Cystitis cystica causing obstructive uropathy andemphysematous pyelonephritis in diabetes: An uncommon clinico-radiologic case report.

Abstract: Cystitis cystica and cystic glandularis are benign chronic reactive lesions of urinary bladder. Cystitis cystica is an uncommon cause of obstructive uropathy. Emphysematous pyelonephritis (EPN) is an acute, severe, life threatening, necrotizing infection of renal and perirenal tissue. It is commonly seen in diabetes, obstructive uropathy and characterized by gas formation. We report here a 55 years diabetic female with cystitis cystica causing obstructive uropathy and emphysematous pyelonephritis. After conservative treatment, resolution of cystitis cystica and emphysematous pyelonephritiswas seen on follow up ultrasound. Mild focal calyctasis was the only complication of EPN found in our case.

Key words:Cystitis cystica, Emphysematous pyelonephritis, Obstructive uropathy.

Introduction:Cystitis cystica and cystic glandularis are chronic reactive lesions of the urinary bladder which occurs in the setting of chronic infection.1Emphysematous pyelonephritis (EPN) is an acute severe necrotizing infection of renal and perirenal tissue characterized by gas formation. The commonest predisposing factors for EPN are diabetes mellitus and obstructive uropathy.2It is a life threatening infection with high mortality rate upto 40% when treated with antibiotics alone.3 Stone, stricture and carcinoma involving urinary tract, benign prostatic hyperplasia, neurogenic bladder are the common causes of obstructive uropathy. Lesion of cystitis cystica at ureterovesical junction causing obstructive uropathy is uncommon.1,4Gupta et al. had reported synchronous existence of EPN and emphysematous cystitis but coexistence of EPN with cystitis cystica is not reported till date.5 We report here an uncommon case of obstructive uropathy due to cystitis cystica with coexistence of EPN managed conservatively with ultrasound follow up.

Case History:A 55 year old Indian woman presented to the urology department with complaint of dysuria, frequency of micturition since 1 month and high grade fever with chills and rigor, left sided abdominal pain since 2 days. Past medical history was significant for diabetes mellitus of 15 years duration. She was taking oral hypoglycemic agents regularly. On clinical examination she was alert, orientedand febrile with temperature of 390C. Tachycardia was present. Cardiovascular, respiratory and neurological system examination revealed no significant abnormality. On abdominal examination renal angle tenderness was positive on left side.

Her laboratory examination showed deranged renal function (serum creatinine-3.9 mg%, blood urea 112mg %), hyperglycemia (RBS- 335 mg %), leukocytosis (11500/mm3) with neutrophilia (89%). Serum electrolytes & platelet count were within normal limits. Urine microscopy showed numerous pus cells and coccobacilli. Urine culture grew E-coli.

The abdominal radiograph was unremarkable. Abdominal ultrasonography revealed echogenic foci in the calyctial system of upper pole and renal pelvis ofthe left kidney giving dirty acoustic shadowing suggestive of airFigure 1A. Polypoidal mass lesion of approximate size 3.7 x 1.5cm was seen in left ureterovesicle junction causing minimal left sided hydronephrosis and hydroureterFigure 2A,Figure 1A. Dopplerrevealed no vascular pedicle within the massFigure 3. Cystoscopyrevealed a polypoidal, transparent, cystic lesion in left ureteric orifice. Biopsy was taken which revealed predominant cystitis cystica with small component of cystic glandularis. Typical cystic spaces were seen in the von Brunn nestslined with urothelial or differentiated mucous cells. No evidence of anaplastic/dysplastic changes seen. Due to renal failure noncontrast CT abdomen was done which confirmed the presence of gas in the upper pole of left kidneyFigure 4. Perinephric and periureteric fat stranding was also present.

Initially patient was put on injectable piperacillin/tazobactum (4.5 gm BD),metronidazole (500mg TDS) and insulin. Percutaneous drainage was done. After seven days injecteble antibiotics were replaced with oral prulifloxacin (600mg OD) and continued till period of one month. Antibiotics were given according to urine culture sensitivity. Patient was discharged and treatment given thereafter on OPD follow up basis according to urine culture sensitivity. Follow up ultrasound showed progressive reduction in renal gasFigure 1B. Progressive reduction of bladder lesion also noted on subsequent ultrasonographyFigure 2B. The complete resolution of bladder lesion with absence of left hydronephrosis and hydroureter was observed at 4 monthFigure 2C. Mild focal calyctasis at upper pole was only complication seenFigure 1C.

Discussion:Cystitis cystica and cystic glandularis are uncommon chronic reactive lesions in the urinary bladder. Cystitis cystica was first reported by Morgagni, but detailed description was provided by Richmond and Robb.1 It can occur at any age group. Infection, irritation and inflammation to carcinogen had been described as causative factors.1 Bapat et al had showed cystitis cystica as polypoidal lesion on ultrasound mimicking like neoplasm.4 Wong-You-Choung et al had reported it as nodular filling defect on intravenous urography.6Microscopically cystitis cystica shows cystic space within von Brunn nest lined by urothelial cells whereas inner lining of columnar or cuboidal epithelium is seen in cystic glandularis.4

Bapat et al and Zahrani et al had reported a case of cystitis cystica causing obstruction of ureterovesicle junction.4,1 Bapat et al had treated four out of six cystitis cystica patients successfully with long term oral antibiotics and strongly recommended the conservative management. Transurethral resection was indicated for those who had failed conservative management, increasing lesion size on follow up and obstructive lesions. They had also recommended close surveillance of patients with mucous secretion, intestinal metaplasia, pelvic lipomatosis as malignant transformation is reported in these lesions.4Its relation with malignancy is still a matter of debate.

Kelly and MacCullum had reported the first case of pneumaturia in 1898. It was Schultz and Klorfein who termed the word emphysematous pyelonephritis to describe the gas within renal parenchyma.7These patients present with fever, flank pain, hyperglycemia, and electrolyte acid-base disturbances.8The mean age of presentation is 55 years and sex ratio is M: F -6:1. Higher susceptibility for urinary tract infection in females is a cause for higher incidence of EPN in females.7 Left kidney is more commonly involved than right kidney.8 E-coli (70%) andKlebsila pneumoconae (29%) are the most commonly involved pathogens and mixed organisms seen in 10% cases.7 Gases like nitrogen, hydrogen, carbon dioxide, oxygen are produced by fermentation of sugar within the urine due to bacterial action.2

EPN occurs commonly in diabetic patients (90%). High tissue glucose level (favoring rapid growth and catabolism), poor tissue perfusion (due to atherosclerosis of renal arteries) and impaired immune response are the responsible factors for predisposition of EPN in diabetic patients.7 Obstruction of the urinary tract is another predisposing factor for EPNcan be found in nondiabetic (>90%) and diabetic (50%) patients commonly due to stone or tumor.9 In our case the cause of obstruction was cystitis cystica.

Sensitivity of ultrasound andcomputed tomography (CT) for air in EPN is 80% and 100% respectively.2Air on ultrasonography is seen as echogenic foci giving dirty shadowing in nondependent position.10 Huang et al had described the CT classification of EPN depending on anatomical location of gas on CTTable 1.11Class 1 and Class 2 EPN could be managed with percuteneous drainage and antibiotics. Risk factors like acute renal failure, thrombocytopenia, stupor/coma and shock are important in management of Class 3 and Class 4 EPN. In the presence of ≤2 above risk factors, percutaneous drainage and antibiotic could be used whereas in the presence of three or more risk factors, nephrectomy gives better result.2

Our patient had Class 1 EPN and obstructive uropathy due to cystitis cystica managed by percutaneous drainage, antibiotics with regular ultrasound follow up. Our patient also had combined two predisposing factors viz. diabetes, ureteric obstruction. Chronic cystitis in our diabetic patient might leads to development of cystitis cystica which in turns causes obstructive uropathy and again adds in predisposition for EPN. Our study had revealed mild focal calyctasis as a complication of conservatively treated EPN. Stricture of the major calyx is likely to be cause of this complication. She is on regular follow up and she does not have any recurrence.

To summarize we had reported an uncommon case of obstructive uropathy due to cystitis cystica leading to EPN in a diabetic woman managed conservatively with ultrasound follow up. Follow up is important in conservative management of diabetic patient and regular check up is important to prevent such complications.

References:

  1. Zaharani AB, Pandyan GVS. An unusual case of obstructive uropathy: Cystitis cystica with ureteritis cystica. Indian J Surg 2005 ;67:210-2.
  2. Vollans SR, Sehjal R, Forster JA, Rogawski KM. Emphysematous pyelonephritis in type II diabetes: A case report of an undiagnosed ureteric colic. Cases Journal 2008;1:192.
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  9. Prkacin I, Novak B, Skegro D, Mrzljak A, Coric S, Tomic M et al. Emphysematous pyelonephritis in a patient with impaired glucose tolerance. Diabetologia Croatica 2001;30(3):97-100.
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Table:

Table 1: CT classification of EPN

Class / Description
Class 1 / Gas confined to the collecting system.
Class 2 / Gas confined to the renal parenchyma alone.
Class 3a / Perinephric gas extension of gas or abscess.
Class 3b / Extension of gas beyond gerota fascia.
Class 4 / Bilateral EPN or unilateral EPN in solitary kidney.

Figure legends;

  1. Figure 1: A) Ultrasound image of left kidney showing echogenic foci (denoted by arrow) within the left upper calyx and in renal pelvis giving dirty acoustic shadowing with minimal left hydronephrosis. B) Follow up renal ultrasound image after 2 weeks showing significant reduction in renal gas but hydronephrosis was present. C) Follow up renal ultrasound image after 4 month showing mild focal calyctasis (denoted by arrow) at upper pole of left kidney.
  2. Figure 2: A) Urinary bladder ultrasound image showing hypoechoic mass lesion(denoted by arrow) at left ureterovesicle junction.B) Follow up urinary bladder ultrasound image after 6 week showing reduction in size of mass lesion (denoted by arrow). C) Follow up urinary bladder ultrasound image after 4 months showing complete absence of bladder lesion.
  3. Figure 3: Doppler ultrasound of mass showing no vascular pedicle within the mass.
  4. Figure 4: Noncontrast CT image showing air foci (denoted by arrow) within the upper pole of left kidney in calyctial region. Bulky left kidney with perirenal fat stranding is also present.