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To the Editor: First reported by E. L. Keyes in 1882,¹ emphysematous cystitis is a rare complication of urinary tract infection, characterized by spontaneous gas formation in the urinary bladder. Patients with cystic fibrosis (CF) have several risk factors that predispose them to urolithiasis, such as hyperoxaluria, hypercalciuria, hyperuricosuria, and increased urinary saturation of calcium oxalate.^2-3 Diabetes due to the pancreatic insufficiency common in CF may further increase risk of urinary infections and formation of renal stones.^2-3 However, to our knowledge occurrence of emphysematous cystitis has not been reported in patients with cystic fibrosis. We describe a case of emphysematous cystitis and renal stones in a patient with cystic fibrosis.

Report of a Case
A 23-year-old white woman was hospitalized for nausea and colicky lower abdominal and back pain lasting 2 days. While hospitalized she passed 6 small stones in her urine. Her past medical history included CF with pancreatic insufficiency, insulin-dependent diabetes, and recurrent pulmonary infections. On physical examination she had bilateral coarse inspiratory and expiratory crackles and hypogastric tenderness. Her 24-hour urinary oxalate excretion was 59.9 mg (reference range, 0-40 mg). Her urine culture grew more than 100 000 colonies of Klebsiella pneumoniae. Analysis of the passed stones revealed calcium oxalate calculus. An intravenous pyelogram showed bilateral renal pelvic calculi. A computed tomographic scan showed the presence of multiple gas pockets within the wall of the urinary bladder (Figure), diagnostic of emphysematous cystitis. The patient was treated with intravenous fluids, analgesics, and intravenous ampicillin/sulbactam for 10 days. Her symptoms gradually resolved over 1 week and she was discharged home.

View larger version (36K): [in this window] [in a new window] Figure. Contrast-Enhanced Computed Tomographic Scan of the Pelvis Showing Emphysematous Cystitis Arrowheads indicate gas within the wall of the urinary bladder.

Comment
Pancreatic insufficiency and fatty acid malabsorption may create an excess of fatty acids in the gut that bind calcium, leading to hyperoxaluria. Low serum vitamin D levels and increased uric acid levels can predispose to hypercalciuria and hyperuricosuria. Hyperoxaluria, hypercalciuria, hyperuricosuria, and increased urinary saturation of calcium oxalate in patients with CF may increase the risk for developing renal calculi.^2-3 The colonic microflora Oxalobactor formigenes regulates the absorption of oxalic acid through the intestine and thereby its plasma concentration. Frequent use of oral antibacterial agents may prevent the bacterial scavenging of oxalate and aggravate hyperoxaluria.^3-4

Emphysematous cystitis is the formation of intraluminal and intramural gas in the urinary bladder and may be seen in patients with acute urinary tract infection and with diabetes mellitus, neurogenic bladder diseases, and/or chronic urinary infection.^5-6 The pathogenesis of emphysematous cystitis remains unclear. Escherichia coli is the most common implicated pathogen; others include Clostridium perfringens; Aerobacter aerogenes; various streptococci, proteus, and nocardia species; Candida albicans; and very rarely K pneumoniae, as seen in this patient.^5-6 The mechanism of gas formation is believed to be fermentation of glucose to carbon dioxide by gas-forming organisms in the bladder. Identification of risk factors, treatment of pancreatic insufficiency, tight glycemic control, treatment and prevention of urinary infection and urolithiasis, and relief of urinary obstruction are critical in the prevention and management of emphysematous cystitis.^6-7 The bladder should be kept at rest by continuous catheter drainage, particularly in patients with urinary retention and neurogenic bladder.⁷ Surgical intervention is indicated only in the presence of obstruction, stone, or other anatomic abnormality.^6-7 While emphysematous cystitis may be fatal, the majority of patients show clinical and radiological resolution within 4 days.⁷

Daya Upadhyay, MD
upadhyayd{at}pol.net
Division of Pulmonary and Critical Care Medicine
Stanford University Medical Center
Stanford, Calif

Manu Jain, MD
Division of Pulmonary and Critical Care Medicine
Northwestern University Feinberg School of Medicine
Chicago, Ill

1. Keyes EL. Pneumuria. Med News. 1882;41:675. 2. Chidekel A, Dolan T Jr. Cystic fibrosis and calcium oxalate nephrolithiasis. Yale J Biol Med. 1996;69:317-321. PUBMED 3. Gibney EM, Goldfarb DS. The association of nephrolithiasis with cystic fibrosis. Am J Kidney Dis. 2003;42:1-11. 4. Sidhu H, Hoppe B, Hess A, et al. Absence of Oxalobacter formigenes in cystic fibrosis patients: a risk factor for hyperoxaluria. Lancet. 1998;352:1026-1029. FULL TEXT | PUBMED 5. Katz DS, Aksoy E, Cunha BA. Clostridium perfringens emphysematous cystitis. Urology. 1993;41:458-460. PUBMED 6. Bartkowski DP, Lanesky JR. Emphysematous prostatitis and cystitis. J Urol. 1988;139:1063-1065. PUBMED 7. Quint HJ, Darch GW, Rappaport WD, Hoffmann CJ. Emphysematous cystitis: a review of the spectrum of disease. J Urol. 1992;147:134-137. ISI | PUBMED

Letters Section Editor: Robert M. Golub, MD, Senior Editor.

JAMA. 2004;292:1953-1954.

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