelamine. Retrograde pyelograms demonstrated slight caliectasis, but only the presence of a few papillary cysts (fig. 2, A). An intravenous pyelogram at this time revealed minimal caliectasis and ureterectasis bilaterally with multiple
Fm. 3. Grape-like clusters with calculi most typical at inferior pole of left kidney.
small sacculations at the proximal ends of the minor calyces bilaterally (fig. 2, B). There was marked improvement on mandelamine therapy and the patient was discharged to be followed in 3 months. He was readmitted on September 9, 1957 with the same complaints as before, namely backache, dysuria, and hematuria. Laboratory studies again showed pyuria. Physical examination this time was completely negative. Intravenous pyelography again revealed the peculiar sacculations at the minor calyces as before. Antibiotic therapy was of no avail. Intravenous injections of furadantin were started. The infection gradually disappeared and the patient left the hospital against advice. Case 3. C. S., A-3462, a 35-year-old white man, was admitted to the McGuire V. A. Hospital in October 1959, his first admission being in 1952 at which time he gave a history of having been discharged from the Army in 1944 because of bilateral renal calculi. Urine had shown pus and blood on several occasions. Repeated serum calcium and phosphate reabsorption tests were normal. He had passed stones on several occasions and required right ureterolithotomy on July 7, 1955. Excretory urogram (fig. 3) revealed grapelike clusters with calculi most typical at the lower pole of the left kidney. Case 4. J. C. D., 38-802, a 52-year-old white
Fm. 4. A, retrograde pyelogram. Bilateral nephrocalcinosis with rounded calculi in group located outside calyces. B, injected autopsy specimen. Note calculi in parenchyma in cyst spaces surrounded by air and blood.
SPOXGE KlllNEY
man, was admitted to tbe :\fcGuire V. A. Hospital on ,Janua.ry 10, 1952 eomplaining of hematuria and the passage of sarnly material in the urine. Retrograde pyelograms showrc! bilateral nephrocakinosis ,,-ith rounded calculi in groups located just outside cal)•ees in the papillae (fig. 4 Frine showed numerous red and white blood cell.,. Serum calcium and phosphorus were normal, blood urea nitrogen 13 mg. per
cent, urea clearance normal, plwnolsulfonphthalcin 63 per cent in 2 hours, Benc(•-.Jonc•,, prot(,ins normal. Blackler inspection ren•ul1:1!, grade 2 infiltrating carcinoma 011 the left wall; partial cystectorny performed on 24, 1952 revealed ncnrnining tumor in of the specimen and on February 28 the, remaind;;i of the bladder was removrd and urctcrosigmoido~ tomy carried out. The patient rxpired on i\larch
Fm. 5. A, x-rn:-· shows bilateral multiple parenchymal ealculi. B, note eystie mass in luwer twu thirds of right, kidne>' with possible c:vst of papillae bila ternlly. C, kidney specimen. Nole large nrnss in miclportion. Area of neoplastic tissue 1 to 3 cm. in outer wall. Several papillae. especiall.1· nt, lower pole, showed cps(ic areas, some containing calculi.
250
S. ,\. PE:'\NISI AND R. C. BUNTS
18 due to small bowel rupture. Pathological report of kidney revealed "tubular elements within the papillae are distended and contain densely calcified concretions." X-ray photographs of the excised kidneys showed rounded and irregular calculi in the parenchyma, some surrounded by rarefied areas, probably either air or blood in the cyst (fig. 4, B). Case 5. R. L., A-3263, a 51-ycar-old white man, was first admitted to the .i\IcGuire V. A. Hospital on February 20, 1956. He gave a history of having been informed in 1952 that he had stones in the "periphery" of his kidneys. He further states that he had several attacks of hematuria, frequency, and pain in the right flank. Urine was loaded with white blood cells, culture was positive for Pseudomonas and A. aerogencs, blood urea nitrogen 12, scrum calcium and phosphorus were normal. A diagnostic x-ray showed bilateral renal parenchymal calculi and calculi in the left renal pelvis, but the patient stated that he had never passed calculi (fig. 5, A). Left ureterolithotomy was required on August 22, 1957. In the latter part of 1958 it was noted that the right kidney began to have a cystic appearance. This became more evident by July 1959 (fig. 5, B) and was thought to be a unilateral polycystic kidney on the right and possible papillary cyst with calculi in the upper and lower poles of the left kidney. Exploration of the right kidney was carried out July 9 and what was thought to be a rather typical polycystic kidney was seen. Several cysts were decompressed. Biopsy of the wall of one cyst, among many examined, revealed dear cell carcinoma. After removal of the stones from the left renal pelvis and several calculi from medullary cystic spaces in this kidney, inulin and P AH clearances having revealed that the left kidney would sustain life, a right nephrectomy was performed on August 26. The gross specimen (fig. 5, C) revealed a large cystic mass in the midportion of the kidney with two small linear areas of clear cell carcinoma. In several papillae and especially at the lower pole there were cystic areas, some containing eakuli. Comment: Vispignani7 found a similar condition of papillary cyst, pyelogenic and polycystic changes in the same kidney. One case reported by Cacchi and Ricci showed an associated benign adenoma.
DISCUSSION
When one compares the rarity of reports of this condition in the American urological literature, viz., 7 cases to the 90 collected cases in the European literature as of October 1957 and at least 35 cases in the Scandinavian literature as of March 1959, 8 it is readily apparent that a digest of a foreign report is worth considering at this point. A thorough and complete delineation of this disease, including renal dearance studies, has been presented by Cacchi at the 30th CongTPSS of the Italian Urological Society, K aples, October 1957. An abstract of Cacchi's report follows: Pathology. The basic gross, macroscopic, and microscopic pathology of this lesion is constant and is described in detail. This histopathology has been confirmed by Powell, Gunther, Rogness and associates, and :\Iulvaney and associates. Powell and Gunther have also noted microlithiasis within the dilated collecting tubules, diverticulum formations coming off the collecting tubules, and in the true cysts. Etiology. A developmental etiology based on McKenna and Karnpmeier's 9 work is favored. It is noteworthy that two sets of siblings with this lesion have been reported. c'rography. Retrograde urography is usually unrevealing while intravenous pyelography dearly demonstrates the lesion. It is not surprising that lithiasis is usually present inasmuch as the lesion demonstrates the ideal situation for stone fornmtion, namely, stagnant urine, cellular debris, and infection. However, other factors must be involved as some cases remain free, or relatively free, of nephrolithiasis over many years while others go on to staghorn formation. Sponge kidney with lithiasis sometimes presents a classic picture of nephrocalcinosis. In advanced cases it is impossible to differentiate the two lesions without prior films. The presence of microlithiasis and frank lithiasis is found more often than realized, viz., 24 of 29 personal cases. Sponge kidney is added to the list of causes of nephrolithiasis. Clinical considerations. There were 77 males and 19 females. Eighty-one patients were between 31 and 60 years of age. Five were between 7 and 14 years of age. In many the first symptoms antedated the x-ray ancl, therefore, the diagnosis by many years. The latent period Lindvall N.: Acta radio!., 51: 193, 1959. McKenn~, C. M. and Kampmeier, 0. F.: ,J. Urol., 32: 37, 1934. 8
9
7
Vispignani: Quoted by Cacchi and Ricci.
SPO,';GJD KfDNEY
of this condition i~ m1wh longer than that of polycrstic renal disease. Sponge kidnc,y patients do not have an unusual inciclem·e of hypert" to a (_i:3.75 per cent cldiciency. On th<: othn hand, tubuhtr function was nmmal in SC\Tll of the 12 patiPnts. In the other 5 patient~ the tubular deficien("y rnng<"d from 4.tl per c<,nt to 66.G7 per cent. Diaqnosis. The symptomatology of sponge kidne)· is inconstant rmcl non1mthog<'nic. Symptoms of cystitis ancl pyelonephritis are the most common presenting complaints. Cases complicated by lithiasis lrnvc repeated episodes of colic. H<,nrnturia is usual!>· gross and accompanied by colic or inflammatory episodes hut can be painles,c; and aS)c111ptomatic:. Tuberculosis, necrotizing papillitis, nqihrocakiuosis, and P>·elosinns reflu:c.: sometimes enter into thl' differential diagnosis of the disease c,ntit)'. Proqnosis. The prognosis is generally good. Some cases show minimal prognc·ssion of the lesion or lithiasis. The prognosis 1s ,nJrse among thme patients \\·ith nqihrocal-
cmosis, staghom calculi, and repeated seven, t~pisorlcs of pyelonephritis. Sponge kidney \1itit obstruction to tlw urinar:' pathwa>·s because' calculi or peritubnlar fibrosis can C'\"l'1Jtually Yelop into a full blom1 hydrom·phrotic 11·hercin both the mc:dulla and the cork:,; thinned out. These patit nts then assmm: a state of chronic renal insuf!icietJC)" with irn·verni bJrc· azotemia and septicemia. Treatment. treatm<'nt of the infection and, within the reduction of the rnmplicatiou ol" urolithi::;,1,, better the prognosis of spougt' kidnc:y Orn· C',l lt newr speak of cnring sponge kidney; thrn· not a single case 1Yhere the rndiologieal has n·grcsserl OYer the yrars. Tlwrap_v is th· trerltment of complications. Bemus<, of the clrainagr from thcsmall saccnlations, treatment of" infection e:c.:tremcly difficult and lmsatitsfactory rt if: ah, onr experiern'P that in one case thC' film showed rrhttin:ly normal µch·io('.a]yceal terns, while the intravenous pp:logram cle:irh rlelineaterl the lesion. In general, tl1i_,, di,,ea,''<' entity can be likened to c!i,sP:tS<,', c:c.:ccpt that tht' prognosrn is cousiclera bl)· hettn in most cases. 0
C
SUMMARY
Fin' cases of sp
Vol. 84, No. 2, August 1960 Printed in U.S.A.
SPONGE KIDNEY SALVATORE A. PENNISI*
AND
R. CARL BUNTS
From the Urological Section, S-u.rgical Service, McGuire Veterans Administration Hospital, Richmond, Va.
Sponge kidney or, more descriptively, cystic disease of the medullary portion of the renal pyramids was apparently first reported in the American literature by Smith and Graham in 1945. 1 This was a single case report. The authors did not attempt to delineate this as a disease entity. In the United States Vermooten, 2 Rogness and Burnell, 3 Mulvaney and Collins, 4 have reported similar cases. In all a total of 7 cases have been reported. In 1939 Lenarduzzi first reported the radiographic appearance of this lesion. 5 However, the credit for the complete delineation of sponge kidney as a disease entity must go to Cacchi and Ricci. 6 In 1949 these authors, in reporting 5 cases, correlated the complete clinical description of this disease entity with its basic pathology. CASE REPORTS
Five additional case reports of this lesion follow: Case 1. E. G., 16-107, a 36-year-old white man, was admitted to the urology service on April 22, 1947 with a chief complaint of a chronic dull backache and a past history of intermittent hematuria and dysuria dating back to 1944. Physical examination showed moderate tenderness in the right upper quadrant but was otherwise unremarkable. Routine laboratory studies, including the blood urea nitrogen, were within normal limits except for the findin;g of albuminuria, pyuria, and inability to concentrate urine above 1.008. Intravenous pyelography revealed a rather normal pelvic and calyceal structure with a small ureterocele (fig. 1, A). Retrograde pyelograms with overfilling revealed bilateral Accepted for publication July 31, 1959. * Present address: 612 Benson St., Camden, N. J. 1 Smith, C. H. and Graham, J. B.: Am. J. Dis. Child., 69: 369, 1945. 2 Vermooten, V.: Yale J. Biol. and Med., 23: 450, 1951.
3 Rogness, J. R. and Burnell, J. M.: Arch. Int. Med., 93: 355, 1954. 4 Mulvaney, W. P. and Collins, W. T.: J. Urol.,
75: 776, 1956. 5 6
Lenarduzzi, G.: Radio!. med., 26: 346, 1939. Cacchi, R. and Ricci, V.: J. d'urol., 55: 498,
1949. 246
dilated calyces with moderate retention, pyeloureterectasis, and numerous small sacculations at the proximal end of each of the minor calyces bilaterally (fig. 1, B). Cystoscopic examination showed chronic cystitis and a mild ureterocele on the right. This was treated by transurethral incision of the ureteral orifice. Both ureters were then dilated transurethrally and sulfa therapy was instituted. There was no objective or subjective improvement. The patient left the hospital against medical advice at this time. On September 17, 1948 the patient was readmitted to the urology service because of pain in the left side, left hip, and thigh. Physical examination revealed a chronically ill white man with puffy eyelids, bilateral tenderness in the costovertebral angle, and suprapubic tenderness. Laboratory work again showed a normal blood urea nitrogen and pyuria with albuminuria. Intravenous pyelography showed some improvement over the previous examination but the bilateral calyceal sacculations were still present. Cystoscopic examination again showed chronic cystitis. Approximately 75 cc purulent urine was aspirated from the left side on catheterization. One-half per cent silver nitrate lavage was carried out on this side. There was no residual urine on the right side. Improvement was marked and the patient was discharged. He was lost to further followup. Case 2. C. S., 89-571, a 38-year-old white man, was admitted to the urology service of Roanoke V. A. Hospital on March 13 1957 complaining of pain in the low back 6 months in duration. Dysuria and intermittent hematuria of a few weeks' duration had also been noted. Studies at that installation showed bilateral Pseudomonas infection resistant to all antibiotics. The patient was then transferred to the McGuire V. A. Hospital for further urologic study. Laboratory examination showed pyuria and 1 + albuminuria. Complete blood chemistry, blood urea nitrogen and serology were within normal limits. Urine cultures were negative for tuberculosis. A chest x-ray was unremarkable. Urine culture which was positive for Pseudomonas was sensitive to
SPONGE KlD2'EY
FrG. 1. A, retrogrnde pyelogram with overfilling revealed dilated calyceP and mtmer011s snw11 s,H·c,1latio1rn ai end of calyces. n, normal appearing 15 minute excretory urograrn.
Frn. 2. A, retrograde pyelogrnm demonstrates slight caliectasis, but only an oecasional papillniy B, excretor~' urogra.rn (1.5 minute) shows multiple sacculations at proximal ends of minor Le rally.
248
S. A. PENNISI AND R. C. BUNTS
mandelamine. Retrograde pyelograms demonstrated slight caliectasis, but only the presence of a few papillary cysts (fig. 2, A). An intravenous pyelogram at this time revealed minimal caliectasis and ureterectasis bilaterally with multiple
Fm. 3. Grape-like clusters with calculi most typical at inferior pole of left kidney.
small sacculations at the proximal ends of the minor calyces bilaterally (fig. 2, B). There was marked improvement on mandelamine therapy and the patient was discharged to be followed in 3 months. He was readmitted on September 9, 1957 with the same complaints as before, namely backache, dysuria, and hematuria. Laboratory studies again showed pyuria. Physical examination this time was completely negative. Intravenous pyelography again revealed the peculiar sacculations at the minor calyces as before. Antibiotic therapy was of no avail. Intravenous injections of furadantin were started. The infection gradually disappeared and the patient left the hospital against advice. Case 3. C. S., A-3462, a 35-year-old white man, was admitted to the McGuire V. A. Hospital in October 1959, his first admission being in 1952 at which time he gave a history of having been discharged from the Army in 1944 because of bilateral renal calculi. Urine had shown pus and blood on several occasions. Repeated serum calcium and phosphate reabsorption tests were normal. He had passed stones on several occasions and required right ureterolithotomy on July 7, 1955. Excretory urogram (fig. 3) revealed grapelike clusters with calculi most typical at the lower pole of the left kidney. Case 4. J. C. D., 38-802, a 52-year-old white
Fm. 4. A, retrograde pyelogram. Bilateral nephrocalcinosis with rounded calculi in group located outside calyces. B, injected autopsy specimen. Note calculi in parenchyma in cyst spaces surrounded by air and blood.
SPOXGE KlllNEY
man, was admitted to tbe :\fcGuire V. A. Hospital on ,Janua.ry 10, 1952 eomplaining of hematuria and the passage of sarnly material in the urine. Retrograde pyelograms showrc! bilateral nephrocakinosis ,,-ith rounded calculi in groups located just outside cal)•ees in the papillae (fig. 4 Frine showed numerous red and white blood cell.,. Serum calcium and phosphorus were normal, blood urea nitrogen 13 mg. per
cent, urea clearance normal, plwnolsulfonphthalcin 63 per cent in 2 hours, Benc(•-.Jonc•,, prot(,ins normal. Blackler inspection ren•ul1:1!, grade 2 infiltrating carcinoma 011 the left wall; partial cystectorny performed on 24, 1952 revealed ncnrnining tumor in of the specimen and on February 28 the, remaind;;i of the bladder was removrd and urctcrosigmoido~ tomy carried out. The patient rxpired on i\larch
Fm. 5. A, x-rn:-· shows bilateral multiple parenchymal ealculi. B, note eystie mass in luwer twu thirds of right, kidne>' with possible c:vst of papillae bila ternlly. C, kidney specimen. Nole large nrnss in miclportion. Area of neoplastic tissue 1 to 3 cm. in outer wall. Several papillae. especiall.1· nt, lower pole, showed cps(ic areas, some containing calculi.
250
S. ,\. PE:'\NISI AND R. C. BUNTS
18 due to small bowel rupture. Pathological report of kidney revealed "tubular elements within the papillae are distended and contain densely calcified concretions." X-ray photographs of the excised kidneys showed rounded and irregular calculi in the parenchyma, some surrounded by rarefied areas, probably either air or blood in the cyst (fig. 4, B). Case 5. R. L., A-3263, a 51-ycar-old white man, was first admitted to the .i\IcGuire V. A. Hospital on February 20, 1956. He gave a history of having been informed in 1952 that he had stones in the "periphery" of his kidneys. He further states that he had several attacks of hematuria, frequency, and pain in the right flank. Urine was loaded with white blood cells, culture was positive for Pseudomonas and A. aerogencs, blood urea nitrogen 12, scrum calcium and phosphorus were normal. A diagnostic x-ray showed bilateral renal parenchymal calculi and calculi in the left renal pelvis, but the patient stated that he had never passed calculi (fig. 5, A). Left ureterolithotomy was required on August 22, 1957. In the latter part of 1958 it was noted that the right kidney began to have a cystic appearance. This became more evident by July 1959 (fig. 5, B) and was thought to be a unilateral polycystic kidney on the right and possible papillary cyst with calculi in the upper and lower poles of the left kidney. Exploration of the right kidney was carried out July 9 and what was thought to be a rather typical polycystic kidney was seen. Several cysts were decompressed. Biopsy of the wall of one cyst, among many examined, revealed dear cell carcinoma. After removal of the stones from the left renal pelvis and several calculi from medullary cystic spaces in this kidney, inulin and P AH clearances having revealed that the left kidney would sustain life, a right nephrectomy was performed on August 26. The gross specimen (fig. 5, C) revealed a large cystic mass in the midportion of the kidney with two small linear areas of clear cell carcinoma. In several papillae and especially at the lower pole there were cystic areas, some containing eakuli. Comment: Vispignani7 found a similar condition of papillary cyst, pyelogenic and polycystic changes in the same kidney. One case reported by Cacchi and Ricci showed an associated benign adenoma.
DISCUSSION
When one compares the rarity of reports of this condition in the American urological literature, viz., 7 cases to the 90 collected cases in the European literature as of October 1957 and at least 35 cases in the Scandinavian literature as of March 1959, 8 it is readily apparent that a digest of a foreign report is worth considering at this point. A thorough and complete delineation of this disease, including renal dearance studies, has been presented by Cacchi at the 30th CongTPSS of the Italian Urological Society, K aples, October 1957. An abstract of Cacchi's report follows: Pathology. The basic gross, macroscopic, and microscopic pathology of this lesion is constant and is described in detail. This histopathology has been confirmed by Powell, Gunther, Rogness and associates, and :\Iulvaney and associates. Powell and Gunther have also noted microlithiasis within the dilated collecting tubules, diverticulum formations coming off the collecting tubules, and in the true cysts. Etiology. A developmental etiology based on McKenna and Karnpmeier's 9 work is favored. It is noteworthy that two sets of siblings with this lesion have been reported. c'rography. Retrograde urography is usually unrevealing while intravenous pyelography dearly demonstrates the lesion. It is not surprising that lithiasis is usually present inasmuch as the lesion demonstrates the ideal situation for stone fornmtion, namely, stagnant urine, cellular debris, and infection. However, other factors must be involved as some cases remain free, or relatively free, of nephrolithiasis over many years while others go on to staghorn formation. Sponge kidney with lithiasis sometimes presents a classic picture of nephrocalcinosis. In advanced cases it is impossible to differentiate the two lesions without prior films. The presence of microlithiasis and frank lithiasis is found more often than realized, viz., 24 of 29 personal cases. Sponge kidney is added to the list of causes of nephrolithiasis. Clinical considerations. There were 77 males and 19 females. Eighty-one patients were between 31 and 60 years of age. Five were between 7 and 14 years of age. In many the first symptoms antedated the x-ray ancl, therefore, the diagnosis by many years. The latent period Lindvall N.: Acta radio!., 51: 193, 1959. McKenn~, C. M. and Kampmeier, 0. F.: ,J. Urol., 32: 37, 1934. 8
9
7
Vispignani: Quoted by Cacchi and Ricci.
SPO,';GJD KfDNEY
of this condition i~ m1wh longer than that of polycrstic renal disease. Sponge kidnc,y patients do not have an unusual inciclem·e of hypert
cmosis, staghom calculi, and repeated seven, t~pisorlcs of pyelonephritis. Sponge kidney \1itit obstruction to tlw urinar:' pathwa>·s because' calculi or peritubnlar fibrosis can C'\"l'1Jtually Yelop into a full blom1 hydrom·phrotic 11·hercin both the mc:dulla and the cork:,; thinned out. These patit nts then assmm: a state of chronic renal insuf!icietJC)" with irn·verni bJrc· azotemia and septicemia. Treatment. treatm<'nt of the infection and, within the reduction of the rnmplicatiou ol" urolithi::;,1,, better the prognosis of spougt' kidnc:y Orn· C',l lt newr speak of cnring sponge kidney; thrn· not a single case 1Yhere the rndiologieal has n·grcsserl OYer the yrars. Tlwrap_v is th· trerltment of complications. Bemus<, of the clrainagr from thcsmall saccnlations, treatment of" infection e:c.:tremcly difficult and lmsatitsfactory rt if: ah, onr experiern'P that in one case thC' film showed rrhttin:ly normal µch·io('.a]yceal terns, while the intravenous pp:logram cle:irh rlelineaterl the lesion. In general, tl1i_,, di,,ea,''<' entity can be likened to c!i,sP:tS<,', c:c.:ccpt that tht' prognosrn is cousiclera bl)· hettn in most cases. 0
C
SUMMARY
Fin' cases of sp