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Emphysematous Pyelonephritis with Perinephric Gas
Vol. 105, Feb. Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
EMPHYSE:.VIATOUS PYELONEPHRITIS WITH PERINEPHRIC GAS ALFRED E. TURMAN
AND
CHARLES RUTHERFORD
From the Division of Urology, Medical College of Alabama, Birmingham, Alabama
Emphysematous pyelonephritis is a rare but frequently lethal urinary tract infection marked by systemic toxicity, necrosis of renal parenchyma and formation of gas in and around the kidney. After 2 recent patients with emphysematous pyelonephritis and perinephric gas stimulated a review of this topic, it was quickly apparent that considerable confusion exists about terms and about the best therapy for this type of urinary tract infection. Furthermore, certain surgical treatment recommendations which have been reported recently seemed unjustified by the weight of the available evidence. 1 • 2 Surprisingly, non-surgical treatment tends to be recommended in the surgical literature while surgical treatment tends to be recommended in the medical literature. The terminology of gas associated with the urinary tract is herein reviewed, 2 cases of emphysematous pyelonephritis are presented and certain concepts of emphysematous pyelonephritis and its treatment will be presented. CASE REPORTS
Case 1. Unit No. E09-62-25, an obese 57-yearold woman with long-standing hypertension and diabetes had left flank pain, nausea, vomiting and diarrhea 3 days prior to admission to another hospital. The woman was stuporous and blood urea nitrogen (BUN) was 50 mg. per cent. After urine culture showed Klebsiella the patient was treated with cephaloridine but became more stuporous and oliguric and was transferred to the University of Alabama Hospital. The woman responded only to painful stimuli. Blood pressure was 150/50, pulse 110 and respiration 30. A 12 by 6 inch smooth, tender mass was noted in the left flank. The BUN was 137 mg. per cent, creatinine 7 .8 mg. per cent, white blood count (WBC) 30,700 packed cell
Accepted for publication March 23, 1970. Read at annual meeting of Southeastern Section, American Urological Association, Hollywood Beach, Florida, March 30-April 3, 1969. 1 Stokes, J. B., Jr.: Emphysematous pyelonephritis. J. Urol., 96: 6, 1966. 2 Schultz, E. H., Jr. and Klorfein, E. H.: Emphysematous pyelonephritis. J. Urol., 87: 762, 1962. 165
volume (PCV) and urinalysis showed gross pyuria. Abdominal radiography revealed an unusual collection of gas in the left upper quadrant. Retrograde pyelography showed medial deviation of the ureter and perinephric gas (fig. 1). The flank was drained under local anesthesia and a large amount of gas and pus was removed. The patient did poorly, became anuric, required hemodialysis, but eventually began to improve from the renal standpoint. Her hospital course was complicated by many lifethreatening cardiac, pulmonary and vascular complications and she remained in a catabolic state even though the BUN had fallen to 20 mg. per cent. Three months following hospitalization left nephrectomy was done. All but the calyces of the lower pole of the kidney had sloughed out. Microscopic examination showed chronic pyelonephritis, arteriolonephrosclerosis and glomerulosclerosis. After a stormy convalescence, she was finally discharged from the hospital. Case 2. Unit No. E18-32-52, a 51-year-old woman had been well until a month prior to hospitalization when mild diabetes and urinary tract infection were diagnosed. She was admitted to the hospital with a history of vomiting and progressive lethargy for 24 hours prior to admission and, on physical examination, she had no recordable blood pressure, pulse was 180 per minute, respiration 33 per minute and temperature 98.6F. The patient was unresponsive to painful stimuli and examination failed to reveal a probable cause for illness. Laboratory studies showed WBC 21,000, blood glucose 681 mg. per cent, BUN 52 mg. per cent, arterial blood pH 7.33, sodium 120 mEq. per L, chloride 80 mEq. per L and carbon dioxide 5 mEq. per L. Urinalysis showed 4 plus glucose, innumerable WBCs and gram-negative rods. She responded to intensive treatment for diabetic acidosis and sepsis and when the blood pressure was 80/60 an excretory urogram (IVP) revealed that the left kidney was filled with gas and a rim of gas was seen just outside the kidney. A retrograde pyelogram was made (fig. 2). At operation there was
166
TURMAN AND RUTHERFORD
The 7 cases reported as emphysematous pyelonephritis and in which the gas formation was confined to the renal collecting system or parenchyma are listed in table 2. All patients
Fra. 1. Case 1. Retrograde pyelogram shows medial deviation of left ureter, emphysematous pyelonephritis and perinephric gas. inflammation of the perinephric fat, gas about the lower pole a,1d necrosis of the upper and lower poles (fig. 3). Following nephrectomy the patient recovered promptly. Pyelonephritis, cortical abscesses and parenchymal necrosis were demonstrated microscopically (fig. 4).
FIG. 2. Case 2. Left retrograde pyelogram shows emphysematous pyelonephritis and perinephric gas. Right kidney is ectopic.
REVIEW OF LITERATURE
Cases of 25 patients with renal gas have been reported as emphysematous pyelonephritis. Records of these 25 patients plus the 2 cases reported herein were reviewed. The 20 cases in which there was radiological and/or surgical demonstration of gas in the parenchyma and perirenal space are listed in table 1. In most cases gross pus in the cortex and perirenal area and extensive necrosis of renal tissue were prominent. Ten patients (50 per cent) died of the disease: 4 patients did not undergo an operation while 6 patients had incision and drainage or nephrectomy. Incision and drainage and/or nephrectomy were done in 9 of the 10 survivors. The single patient who survived and had not had an operation died about a year later of gram-negative sepsis, after having been treated for significant urinary tract infections. 2
FIG. 3. Case 2. Kidney shows necrosis of upper and lower poles and abscesses in middle segment.
167
EMPHYSE:VIATOUS PYELONEPHRITIS WITH PERINEPHRIC GAS
FIG. 4. Case 2. Microscopic sections of upper pole show loss of tubular organization and disintegration of individual cells. TABLE
Authors
Side
Diabetes
Tissue Necrosis Prominent*
1
Pus
Treatment
Result
Gillies and Flocks, 194!3
Both
Yes
N.R.
Alexander, 1941 4 Harrison and Bailey, 1942 5 Welch and Prather, 1949 6 Levy and Schwinger, 1953 7 Braman and Cross, 1956 8 Olson, 1957 9 Porter and Wright, 195110 Clifford and Katz, 1962 11 Clifford and Katz, 1962 11 Schultz and Klorfein, 1962 2
Left Both Left Right Left Left Left Right Right Right
Yes Yes Yes Yes Yes Yes Yes Yes Yes
Yes Yes Yes Yes Yes Yes Yes Yes N.R. Not op.
Yes N.R. Not op.
Incision and drainage None None
Died Died Died Died Lived Lived Lived Died Died Lived
Harrmv and Sloane, 1963J 2 Stokes, 1966' Klein and associates, 196613 Klein and associates, 196613
Left Right Right Right
Yes Yes Yes Yes
No Yes Yes Yes
N.R. Yes Yes N.R.
N ephrectomy Incision and drainage None N ephrectomy
Lived Died Died Lived
Yu, 1966 14
Right
Yes
Yes
Lived
Sun, 196816 Schainuck and associates, 196816 Present case, 1968
Right Left
Yes Yes
Yes Yes
N.R. Yes
Incision and drainage, nephrectomy None N ephrectomy
Left
Yes
Yes
Yes
Present case, 1968
Left
Yes
Yes
Yes
Yes
Pos. culture Yes Yes Yes Yes Yes Yes
Rt. incision and drainage Nephrectomy Rt. nephrostomy N ephrectomy None Incision and drainage Incision and drainage
Yes
Nephrectomy
Incision and drainage, nephrectomy N ephrectomy
Comment
Died
Also had GI fistula
Had recurrent UTI and died in sepsis 1 year later
Died 8 days postop., liver failure
Died Lived Lived Lived
' N.R.-not recorded.
previously reported to have had emphysematous pyelonephritis without diabetes are found here.
Only 5 of the 7 original reports were available to us but, of these five, only Plaggemeyer described
3 Gillies, C. L. and Flocks, R.: Spontaneous renal and perirenal emphysema. Amer. J. Roentgenol., 46: 173, 1941. 4 Alexander, J. C.: Pneumopyonephrosis m diabetes mellitus. J. Urol., 45: 570, 1941.
5 Harrison, J. H. and Bailey, 0. T.: Significance of necrotizing pyelonephritis in diabetes mellitus. J.A.M.A., 118: 15, 1942. 6 Welch, N. M. and Prather, G. C.: Pneumo-
168
TURMAN AND RUTHERFORD TABLE 2 Dia- Tissue Necrosis Pus Prominent*
Author
Side
Kelly and MacCallum, 1898,; Randall, 1927"
Left
No
N.R.
Yes
N ephrostomy
Lived
Left
No
Shell, yes
Yes
Lived Lived
Treatment
betes
Result
Mathe and de la Pena y Pineda, 19331 9 Nogueira, 193520 Olsson, 1939 21
Left
No
Shell, yes
Yes
Incision and drainage Nephrectomy
Left Left
No Yes
Yes
Yes
None None
Lived Died
Jensen, 193922
Right
No
N ephrectomy
Lived
Plaggemeyer and associates, 1947 23
Both
Yes
None
Lived
N.R.
Infected urine
Comment Probable hydronephrosis Probable duplication and hydronephrosis Probable duplication and hydronephrosis Cited in Schultz 2 Papillary necrosis, gas in pelvis, probably Cited by Schultz. 2 Probable hydronephrosis ?Obstruction
* N. R.-not recorded, shell-shell of kidney at operation.
gas inside or outside the renal parenchyma. 23 Indeed, judging from the illustrations and text, we did not believe these cases represent emphysematous pyelonephritis but rather urinary tract infection with gas in the collecting system. Four cases probably represent infected hydronephrosis11-19, 22 and Olsson's case had papillary necrosis. 21 nephrosis: a complication of necrotizing pyelonephritis. J. Urol., 61: 712, 1949. 7 Levy, A.H. and Schwinger, H. N.: Gas containing perinephric abscess. Radiology, 60: 720, 1953. 8 Braman, R. and Cross, R.R., Jr.: Perinephric abscess producing a pneumonephrogram. J. Urol., 75: 194, 1956. 9 Olson, K. L.: Renal Escherichia coli infection associated with diabetes mellitus. Amer. J. Roentgenol., 78: 719, 1957. 10 Porter, R. and Wright, F. W.: Intracapsular perinephric gas forming infection in a patient with diabetic coma. Brit. J. Radio!., 34: 201, 1961. 11 Clifford, N. J. and Katz, I.: Subcutaneous emphysema complicating renal infection by gasforming coliform bacteria. New Engl. J. Med., 266: 437, 1962. 12 Harrow, B. R. and Sloane, J. A.: Ureteritis emphysematosa; spontaneous ureteral pneumogram; renal and perirenal emphysema. J. Urol., 89: 43, 1963. 13 Klein, D. E., Mahoney, S. A., Youngen, R. and Schneider, D. H.: Renal emphysema. J. Urol., 95: 625, 1966. 14 Yu, S. F.: Spontaneous renal and perirenal emphysema. Brit. J. Radio!., 39: 466, 1966. 15 Sun, N. C.: Nonclostridial emphysematous nephritis, ureteritis, cystitis and adrenalitis due to Escherichia coli. Southern Med. J., 61: 400, 1968. 16 Schainuck, L. I., Fouty, R. and Cutler, R. E.: Emphysematous pyelonephritis. A new case and review of previous observations. Amer. J. Med., 44: 134, 1968. 17 Kelly, H. A. and MacCallum, W. G.: Pneumaturia. J.A.M.A., 31: 375, 1898. 18 Randall, A.: Pneumopyonephrosis with pneumaturia. Trans. Amer. Ass. Genito-Urin. Surg., 20: 261, 1927.
These latter cases are not similar to those described in table 1 and we do not consider these cases to be emphysematous pyelonephritis. DISCUSSION
Etiology. Table 1 shows that contrary to previous opinions there was no predilection for the left side; only one example of bilateral disease has been reported and all patients were diabetic. In addition to dramatic radiographs and profound toxicity of the patient, gross perirenal pus and extensive necrosis are constant findings. Nonetheless, the etiology of this disease is obscure. It has been suggested that the high tissue glucose levels found in diabetics represent a favorable situation for the production of carbon dioxide. 1 • 2· 11 • 16 Schainuck and associates recently pointed out that this explanation does not account for the extreme rarity of emphysematous pyelonephritis as compared to the frequency of coliform infections in diabetic patients. 16 They stated that 2 features uniformly associated with emphysematous pyelonephritis are a severe and necrotizing infection and impaired tissue and vascular re19 Mathe, C. P. and de la Pena y Pineda, E.: Pneumonephrosis: report of case. Urol. & Cu tan. Rev., 37: 732, 1933. 20 Nogueira, A.: Spontane Gasfullung der Harnwege. Ztschr. f. Urol., 29: 275, 1935. 21 Olsson, 0.: Spontanes Gaspyelogramm. Acta Radio!., 20: 578, 1939. 22 Jensen, J.: Tilfaedle af Pneumopyonephrose. Nord. Med., 4: 3129, 1939. 23 Plaggemeyer, H. W., Weitman, C. G., Sorenson, M. C. and Schmaltz, J. D.: Pneumopyonaephrosis of B. proteus origin. Grace Hosp. Bull., 25: 78, 1947.
EMPHYSEMATOUS PYELONEPHRITIS WITH PERINEPHRIC GAS TABLE
3. Terminologies for urinary pneumatosis* Mural Gas
Ureter
Kidney
Calyces and pelvis
Bladder
Intraluminal Gas
Emphysematous ureteritis Ureteritis emphysematosa Ureteral emphysema Emphysematous pyelonephritis Pyelonephritis emphysematosa Renal emphysema Pneumonephrogram Pneumonephritis Ernphysematous pyelitis Pyelitis emphysematosa Calyceal or pelvic emphysema Emphysematous cystitis Cystitis emphysematosa Vesical emphysema
Gas ureterogram
Pneumo-ureterogram
Gas pyelogram Pneumopyelogram Pneumopyoneph-
rosis Gas cystogram Pneumocystogram
* Reprinted with permission of The Williams & Wilkins Company from Harrow and Sloane. 12 TABLE
Gas Confined to Collecting System Kidney
xx
Pelvis
Intrapelvic gas
Ureter
Bladder
Intraureteral
I Intracystic gas gas
4
Gas in Tissues
Emphysematous pyelonephritis Emphysematons pyelitis Emphysematous ureteritis Emphysematous cystitis
Gas Outside Urinary Tract
Perirenal gas
xx Periureteral gas Pericystic gas
sponse caused by local factors such as obstruction or a systemic condition such as diabetes. They postulated that this impaired response on the part of the host allows organisms which have the capability of producing carbon dioxide in vitro, to use necrotic tissue as substrate to generate gas in vivo. Perhaps edema secondary to a severe inflammatory reaction may play a part in producing the necrosis. Terminology. There are 3 circumstances m which gas may be found in the urinary tract: 1) atmospheric gas introduced by cystoscopy or trauma, 2) gas introduced by a fistula with a the action of hollow viscus and 3) gas produced bacteria. The third situation is the one with which this report deals and in which confusion of terminology and treatment exists.
169
In 1963 Harrow discussed the problem of terminology, presented various terms that have been used (table 3) and suggested that entomologists decide which terms should be applied to conditions in which gas is associated with the urinary tract. 12 Tables 1 and 2 show that critical distinction among gas confined to the collecting system, gas confined to the parenchyma and gas outside the parenchyma has not been made. The need for this distinction is suggested by differences in mortality. This lack of clarification has adversely affected treatment recommendations. Contrary to previously expressed views, table 1 shows that when gas is demonstrated both in the renal parenchyma and in the perinephric space that perinephric abscess is almost surely present. Table 4 represents a logical classification of gas in and around the kidney and urinary tract. The use of uniform terms ,.,.-ill result in a clearer understanding and better treatment of the disease process. The scheme presented in table 4 can be applied to the entire urinary tract and is compatible with more established terms such as ureteritis cystica and perinephric abscess. We believe the literature supports the concept that morbidity and mortality rates rise sharply as the gas-forming infectious process extends from the collecting system to the parenchyma and finally to the perirenal area. The use of appropriate terms will best define the disease to be considered. Treatment. Schultz and Klorfein did not clearly distinguish between gas formation limited to the renal collecting system, the renal parenchyma and the perinephric tissues. 2 Since their patient with emphysematous pyelonephritis and perinephric gas and one other patient without perirenal gas23 had survived on medical management these authors recommend that the basic treatment for emphysematous pyelonephritis should be non-operative. A short time later Harrow quc::;tioned the wisdom of their recommendations but did not document his position. 12 In 1966 Stokes reported on a patient who had emphysematous pyelonephritis with perinephric gas and, following incision and drainage of a large perinephric abscess, did poorly and died. 1 He then concluded that "conventional medical therapy, after radiological diagnosis, is best in both the diabetic and non-diabetic". The adverse effects of these recommendations are shown perhaps in the 1968 report of Sun in which a patient with emphysematous pyelonephritis with perinephric gas formation was
170
TURMAN AND RUTHERFORD
treated with antibiotics, fluids and steroids before dying in sepsis. 15 The postmortem examination showed a large amount of perinephric gas and cortical abscesses. In that report, Schultz and Klorfein, 2 Klein and associates13 and Stokes1 were cited as recommending "non-surgical" treatment except to "relieve urinary tract obstruction". In their review of this problem in 1968 Schainuck and associates emphasized that emphysematous pyelonephritis should be regarded as a complicated, severely necrotizing urinary tract infection and not as a distinct clinical entity. 16 They believe that patients with urological obstruction and diabetes are predisposed to this condition only insofar as their basic condition predisposes them to severe renal infection. Schainuck has suggested that, since most gases diffuse readily, the continuing presence of gas, in spite of medical treatment, is graphic evidence that this treatment is ineffective. 16 We agree with Schainuck that the principles of management should be those applied to any severe pyelonephritis with or without abscess formation. Massive necrosis alone would suggest that an operation is required but, since the diagnosis of emphysematous pyelonephritis with perinephric gas is associated with perinephric abscess, appropriate surgical therapy is required. Since preparation of this manuscript 2 addi-
tional patients who died in spite of vigorous nonsurgical therapy have been described. 24 This experience and the evaluation of other reports led the authors to recommend surgical therapy if at all possible. SUMMARY
Emphysematous pyelonephritis is a fulminating renal infection usually marked by sepsis, renal necrosis, abscesses and gas formation in the renal and perirenal tissues. Two additional cases of emphysematous pyelonephritis have been presented and previous reports have been reviewed. Several cases previously reported as emphysematous pyelonephritis were found to be relatively simple pyonephrosis with gas confined to the collecting system. A simple scheme for referring to gas as it relates to the urinary tract has been proposed. After cases which probably do not represent emphysematous pyelonephritis were eliminated, surgical treatment has been shown to be the treatment of choice and should be strongly considered in all cases of emphysematous pyelonephritis. Dr. H. Walker Brown assisted in the management of these patients. 24 Banks, D. E., Jr., Persky, L. and Mahoney, S. A.: Renal emphysema. J. Urol., 102: 390, 1969.
THE JOURNAL OF UROLOGY
Copyright© 1971 by The Williams & Wilkins Co.
EMPHYSE:.VIATOUS PYELONEPHRITIS WITH PERINEPHRIC GAS ALFRED E. TURMAN
AND
CHARLES RUTHERFORD
From the Division of Urology, Medical College of Alabama, Birmingham, Alabama
Emphysematous pyelonephritis is a rare but frequently lethal urinary tract infection marked by systemic toxicity, necrosis of renal parenchyma and formation of gas in and around the kidney. After 2 recent patients with emphysematous pyelonephritis and perinephric gas stimulated a review of this topic, it was quickly apparent that considerable confusion exists about terms and about the best therapy for this type of urinary tract infection. Furthermore, certain surgical treatment recommendations which have been reported recently seemed unjustified by the weight of the available evidence. 1 • 2 Surprisingly, non-surgical treatment tends to be recommended in the surgical literature while surgical treatment tends to be recommended in the medical literature. The terminology of gas associated with the urinary tract is herein reviewed, 2 cases of emphysematous pyelonephritis are presented and certain concepts of emphysematous pyelonephritis and its treatment will be presented. CASE REPORTS
Case 1. Unit No. E09-62-25, an obese 57-yearold woman with long-standing hypertension and diabetes had left flank pain, nausea, vomiting and diarrhea 3 days prior to admission to another hospital. The woman was stuporous and blood urea nitrogen (BUN) was 50 mg. per cent. After urine culture showed Klebsiella the patient was treated with cephaloridine but became more stuporous and oliguric and was transferred to the University of Alabama Hospital. The woman responded only to painful stimuli. Blood pressure was 150/50, pulse 110 and respiration 30. A 12 by 6 inch smooth, tender mass was noted in the left flank. The BUN was 137 mg. per cent, creatinine 7 .8 mg. per cent, white blood count (WBC) 30,700 packed cell
Accepted for publication March 23, 1970. Read at annual meeting of Southeastern Section, American Urological Association, Hollywood Beach, Florida, March 30-April 3, 1969. 1 Stokes, J. B., Jr.: Emphysematous pyelonephritis. J. Urol., 96: 6, 1966. 2 Schultz, E. H., Jr. and Klorfein, E. H.: Emphysematous pyelonephritis. J. Urol., 87: 762, 1962. 165
volume (PCV) and urinalysis showed gross pyuria. Abdominal radiography revealed an unusual collection of gas in the left upper quadrant. Retrograde pyelography showed medial deviation of the ureter and perinephric gas (fig. 1). The flank was drained under local anesthesia and a large amount of gas and pus was removed. The patient did poorly, became anuric, required hemodialysis, but eventually began to improve from the renal standpoint. Her hospital course was complicated by many lifethreatening cardiac, pulmonary and vascular complications and she remained in a catabolic state even though the BUN had fallen to 20 mg. per cent. Three months following hospitalization left nephrectomy was done. All but the calyces of the lower pole of the kidney had sloughed out. Microscopic examination showed chronic pyelonephritis, arteriolonephrosclerosis and glomerulosclerosis. After a stormy convalescence, she was finally discharged from the hospital. Case 2. Unit No. E18-32-52, a 51-year-old woman had been well until a month prior to hospitalization when mild diabetes and urinary tract infection were diagnosed. She was admitted to the hospital with a history of vomiting and progressive lethargy for 24 hours prior to admission and, on physical examination, she had no recordable blood pressure, pulse was 180 per minute, respiration 33 per minute and temperature 98.6F. The patient was unresponsive to painful stimuli and examination failed to reveal a probable cause for illness. Laboratory studies showed WBC 21,000, blood glucose 681 mg. per cent, BUN 52 mg. per cent, arterial blood pH 7.33, sodium 120 mEq. per L, chloride 80 mEq. per L and carbon dioxide 5 mEq. per L. Urinalysis showed 4 plus glucose, innumerable WBCs and gram-negative rods. She responded to intensive treatment for diabetic acidosis and sepsis and when the blood pressure was 80/60 an excretory urogram (IVP) revealed that the left kidney was filled with gas and a rim of gas was seen just outside the kidney. A retrograde pyelogram was made (fig. 2). At operation there was
166
TURMAN AND RUTHERFORD
The 7 cases reported as emphysematous pyelonephritis and in which the gas formation was confined to the renal collecting system or parenchyma are listed in table 2. All patients
Fra. 1. Case 1. Retrograde pyelogram shows medial deviation of left ureter, emphysematous pyelonephritis and perinephric gas. inflammation of the perinephric fat, gas about the lower pole a,1d necrosis of the upper and lower poles (fig. 3). Following nephrectomy the patient recovered promptly. Pyelonephritis, cortical abscesses and parenchymal necrosis were demonstrated microscopically (fig. 4).
FIG. 2. Case 2. Left retrograde pyelogram shows emphysematous pyelonephritis and perinephric gas. Right kidney is ectopic.
REVIEW OF LITERATURE
Cases of 25 patients with renal gas have been reported as emphysematous pyelonephritis. Records of these 25 patients plus the 2 cases reported herein were reviewed. The 20 cases in which there was radiological and/or surgical demonstration of gas in the parenchyma and perirenal space are listed in table 1. In most cases gross pus in the cortex and perirenal area and extensive necrosis of renal tissue were prominent. Ten patients (50 per cent) died of the disease: 4 patients did not undergo an operation while 6 patients had incision and drainage or nephrectomy. Incision and drainage and/or nephrectomy were done in 9 of the 10 survivors. The single patient who survived and had not had an operation died about a year later of gram-negative sepsis, after having been treated for significant urinary tract infections. 2
FIG. 3. Case 2. Kidney shows necrosis of upper and lower poles and abscesses in middle segment.
167
EMPHYSE:VIATOUS PYELONEPHRITIS WITH PERINEPHRIC GAS
FIG. 4. Case 2. Microscopic sections of upper pole show loss of tubular organization and disintegration of individual cells. TABLE
Authors
Side
Diabetes
Tissue Necrosis Prominent*
1
Pus
Treatment
Result
Gillies and Flocks, 194!3
Both
Yes
N.R.
Alexander, 1941 4 Harrison and Bailey, 1942 5 Welch and Prather, 1949 6 Levy and Schwinger, 1953 7 Braman and Cross, 1956 8 Olson, 1957 9 Porter and Wright, 195110 Clifford and Katz, 1962 11 Clifford and Katz, 1962 11 Schultz and Klorfein, 1962 2
Left Both Left Right Left Left Left Right Right Right
Yes Yes Yes Yes Yes Yes Yes Yes Yes
Yes Yes Yes Yes Yes Yes Yes Yes N.R. Not op.
Yes N.R. Not op.
Incision and drainage None None
Died Died Died Died Lived Lived Lived Died Died Lived
Harrmv and Sloane, 1963J 2 Stokes, 1966' Klein and associates, 196613 Klein and associates, 196613
Left Right Right Right
Yes Yes Yes Yes
No Yes Yes Yes
N.R. Yes Yes N.R.
N ephrectomy Incision and drainage None N ephrectomy
Lived Died Died Lived
Yu, 1966 14
Right
Yes
Yes
Lived
Sun, 196816 Schainuck and associates, 196816 Present case, 1968
Right Left
Yes Yes
Yes Yes
N.R. Yes
Incision and drainage, nephrectomy None N ephrectomy
Left
Yes
Yes
Yes
Present case, 1968
Left
Yes
Yes
Yes
Yes
Pos. culture Yes Yes Yes Yes Yes Yes
Rt. incision and drainage Nephrectomy Rt. nephrostomy N ephrectomy None Incision and drainage Incision and drainage
Yes
Nephrectomy
Incision and drainage, nephrectomy N ephrectomy
Comment
Died
Also had GI fistula
Had recurrent UTI and died in sepsis 1 year later
Died 8 days postop., liver failure
Died Lived Lived Lived
' N.R.-not recorded.
previously reported to have had emphysematous pyelonephritis without diabetes are found here.
Only 5 of the 7 original reports were available to us but, of these five, only Plaggemeyer described
3 Gillies, C. L. and Flocks, R.: Spontaneous renal and perirenal emphysema. Amer. J. Roentgenol., 46: 173, 1941. 4 Alexander, J. C.: Pneumopyonephrosis m diabetes mellitus. J. Urol., 45: 570, 1941.
5 Harrison, J. H. and Bailey, 0. T.: Significance of necrotizing pyelonephritis in diabetes mellitus. J.A.M.A., 118: 15, 1942. 6 Welch, N. M. and Prather, G. C.: Pneumo-
168
TURMAN AND RUTHERFORD TABLE 2 Dia- Tissue Necrosis Pus Prominent*
Author
Side
Kelly and MacCallum, 1898,; Randall, 1927"
Left
No
N.R.
Yes
N ephrostomy
Lived
Left
No
Shell, yes
Yes
Lived Lived
Treatment
betes
Result
Mathe and de la Pena y Pineda, 19331 9 Nogueira, 193520 Olsson, 1939 21
Left
No
Shell, yes
Yes
Incision and drainage Nephrectomy
Left Left
No Yes
Yes
Yes
None None
Lived Died
Jensen, 193922
Right
No
N ephrectomy
Lived
Plaggemeyer and associates, 1947 23
Both
Yes
None
Lived
N.R.
Infected urine
Comment Probable hydronephrosis Probable duplication and hydronephrosis Probable duplication and hydronephrosis Cited in Schultz 2 Papillary necrosis, gas in pelvis, probably Cited by Schultz. 2 Probable hydronephrosis ?Obstruction
* N. R.-not recorded, shell-shell of kidney at operation.
gas inside or outside the renal parenchyma. 23 Indeed, judging from the illustrations and text, we did not believe these cases represent emphysematous pyelonephritis but rather urinary tract infection with gas in the collecting system. Four cases probably represent infected hydronephrosis11-19, 22 and Olsson's case had papillary necrosis. 21 nephrosis: a complication of necrotizing pyelonephritis. J. Urol., 61: 712, 1949. 7 Levy, A.H. and Schwinger, H. N.: Gas containing perinephric abscess. Radiology, 60: 720, 1953. 8 Braman, R. and Cross, R.R., Jr.: Perinephric abscess producing a pneumonephrogram. J. Urol., 75: 194, 1956. 9 Olson, K. L.: Renal Escherichia coli infection associated with diabetes mellitus. Amer. J. Roentgenol., 78: 719, 1957. 10 Porter, R. and Wright, F. W.: Intracapsular perinephric gas forming infection in a patient with diabetic coma. Brit. J. Radio!., 34: 201, 1961. 11 Clifford, N. J. and Katz, I.: Subcutaneous emphysema complicating renal infection by gasforming coliform bacteria. New Engl. J. Med., 266: 437, 1962. 12 Harrow, B. R. and Sloane, J. A.: Ureteritis emphysematosa; spontaneous ureteral pneumogram; renal and perirenal emphysema. J. Urol., 89: 43, 1963. 13 Klein, D. E., Mahoney, S. A., Youngen, R. and Schneider, D. H.: Renal emphysema. J. Urol., 95: 625, 1966. 14 Yu, S. F.: Spontaneous renal and perirenal emphysema. Brit. J. Radio!., 39: 466, 1966. 15 Sun, N. C.: Nonclostridial emphysematous nephritis, ureteritis, cystitis and adrenalitis due to Escherichia coli. Southern Med. J., 61: 400, 1968. 16 Schainuck, L. I., Fouty, R. and Cutler, R. E.: Emphysematous pyelonephritis. A new case and review of previous observations. Amer. J. Med., 44: 134, 1968. 17 Kelly, H. A. and MacCallum, W. G.: Pneumaturia. J.A.M.A., 31: 375, 1898. 18 Randall, A.: Pneumopyonephrosis with pneumaturia. Trans. Amer. Ass. Genito-Urin. Surg., 20: 261, 1927.
These latter cases are not similar to those described in table 1 and we do not consider these cases to be emphysematous pyelonephritis. DISCUSSION
Etiology. Table 1 shows that contrary to previous opinions there was no predilection for the left side; only one example of bilateral disease has been reported and all patients were diabetic. In addition to dramatic radiographs and profound toxicity of the patient, gross perirenal pus and extensive necrosis are constant findings. Nonetheless, the etiology of this disease is obscure. It has been suggested that the high tissue glucose levels found in diabetics represent a favorable situation for the production of carbon dioxide. 1 • 2· 11 • 16 Schainuck and associates recently pointed out that this explanation does not account for the extreme rarity of emphysematous pyelonephritis as compared to the frequency of coliform infections in diabetic patients. 16 They stated that 2 features uniformly associated with emphysematous pyelonephritis are a severe and necrotizing infection and impaired tissue and vascular re19 Mathe, C. P. and de la Pena y Pineda, E.: Pneumonephrosis: report of case. Urol. & Cu tan. Rev., 37: 732, 1933. 20 Nogueira, A.: Spontane Gasfullung der Harnwege. Ztschr. f. Urol., 29: 275, 1935. 21 Olsson, 0.: Spontanes Gaspyelogramm. Acta Radio!., 20: 578, 1939. 22 Jensen, J.: Tilfaedle af Pneumopyonephrose. Nord. Med., 4: 3129, 1939. 23 Plaggemeyer, H. W., Weitman, C. G., Sorenson, M. C. and Schmaltz, J. D.: Pneumopyonaephrosis of B. proteus origin. Grace Hosp. Bull., 25: 78, 1947.
EMPHYSEMATOUS PYELONEPHRITIS WITH PERINEPHRIC GAS TABLE
3. Terminologies for urinary pneumatosis* Mural Gas
Ureter
Kidney
Calyces and pelvis
Bladder
Intraluminal Gas
Emphysematous ureteritis Ureteritis emphysematosa Ureteral emphysema Emphysematous pyelonephritis Pyelonephritis emphysematosa Renal emphysema Pneumonephrogram Pneumonephritis Ernphysematous pyelitis Pyelitis emphysematosa Calyceal or pelvic emphysema Emphysematous cystitis Cystitis emphysematosa Vesical emphysema
Gas ureterogram
Pneumo-ureterogram
Gas pyelogram Pneumopyelogram Pneumopyoneph-
rosis Gas cystogram Pneumocystogram
* Reprinted with permission of The Williams & Wilkins Company from Harrow and Sloane. 12 TABLE
Gas Confined to Collecting System Kidney
xx
Pelvis
Intrapelvic gas
Ureter
Bladder
Intraureteral
I Intracystic gas gas
4
Gas in Tissues
Emphysematous pyelonephritis Emphysematons pyelitis Emphysematous ureteritis Emphysematous cystitis
Gas Outside Urinary Tract
Perirenal gas
xx Periureteral gas Pericystic gas
sponse caused by local factors such as obstruction or a systemic condition such as diabetes. They postulated that this impaired response on the part of the host allows organisms which have the capability of producing carbon dioxide in vitro, to use necrotic tissue as substrate to generate gas in vivo. Perhaps edema secondary to a severe inflammatory reaction may play a part in producing the necrosis. Terminology. There are 3 circumstances m which gas may be found in the urinary tract: 1) atmospheric gas introduced by cystoscopy or trauma, 2) gas introduced by a fistula with a the action of hollow viscus and 3) gas produced bacteria. The third situation is the one with which this report deals and in which confusion of terminology and treatment exists.
169
In 1963 Harrow discussed the problem of terminology, presented various terms that have been used (table 3) and suggested that entomologists decide which terms should be applied to conditions in which gas is associated with the urinary tract. 12 Tables 1 and 2 show that critical distinction among gas confined to the collecting system, gas confined to the parenchyma and gas outside the parenchyma has not been made. The need for this distinction is suggested by differences in mortality. This lack of clarification has adversely affected treatment recommendations. Contrary to previously expressed views, table 1 shows that when gas is demonstrated both in the renal parenchyma and in the perinephric space that perinephric abscess is almost surely present. Table 4 represents a logical classification of gas in and around the kidney and urinary tract. The use of uniform terms ,.,.-ill result in a clearer understanding and better treatment of the disease process. The scheme presented in table 4 can be applied to the entire urinary tract and is compatible with more established terms such as ureteritis cystica and perinephric abscess. We believe the literature supports the concept that morbidity and mortality rates rise sharply as the gas-forming infectious process extends from the collecting system to the parenchyma and finally to the perirenal area. The use of appropriate terms will best define the disease to be considered. Treatment. Schultz and Klorfein did not clearly distinguish between gas formation limited to the renal collecting system, the renal parenchyma and the perinephric tissues. 2 Since their patient with emphysematous pyelonephritis and perinephric gas and one other patient without perirenal gas23 had survived on medical management these authors recommend that the basic treatment for emphysematous pyelonephritis should be non-operative. A short time later Harrow quc::;tioned the wisdom of their recommendations but did not document his position. 12 In 1966 Stokes reported on a patient who had emphysematous pyelonephritis with perinephric gas and, following incision and drainage of a large perinephric abscess, did poorly and died. 1 He then concluded that "conventional medical therapy, after radiological diagnosis, is best in both the diabetic and non-diabetic". The adverse effects of these recommendations are shown perhaps in the 1968 report of Sun in which a patient with emphysematous pyelonephritis with perinephric gas formation was
170
TURMAN AND RUTHERFORD
treated with antibiotics, fluids and steroids before dying in sepsis. 15 The postmortem examination showed a large amount of perinephric gas and cortical abscesses. In that report, Schultz and Klorfein, 2 Klein and associates13 and Stokes1 were cited as recommending "non-surgical" treatment except to "relieve urinary tract obstruction". In their review of this problem in 1968 Schainuck and associates emphasized that emphysematous pyelonephritis should be regarded as a complicated, severely necrotizing urinary tract infection and not as a distinct clinical entity. 16 They believe that patients with urological obstruction and diabetes are predisposed to this condition only insofar as their basic condition predisposes them to severe renal infection. Schainuck has suggested that, since most gases diffuse readily, the continuing presence of gas, in spite of medical treatment, is graphic evidence that this treatment is ineffective. 16 We agree with Schainuck that the principles of management should be those applied to any severe pyelonephritis with or without abscess formation. Massive necrosis alone would suggest that an operation is required but, since the diagnosis of emphysematous pyelonephritis with perinephric gas is associated with perinephric abscess, appropriate surgical therapy is required. Since preparation of this manuscript 2 addi-
tional patients who died in spite of vigorous nonsurgical therapy have been described. 24 This experience and the evaluation of other reports led the authors to recommend surgical therapy if at all possible. SUMMARY
Emphysematous pyelonephritis is a fulminating renal infection usually marked by sepsis, renal necrosis, abscesses and gas formation in the renal and perirenal tissues. Two additional cases of emphysematous pyelonephritis have been presented and previous reports have been reviewed. Several cases previously reported as emphysematous pyelonephritis were found to be relatively simple pyonephrosis with gas confined to the collecting system. A simple scheme for referring to gas as it relates to the urinary tract has been proposed. After cases which probably do not represent emphysematous pyelonephritis were eliminated, surgical treatment has been shown to be the treatment of choice and should be strongly considered in all cases of emphysematous pyelonephritis. Dr. H. Walker Brown assisted in the management of these patients. 24 Banks, D. E., Jr., Persky, L. and Mahoney, S. A.: Renal emphysema. J. Urol., 102: 390, 1969.