BILATERAL EMPHYSEMATOUS PYELONEPHRITIS AUGUST ZABBO,
M.D.
JAMES E. MONTIE,
M.D.
KATHRYN L. POPOWNIAK, ALLAN J. WEINSTEIN,
M.D.
M.D.
From the Departments of Urology, Hypertension-Nephrology, and Infectious Diseases, Cleveland Clinic Foundation, Cleveland, Ohio
ABSTRACT-Emphysematous pyelonephritis is a severe infection found almost exclusively in diabetics, characterized by the presence of gas within the renal parenchyma. The diagnosis is established radiographically. An additional case is added to the 52 cases reported in the literature; we believe this is the fifth reported case with bilateral emphysematous pyelonephritis. I’ appropriate diagnostic studies demonstrate no evidence of either perinephric abscess or urinary obstruction, intensive medical management should be the initial therapy for this condition. Surgical intervention is necessary in patients without prompt response to medical therapy.
Diabetes mellitus is associated with a higher incidence of a variety of bacterial and fungal infections, and urinary tract infections are more prevalent in diabetic women.’ A rare and particularly severe infection of the urinary tract, emphysematous pyelonephritis, has been reported almost exclusively in diabetics. We describe a patient with bilateral emphysematous pyelonephritis and review the literature. Case Report A seventy-three-year-old black female, insulin-dependent diabetic was admitted to the Cleveland Clinic Foundation Hospital on June 19, 1982, because of a two-week history of malaise, increasing weakness and confusion, fever, nausea, and right upper quadrant abdominal pain. The patient had been hospitalized at another institution two weeks prior for apparent pneumonia. Physical examination revealed a severely ill-appearing patient with labored tachypnea. Her temperature was 38.2 C,
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blood pressure 70/50 mm Hg, and pulse 1001 min. There was slight right flank tenderness, but no mass was palpated. Laboratory values showed hematocrit of 34.6, white blood cell count of 26,700/mm3, platelet count of 107,000/mm3, and elevated prothrombin time. Serum creatinine was 6.2 mgldl. Urinalysis showed marked pyuria and glycosuria. Blood glucose was 426 mg/dl. Urine and blood cultures were positive for Escherichia coli. Initial radiographic examination included a plain chest film which suggested moderate congestive heart failure. Ultrasound scan of the kidneys was negative for obstruction. Admitting diagnosis was pyelonephritis with sepsis causing uncontrolled diabetes. She was treated in the medical intensive care unit with antibiotics, but over the course of the next twenty-four hours her abdominal pain worsened. A plain film of the abdomen revealed gas around the right kidney, and urologic consultation was obtained. On review of the abdominal radiographs, multiple small gas bubbles were
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noted in the area of the right kidney, and right perinephric gas. Also present but obscured by gas in the left colon was a mottled gas pattern overlying the left kidney. Retrograde pyelograms demonstrated no obstruction of either the left or right collecting systems. By the time these films had been obtained gas was also readily apparent in and around the left kidney. A CT scan of the abdomen demonstrated extensive gas in and around the left kidney. The right kidney also was involved, but to a lesser extent. No frank abscess was identified (Fig. 1). Because of the patient’s worsened condition and the presence of perinephric gas thought to indicate abscess, a left flank exploration was performed on June 22, 1982. No perinephric abscess was found; but the kidney was soft and dusky, and a biopsy of renal specimen demonstrated necrotic tissue. Drains were left in the left flank, and the patient was returned to the intensive care unit. She had a stormy postoperative course with evidence of disseminated intravascular coagulopathy, and required hemodialysis for acute oliguric renal failure from June 24, 1982, to July 2, 1982. Gradually the urine output increased, and serum creatinine stabilized at a level of 2.5 to 3.0 mg/dl. During this time persistent purulent drainage from the left flank and intermittent fever were noted. Culture of this drainage showed E. coli and Candida albicans. A renal flow scan (ggmTcDTPA) demonstrated minimal perfusion of the left kidney and fair perfusion of the right kidney. A left nephrectomy was done on July 12, 1982. A grossly necrotic kidney was removed in multiple sections since the tissue was so friable that the kidney could not be removed en masse. Pathologic specimen again showed severe acute pyelonephritis with infarction and necrosis of the kidney. Postoperatively the patient recovered slowly with a gradual decrease in drainage from the left flank. She was discharged on August 7, 1982, and placed on long-term supwith trimethoprimpressive therapy sulfamethoxizole. Follow-up as of March 10, 1983, showed a serum creatinine of 2.5 mgldl, but urine culture again demonstrated E. coli which was treated with oral antibiotics. Comment Spontaneous appearance of gas within the upper urinary tract can be explained by four mechanisms: (1) Atmospheric gas can enter by
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FIGURE 1. CT scan showing both kidneys.
gas in and around
iatrogenic manipulation. (2) Visceral gas can enter by fistula from the digestive or pulmonary system. (3) Glucose within the urinary tract can be fermented by microorganisms to form organic acids, carbon dioxide, and hydrogen. (4) Anaerobic gas-forming organisms, such as clostridia, theoretically could infect the upper urinary tact and produce gas. This has not yet been reported as a spontaneous occurrence, but seen only in postsurgical patients. Spontaneous generation of gas within the upper urinary tract by glucose-fermenting organisms has been reported in isolated cases under various titles. It is difficult to determine from the literature the results of therapy for conditions which are different but reported under the same title. Several authors have suggested rational schema for terminology of gas in the upper urinary tract.2 The following terms and definitions seem most appropriate: 1, Emphysematous pyelonephritis should be reserved for severe necrotizing infection characterized by gas formation within the renal parenchyma. 2. Pneumopyonephrosis is a purulent collection of gas formation limited to the renal collecting system and is associated with ureteral obstruction. 3. Perirenal abscess with gas formation should be used to describe an abscess with gas formation outside the renal capsule but not within the kidney or collecting system. The need for stricter use of these terms is reflected in two recent reports of “emphysematous pyelonephritis” that we have characterized as other types of infection.3,4
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Using the aforementioned definitions we have identified 52 cases of true emphysematous pyelonephritis reported previously. That which many consider to be the first case of emphysematous pyelonephritis, reported by Kelly and MacCallum in 1898,5 we and others2 have believed was pneumopyonephrosis. No radiographs were obtained in this case, and gas was obtained by ureteral catheterization. Of the 53 reported cases, 52 are in diabetics. The sole remaining case, in a polycystic kidney of a patient on long-term hemodialysis, probably represents an abscess within a cyst with gas formation.6 Overall mortality has been 33 per cent. Although some reports describe disproportionate involvement of the left kidney, we have found 26 cases involving the left kidney, 22 involving the right kidney, and our case representing the fifth case of bilateral involvement. Only 17 of the patients were males, whereas 36 (68%) were females. Typically, the clinical presentation of these patients is similar to that of other patients with pyelonephritis ‘: fever, flank pain, nausea, and vomiting are the most common presenting symptoms. Pneumaturia has been reported but is not usually found in this condition. Physical examination may reveal flank or abdominal tenderness, and occasionally a mass will be present. Uncontrolled diabetes, pyuria, leukocytosis, and positive urine and blood cultures are the most frequent laboratory findings. E. coli is the most common pathogen causing this disease, with other enteric pathogens occurring less frequently (Table I). TABLE
I.
Organisms in emphysematous pyelonephritis *
Organism
Emphysematous Pyelonephritis
E. coli Enterobacter Psuedomonas Proteus Klebsiella
40 4 2
3 5
*Also single case reports of Cryptococcus, C. albicans, C. tropicalis, Paracolon bacillus, S. epidermidis.
Diagnosis is established by the clinical and laboratory findings mentioned along with radiographic demonstration of gas within the renal parenchyma. Renal gas is sometimes mistaken for gas within the gastrointestinal tract on initial examinations of the radiographs. In the
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TABLE
II.
Therapy for emphysematous pyelonephritis
Therapy Medical only Nephrectomy Incision and drainage (I&D) I&D followed by nephrectomy Other TOTALS
Patients
Mortality
11 28 7
8 7 3
4
0
3
0 18
-
53
current case and in 2 otherss,Q CT scanning vividly demonstrated gas within and around the renal parenchyma. Evaluation of a patient with these findings should include a search for obstruction. The 10 patients from the literature who had obstruction along with emphysematous pyelonephritis responded well to surgery. Only two deaths occurred in this group. Recommendations for management of emphysematous pyelonephritis have been derived from experiences with scattered case reports. Most recent reviews favor surgical therapylJOJ1 since previous attempts at medical therapy have met with little success (Table II). Those cases in which autopsy was obtained demonstrated both chronic pyelonephritic and diabetic changes in the contralateral kidney, establishing that renal damage was present bilaterally in most cases and renal salvage should be recommended whenever possible. Pathology of the involved kidneys showed acute and chronic inflammation, cortical abscesses, and often infarction of segments or the entire kidney. A kidney can be destroyed by the disease, becoming soft and friable and, at times, unrecognizable at surgery. However, many of the patients treated with medical therapy alone were in such desparate condition at the time of diagnosis that they probably would not have survived immediate emergency surgery. The current case, along with 2 recent cases of emphysematous pyelonephritis in solitary kidneys12J3 and other reports of patients surviving with medical therapy or incision and drainage, demonstrates that the condition can be treated successfully in the proper setting without nephrectomy. We recommend the following management plan for emphysematous pyelonephritis. Once the diagnosis is made the patient should be started on a course of antibiotics to cover enteric bacilli, and stabilized in relation to fluids,
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TABLE
III.
Five cases of bilateral emphysematous
pyelonephritis
Patient’s Series
Age
Sex
Gillies and Flocks, 194114 Harrison and Bailey, 1942r5 Costas, 197216 Kumar and Rao, 1982l’
52 46 54 47
F F F M
Zabbo et al.
73
F
electrolytes, and diabetes. Retrograde pyelograms should be done to exclude urinary obstruction. Abdominal CT scan can identify intra-renal and perinephric gas or an abscess. Abscess or obstruction requires surgical relief. The radiographic finding of perinephric gas does not necessarily imply an abscess. Abscesses were absent in our case and in many others in which perinephric gas was demonstrated clearly. Carbon dioxide and hydrogen, which can diffuse through the body tissues and into the blood stream, are excellent markers for response to therapy in emphysematous pyelonephritis. If the gas is not resolving or the clinical picture not improving with antibiotic therapy, then prompt surgical therapy with nephrectomy or incision and drainage should be done. The renal flow scan may play an important role in identifying those kidneys that would be amenable to salvage with medical management or incision and drainage. In our own case, one kidney was nonperfusing and eventually came to nephrectomy, whereas the other kidney, although markedly involved with renal and perirenal gas, showed adequate perfusion and responded to medical therapy. Using the renal flow scan to make therapeutic decisions in this disease has not been reported previously but seems logical, especially in light of the reported pathology of those kidneys that came to nephrectomy or autopsy demonstrating necrosis and infarction in many cases. Once the acute episode is resolved, long-term suppressive medication is warranted along with periodic surveillance urine cultures. We have reported survival of a patient with bilateral emphysematous pyelonephritis. Four other cases of bilateral emphysematous pyelonephritis have been reported with only one survivor in this group (Table III). In the ab-
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Organism
Therapy
E. E. E. E.
coli coli coli coli; Cryptococcus E. coli
Result
R I&D R nephrectomy Bilateral I&D None
Death Death Survival Death
L I&D, L nephrectomy
Survival
sence of obstructive uropathy or abscess, management of the patient with emphysematous pyelonephritis should be intensive medical therapy of the infection and diabetes initially, followed by prompt surgical intervention if response to medical therapy is inadequate. 9500 Euclid Avenue Cleveland, Ohio 44106 (DR. ZABBO) References 1. Stamey TA: Urinary tract infections in women, in: Pathogenesis and Treatment of Urinary ‘Ract Infections, Baltimore, Williams and Wilkins, 1980. 2. Turman AE, and Rutherford CR: Emphysematous pyelonephritis with perinephric gas, J Urol 105: 165 (1971). 3. Lee EL, Yoon DK, and Kim YK: Emphysematous ovelonenhritis. ibid 118: 916 (1977). -.4. YAumoto R, et al: Emphysematous pyelonephritis: report of two cases and review of the literature, Osaka City Med J 26: 78 (1980). 5. Kelly HA, and MacCallum WG: Pneumaturia, JAMA 31: 375 (1898). 6. Levinson CD, and Weidner FA: Emphysematous pyelonephritis in a polycystic kidney, Urol Radio1 3: 39 (1981). 7. Schainuck LI, Fouty R, and Cutler RE: Emphysematous pyelonephritis: a new case and review of previous observations, Am J Med 44: 134 (1968). 8.- Lautin EM, et al: Emphysematous pyelonephritis: optimal diagnosis and treatment, Urol Radio1 1: 93 (1979). 9. Kim CJ, Woesner ME, Howard TF, and Olson LK: Emphysematous pyelonephritis demonstrated by computed tomography, AJR 132: 287 (1979). 10. Spagnola AM: Emphysematous pyelonephritis: a report of two cases, Am J Med 64: 40 (1978). 11. Freiha FS, Messing EM, and Gross DM: Emphysematous pyelonephritis, ICE Urol 18: 9 (1979). __ 12. dodec CJ, Cax AS, and Berkseth R: Emphysematous nvelonenhritis in a solitarv kidnev. I Urol 124: 119 11980). _I 13. DePauw Ap, and Ross G: ‘Emphysematous pyelonkphritis in a solitary kidney, ibid 125: 734 (1981). 14. Gillies CL, and Flocks R: Spontaneous renal and perirenal emphysema: report of a case in a diabetic from Escherichia coli infection, AJR 46: 173 (1941). 15. Harrison JH, and Bailey OT: The significance of necrotizing pyelonephritis in diabetes mellitus, JAMA 118: 15 (1942). 16. Costas S: Renal and perirenal emphysema, Br J Urol 44: 311 (1972). 17. Kumar D, and Rao BR: Bilateral emphysematous pyelonephritis, Urology 20: 96 (1982).
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