Emphysematous pyelonephritis presenting with coexistent Pneumatosis intestinalis

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1 International Journal of advances in health sciences (IJHS) ISSN Vol2, Issue1, 2015, pp Case Report Emphysematous pyelonephritis presenting with coexistent Pneumatosis intestinalis 1* Bharati Pandya, 1 Ravinder Narang and 2 Sushil Kale. 1 Department of Surgery, MGIMS, Sevagram, Wardha, India. 2 Department of Radiology, MGIMS, Sevagram. Wardha, India. * bharati_pandya@rediffmail.com [Received-24/12/2014, Accepted-17/01/2015] ABSTRACT Emphysematous pyelonephritis is a rare, life threatening, necrotizing infection of the renal parenchyma and perirenal tissues. It is characterized by gas formation in the kidney and its surrounding structures. Pneumatosis intestinalis is defined as gas in the bowel wall and is diagnosed radiologically on abdominal radiographs or computed tomography scans. Its etiology varies from benign conditions to fulminant gastrointestinal diseases. We report a case of emphysematous pyelonephritis presenting with fulminant colitis and having pneumatosis intestinalis, along with a brief review of literature. The patient, a diabetic male, presented with primary gastrointestinal symptoms and had rapid deterioration in his condition despite treatment. Radiological investigations diagnosed emphysematous pyelonephritis in addition to pneumatosis intestinalis. This is a rare clinical combination and due to the initial nonspecific symptomatology, may cause delay in the diagnosis of both potentially lifethreatening diseases. Key words: Pneumatosis intestinalis, Pneumocystoides intestinalis, Emphysematous pyelonephritis. INTRODUCTION Emphysematous pyelonephritis (EPN) is a rare, severe, gas-forming infection of renal parenchyma and its surrounding tissues. In extensive EPN risk factors are thrombocytopenia, acute renal function impairment, disturbance of consciousness and shock [1]. Those with less than two risk factors are successfully treated using drainage procedures combined with antibiotic treatment; patients with two or more risk factors have a significantly higher morbidity and may require subsequent nephrectomy. Pneumatosis intestinalis (PI) is an unrelated entity, not so far reported with EPN. It occurs in primary and secondary forms. Primary, benign form is uncommon, incidentally detected and is also called pneumatosis cystoides intestinalis. Secondary form is associated with bowel ischemia, obstructive and necrotic gastrointestinal disease, chronic obstructive pulmonary disease, connective tissue disorders, celiac disease, leukemia, amyloidosis, and acquired immunodeficiency syndrome; and also in association with organ transplantation, steroid use, and chemotherapy. We describe a diabetic patient who presented with fulminant colitis and rapidly

2 deteriorated after admission, despite treatment. His radiological investigations revealed coexistence of emphysematous pyelonephritis with pneumatosis intestinalis affecting the colon. Patient was successfully managed with treatment aimed at EPN with perinephric drainage and double J stenting. Appropriate antibiotics and adequate blood sugar control took care of both clinical entities. CASE PRESENTATION A 66 year old, diabetic male, presented with 3 day history of pain in abdomen, loose stools, vomiting and fever. Abdominal pain was diffuse to start with and later got localized to the right iliac fossa. He had severe back ache and loose stools 6-8 times a day associated with hematochezia. He had past history of ischemic heart disease. On initial examination he had fever and tachycardia, no tachypnoea or hypotension. Pallor was present. Abdomen was distended. On palpation, gaurding and rebound tenderness were present, more in the right iliac fossa. His initial investigations revealed mild anaemia (9.0 gm%), leukocytosis, normal platelet counts and a creatinine of 2.2 mg/dl. Blood sugar levels were high and ketone bodies tested negative. Abdominal and chest radiographs were essentially normal. Emergency sonography showed gas filled bowel loops and marked probe tenderness in right iliac fossa but failed to localize the right kidney. Patient was kept nil orally with intravenous hydration and antibiotics. Parenteral insulin was started. Transient improvement was seen the next day, but fever spikes and diarrhoea persisted. On the third day of admission, patient deteriorated suddenly with increased abdominal distension, chest pain, tachycardia, tachypnoea and hypotension and was shifted to ICU. His counts, CRP and serum creatinine had increased. Serum Amylase and lipase were normal. Chest X-ray and tests for myocardial ischaemia were negative. Abdomen was tense, tender with guarding and rigidity in the right iliac fossa and the right flank. Provisional diagnosis kept were pyonephrosis in an ectopic kidney or an appendicular pathology or bowel ischaemia. X-rays were repeated. They showed right sided EPN along with abnormal colonic gas pattern. Class 3b EPN was immediately confirmed on plain CT scan of abdomen. CT also revealed right colonic macrovesicular pneumocystosis with white, enhanced mucosal thickening suggestive of severe colitis. In view of radiological findings, an emergency intervention with placement of DJ stent and drainage of perinephric collection was done. On cystoscopy, gas bubbles were seen exuding from the right ureteric orifice. Foul, dirty, blackishbrown pus with lots of gas was released from the perinephric space. Urine and pus sent for culture showed growth of E.coli and Proteus mirabilis sensitive to Piperacillin. Post operatively patient settled down rapidly with stabilization of vital signs and improvement in blood parameters. Fever, diarrhea and hematochezia subsequently abated. He was discharged on the 10 th day after removal of the perinephric drain but with DJ stent in place. Follow up X-rays showed minimal air in a normal sized kidney. Bharati Pandya, et al. 16

3 thickened colonic wall. White mucosal thickening of colon is suggestive of severe colitis. DISCUSSION Fig. 1 and 2: Plain X-ray KUB and Non-contrast CT scan showing EPN class 3b and abnormal bowel gas pattern in the right colon. Fig 3 and 4: Non-contrast CT scan showing gas in the renal pelvis, parenchyma, peri-renal and para-renal space. Macro-vesicular gas pattern is seen in the Kelly and Mac Callum reported the first case of emphysematous pyelonephritis (EPN) in 1898 [2]. Terms like renal emphysema, pneumonephritis and emphysematous pyelonephritis were used for this gas-forming disease. Schultz and Klorfein [2, 5] in 1962 suggested emphysematous pyelonephritis as the preferred designation, as it stresses the relation between acute infectious process and gas formation. EPN is defined as a disease characterized by gas formation in the renal parenchyma and its surrounding structures. More than 90% of cases occur in diabetics with poor glycemic control.[1,2] Other predisposing factors include urinary tract obstruction, polycystic kidneys, end stage renal disease and immunosuppression [1]. Mixed acid fermentation of glucose by Enterobacteriaceae leads to gas formation [3]. Its diagnosis is established radiologically. Escherichia coli and Klebsiella pneumoniae are the most common organisms isolated from urine culture [2]. Accepted classification of EPN is; class 1: gas confined to the collecting system; class 2: gas confined to renal parenchyma; class 3a: extension of gas to perinephric space; class 3b: extension of gas to pararenal space; class 4: bilateral or solitary kidney with EPN.[1,2] Thrombocytopenia, shock, altered sensorium and acute renal function impairment are considered as poor prognostic factors[1,5]. The clinical manifestations of EPN are similar to classical features of upper urinary tract infections. According to Huang and Tseng [1,5] fever is encountered in 79% of the patients, abdominal or back pain in 71%, nausea and vomiting in 17%, lethargy and confusion in 19%, dyspnoea in 13% and shock in 29%. Laboratory reports show elevated glycosylated hemoglobin in 72%, leukocytosis in 67%, thrombocytopenia in 46% and pyuria in 79% [1]. Diarrhoea and hematochezia are not reported in patients with Bharati Pandya, et al. 17

4 EPN. Ultrasound is insensitive in detecting renal gas [1], Non-contrast CT scan is the diagnostic method of choice. It shows presence of gas, the extent of infection and obstruction if present [1, 3]. Management of EPN has evolved from aggressive surgical intervention to conservative management. Antibiotic treatment alone has 40% mortality and is not recommended [1]. Conservative treatment with parenteral antibiotics, percutaneous drainage and DJ stenting is recommended as the first line treatment [1, 3]. If these fail an emergency nephrectomy is indicated. Pneumatosis intestinalis (PI) also referred to as pneumatosis cystoides intestinalis, pneumatosis coli, and intestinal emphysema, is defined as the presence of extraluminal bowel gas that is confined within the bowel wall. It is an uncommon pathology, first described in 1730 [6]. It is characterized by the presence of gas within the intestinal wall, usually in the mucosal and submucosal layers of the antimesenteric border [7]. Primary PI is a benign idiopathic condition, while secondary PI can be associated with various underlying conditions, including necrotizing enterocolitis, bowel ischemia, mechanical trauma, inflammatory or autoimmune bowel diseases, intestinal neoplasias, bowel infection, obstructive pulmonary disease, immunosuppressive drugs and connective tissue diseases [7,8,9].. Microvesicular gas collections, defined as mm cysts or bubbles within the lamina propria, are predominantly associated with primary (benign) pneumatosis intestinalis (known as Pneumatosis cystoides intestinalis). Linear or curvilinear gas collections seen parallel to the bowel wall are found in secondary pneumatosis and represent an ominous sign. It is an alarming radiological finding that usually prompts an emergency surgical consultation for concerns of bowel ischemia and impending bowel rupture [7]. Patients with colonic PI most commonly present with symptoms of diarrhea (56%), hematochezia (50%), abdominal discomfort (32%), and abdominal distension (28%) [8]. Complications are present in 3% of PI patients [8] and include pneumoperitoneum, bowel obstruction, volvulus, intussusception, and hemorrhage. Due to the risk of these complications, suspected PI patients need evaluation for possible surgery. Management of mild-to-moderate PI is treatment of underlying disease along with antibiotics, oxygen therapy, and elemental diet. [8]. In the pathogenesis of EPN, four factors have been implicated. They are, gas-forming bacteria, high tissue glucose level (favoring rapid bacterial growth), impaired tissue perfusion (diabetic nephropathy leads to compromised regional oxygen delivery in the kidney, resulting in tissue ischemia and necrosis with nitrogen being released during tissue necrosis) and a defective immune response. [5]. On the other hand, in the pathophysiology of PI two main theories have been proposed. The mechanical theory advocates that increased luminal pressure caused by intestinal obstruction allows gas to penetrate into the submucosal space through a mucosal breach. The bacterial theory supports that gas-producing bacteria, such as Clostridium difficile or Clostridium perfringens, invade the submucosal layer through mucosal rents and produce gas within the intestinal wall. Immunosuppressed condition of the patient will weaken the mucosal barrier and contribute to both [9]. We describe a case of EPN along with PI. Probably uncontrolled diabetes caused infection, leading to altered immune status to cause such a fulminant gas producing infection. The simultaneous affection of two systems could be either due to the severity itself, or, one might have led to the other. The initial presentation and investigations were pointing to bowel pathology alone. Ultrasound raised suspicion of an ectopic kidney. X-rays repeated, showed features of EPN. CT scan confirmed class 3b EPN and detected coexistent Pneumatosis coli. Subsequent treatment was intervention for EPN along with antibiotics and parenteral insulin. Close observation was kept Bharati Pandya, et al. 18

5 to detect complications of fulminant colitis, but it resolved with treatment executed for EPN. CONCLUSION Emphysematous pyelonephritis should be a possibility kept in mind for diabetic patients presenting with fever and abdominal symptoms. Serial plain radiographs are basic screening investigations. If there be any suspicion or diagnostic dilemma a non-contrast abdominal computed tomography is recommended, especially if the condition of the patient shows deterioration. For EPN, patients should be given a fair chance with conservative treatment (drainage, antibiotics and control of diabetes). PI is an unusual association and the best modality of diagnosis again, is CT scan. Conservative treatment of PI with close observation for complications is mandatory. Archives of Pathology, vol. 53, no. 6, pp , Pneumatosis Intestinalis Imaging. Medscape: e-medicine, Author: Sameer K Goyal, MD; Chief Editor: Eugene C Lin, MD 8. Haijing Zhang, Stephanie L. Jun and Todd V. Brennan, Pneumatosis Intestinalis: Not Always a Surgical Indication. Case Reports in Surgery: Volume 2012 (2012), Article ID Farshid Ejtehadi, Nikolaos A. Chatzizacharias and Hugh Kennedy, Pneumatosis Intestinalis as the Initial Presentation of Systemic Sclerosis: A Case Report and Review of the Literature Case Reports in Medicine, Volume 2012 (2012), Article ID REFERENCES 1. Jeng-Jong Huang, MD; Chin-Chung Tseng, MD Emphysematous Pyelonephritis Clinicoradiological Classification, Management, Prognosis, and Pathogenesis Arch Intern Med. 2000; 160(6): Karthikeyan A, Kumar S, Ganesh G. Emphysematous pyelonephritis. Indian Journal of Urology 2005;21: Shokeir AA, El-Azab M, Mohsen T, El-Diasty T. Emphysematous pyelonephritis:15 years experience with 20 cases. Urology. 1997;49: Schultz EHKlorfein EH Emphysematous pyelonephritis. J Urol. 1962; Alexandros Strofilas, Andreas Manouras, Emmanuel E Lagoudianakis,et al Emphysematous pyelonephritis, a rare cause of pneumoperitoneum: a case report and review of literature. Cases Journal 2008,1: L. G. Koss, Abdominal gas cysts (pneumatosis cystoides intestinorum hominis), an analysis with a report of a case and a critical review of the literature, A.M.A. Bharati Pandya, et al. 19