Emphysematous Pyelonephritis

(EPN)

Prepared by
Hardi Tariq Hamma
Emphysematous Pyelonephritis (EPN)
• Emphysematous pyelonephritis is a urologic emergency characterized
by an acute necrotizing parenchymal and perirenal infection caused
by gas Forming uropathogens.
• Its presentation is similar to that of acute pyelonephritis, but EPN
often has a fulminating course, and can be fatal if not recognized and
treated promptly.
Etiology
• Emphysematous pyelonephritis (EPN) is typically caused by enteric
gram-negative facultative anaerobes.
• Escherichia coli is most common cause.
• Klebsiella species .
• Proteus,Pseudomonas, and Streptococcus species .
• Mixed organisms are observed in 10% of patients.
• Rarely, fungi (eg, Aspergillus fumigatus, Candida species) and
protozoa (Entamoeba histolytica) have been isolated in patients with
EPN.
Pathophysiology
• EPN is a severe infection of the renal parenchyma that causes gas
accumulation in the tissues.
• Because the condition usually occurs in patients with diabetes, it has
been postulated that the high tissue glucose levels provide the
substrate for microorganisms such as E. coli, which are able to
produce carbon dioxide by the fermentation of sugar.
• factors that predispose to EPN in persons with diabetes may include
uncontrolled diabetes, high levels of glycosylated hemoglobin, and
impaired host immune mechanisms.
Pathophysiology
• Fermentation of glucose with carbon dioxide production by the
pathogens has been proposed as the cause of gas in the tissues.
• In addition to diabetes, many patients have urinary tract
obstruction associated with urinary calculi or papillary necrosis and
significant renal functional impairment.
• Schainuck et al proposed that fermentation products from tissue
necrosis produced carbon dioxide .
• Diabetic microangiopathy may contribute to the slow transport of
catabolic products and may lead to accumulation of gas.
Epidemiology
• About 80-90% of EPN DM
• Women are affected more often than men.
• Obstruction is demonstrated in approximately 25% of the cases
• Rare cases have been reported in persons who do not have diabetes,
with renal failure and immunosuppression as contributing factors.
• The left kidney is affected more commonly than the right. Bilateral
cases have also been reported.
Clinical Presentation
* Almost all patients display the classic triad of fever, vomiting, and
flank pain.
• Altered sensorium, shock.
• Acute renal impairment.
• Crepitus over the flank area may occur in advanced cases of EPN.
• Pneumaturia is uncommon unless emphysematous cystitis or
collecting system affected.
• Comorbidities include alcoholism, malnourishment, renal calculi,
and diabetic ketoacidosis are present.
Emphysematous Pyelonephritis (EPN) Workup
• Urinalysis - Pyuria, infected urine.
• Complete blood cell count with differential - Leukocytosis with a left
shift, thrombocytopenia.
• Renal function tests - Elevated creatinine level.
• Blood cultures – Positive.
• Urine culture-positive.
Emphysematous Pyelonephritis (EPN) Workup
Imaging studies used for diagnosis of emphysematous pyelonephritis
include the following:
• Kidney, ureter, bladder (KUB) radiographs
• Renal ultrasonography
• Computed tomography (CT) - The definitive technique
• CT is the imaging procedure of choice in defining the extent of the
emphysematous process and guiding management.
Emphysematous
pyelonephritis. Kidneys,
ureter, and bladder
imaging showing gas over
the region of the right
kidney. White arrows
outline the area. The faint
outline of a staghorn
calculus can be seen in
the right kidney.
Abdominal radiography
showing
*abnormal gas
shadows in the left
renal fossa (white
arrowheads)
*ureter (white arrows)
*radiopaque stone
(black arrow).
Emphysematous Pyelonephritis (EPN) Workup
• Ultrasonography
• Renal ultrasonograms often reveal high echogenic areas with dirty
shadowing. Hydronephrosis and perinephric fluid may also be seen.
• strong focal echoes suggesting the presence of intraparenchymal gas.
Emphysematous
pyelonephritis.
Renal sonogram
showing
hyperechoic
shadows
suggestive of gas
along the lower
pole of the kidney.
Emphysematous Pyelonephritis (EPN) Workup
• Computed tomography
Patterns of gas seen on CT in patients with EPN may include the following:
• Streaky, streaky and mottled, or streaky and bubbly.
• Gas may be rimlike or crescent-shaped in the perinephric area
• Gas may be seen in the renal vein or inferior vena cava.
• Gas may be seen along the psoas muscle.
• Perinephric abscess may lead to significant gas accumulation in the
perinephric space.
• A stone may be seen in the collecting system.
CT scan showing
right renal and
perinephric gas
in a patient with
emphysematous
pyelonephritis
CT scan showing
gas in both
kidneys and the
inferior vena cava
in a patient with
bilateral
emphysematous
pyelonephritis.
 Air collections
in the left
renal
parenchyma,
perinephric
space, and
ureter
(arrow).
Emphysematous Pyelonephritis (EPN) Workup

Radiologic Classification
Over time, several groups of
investigators have proposed
classification systems for EPN
Radiologic Classification

• In 1970, Langston and Pfister described 3 main radiographic

patterns in EPN, as follows :
• Diffuse mottling of the renal parenchyma

• Bubbly renal parenchyma surrounded by crescent-shaped gas in the

perinephric space

• Extension of gas through the Gerota fascia

Radiologic Classification

• In 1984, Michaeli et al suggested 3 stages of EPN, as follows :

• Stage I - Gas within the renal parenchyma or the perinephric tissue.

• Stage II - Presence of gas in the kidney and its surroundings.

• Stage III - Extension of gas through Gerota fascia or bilateral EPN

Radiologic Classification
In 1996, Wan et al described 2 distinct types of EPN, as follows ;
• Type I - Characterized by parenchymal destruction, with streaky or
mottled parenchymal gas and an absence of fluid collection; has a
fulminant course and high risk of mortality.
• Type II - Characterized by renal or perirenal fluid collection, with
bubbly gas collection in the perinephric space or in the collecting
system and a mortality rate of 18%.
• according to Wan et al, the compromised immune state of the host
leads to fulminant and dry-type EPN, which is fatal.
Radiologic Classification
• In 2000, Huang and Tseng modified the staging proposed by
Michaeli et al, as follows :
• Class 1 - Gas confined to the collecting system.
• Class 2 - Gas confined to the renal parenchyma alone.
• Class 3A - Perinephric extension of gas or abscess.
• Class 3B - Extension of gas beyond the Gerota fascia.
• Class 4 - Bilateral EPN or EPN in a solitary kidney.
Management
• Patients with emphysematous pyelonephritis (EPN) are extremely ill
and need resuscitative measures in the intensive care unit.
• Oxygen.
• intravenous (IV) fluids, and correction of acid-base imbalances, along
with glycemic control.
• Systolic blood pressure should be maintained above 100 mm Hg, with
fluid or inotropic support.
• Surgical intervention should be performed only after stabilization of
the cardiorespiratory status.
Management
• Prompt initiation of empiric IV antibiotic therapy is critical.
• The regimen chosen should be broad spectrum, primarily target
gram-negative bacteria.
• Most patients are septic, and fluid resuscitation, glucose and
electrolyte management, and broad-spectrum antimicrobial
therapy are essential.
• Ureteral obstruction, if present, is alleviated by a percutaneous
nephrostomy tube or a stent.
Management
Conservative treatment using percutaneous drainage with antibiotics
is indicated as follows:
❖Patients with compromised renal function.
❖Early cases associated with gas in the collecting system alone and
patient is in otherwise in stable condition.
❖Class 1 and class 2 EPN.
❖Class 3 and class 4 EPN - In the presence of fewer than 2 risk factors
(eg, thrombocytopenia, elevated serum creatinine levels, altered
sensorium, shock).
Management
The use of nephrectomy is indicated as follows:

❖No access to percutaneous drainage or internal stenting (after

patient is stabilized).
❖Gas in the renal parenchyma or "dry-type" EPN .
❖Class 3 and class 4 EPN - In the presence of two or more risk factors
(eg, thrombocytopenia, elevated serum creatinine, altered
sensorium, shock).
Algorithm for the
management of
emphysematous
pyelonephritis.
prognosis
• Huang and Tseng reported an overall EPN mortality rate of 19%.
• They also reported significant treatment success rates with
percutaneous drainage and antibiotics (66%) and with nephrectomy
(90%).
• Factors associated with a poor prognosis in patients with EPN include
altered level of consciousness, multiple organ failure,
hyperglycemia, and leukocytosis.
• An absence of fluid in CT images or the presence of streaky or
mottled gas with or without bubbly and loculated gas appears to be
associated with rapid destruction of renal parenchyma and a 50% to
60% mortality rate.
prognosis
❖The presence of renal or perirenal fluid, the presence of bubbly or
loculated gas or gas in the collecting system, and the absence of
streaky or mottled gas patterns are associated with a less than 20%
mortality rate.
Reference
❖-Oxford hand book of urology.
4th edition.

❖-Campbell-Walsh-Wein Urology. 12th edition

❖Smith & Tanagho~s
GENERAL UROLOGY 18th edition
❖-MEDSCAPE
Thank you