Hrev_master [Emergency Care Journal 2013; 9:e12] [page 33] Pylephlebitis secondary to strangulated umbilical hernia with small bowel ischemia Ennio Bruschi,1 Giovanna Graziani,1 Maurizio Vergendo,1 Antonello Conte,2 Massimo Valentino1 1Radiology Department, Sant’Antonio Abate Hospital; 2Surgery Department, Sant’Antonio Abate Hospital, Tolmezzo, Italy Abstract Pylephlebitis is a septic thrombophlebitis of the portal venous system that infrequently complicates small bowel infarction. We present a case of pylephlebitis with portomesenteric vein gas bubbles secondary to small bowel ischemia caused by a strangulated umbilical hernia, diagnosed on computed tomography (CT) and confirmed in the operating theater. This case is an example of the usefulness of CT in early recognition of suggestive radiolog- ic findings of pylephlebitis associated with intestinal ischemia for prompt treatment of the patient. Introduction We report a case of portomesenteric venous gas combined with band-like pneumatosis, a diagnosis based on computed tomography (CT) evidence of both findings. We performed an emergency laparotomy for suspected acute bowel ischemia, which was confirmed by the operative findings. Case Report A 58-year-old man came to Sant’Antonio Abate Hospital with severe abdominal pain, tachycardia, hypotension and hypoxia. His med- ical history included hypertension, congestive heart failure, atrial fibrillation, replacement of the ascending aorta, HCV-related liver disease and obesity. Laboratory studies revealed leuko- cytosis (white cell count of 12.13×103/µL vs nor- mal range 4.20-11.0×103/µL), with normal hemoglobin level and platelet count. Liver enzyme levels were normal. Total bilirubin level was normal and direct bilirubin level was elevat- ed (0.56 mg/dL vs normal range 0-0.40 mg/dL). International normalized ratio was 10.52 (nor- mal range 2-4.5) and C-reactive protein (CRP) was 69 mg/L (normal range 0-5 mg/L). Ultrasound examination, which was per- formed in the emergency department, revealed absence of fluid in the abdominal cavity, although the examination was limited because of the patient’s body type and the presence of abundant meteorism. Computed tomography examination, per- formed before and after intravenous adminis- tration of contrast media (120 mL of Ioexolo 350 mgI/mL, Omnipaque 350; Schering, Berlin- Wedding, Germany) showed evidence of several gas bubbles within superior mesenteric vein and its branches diffusely extended to the intra- hepatic portal branches (Figures 1 and 2). Signs of pneumatosis parietalis were also demonstrat- ed (Figures 2 and 3). In the umbilical region, a strangulated small bowel hernia was demon- strated, with dilatation of multiple proximal small bowel loops (Figure 4), which showed thickened and contrast-enhancing walls. Diagnosis of small bowel ischemia secondary to strangulated hernia with pylephlebitis was made on the basis of CT findings. The patient underwent surgical treatment with resection of a small bowel loop and latero-lateral ileal anas- tomosys was made. The patient gradually recov- ered with reduction of white blood cell (WBC) levels and CRP. Discussion Pylephlebitys is a septic thrombophlebitis of the portal venous system, usually secondary to an infection in the region drained by the portal system or in the structures contiguous to the portal vein. Pylephlebitis begins with throm- bophlebitis of the small veins that drain the infected area.1 There are a few articles that describe the imaging presentation of pyle- phlebitys, an infrequent but known complica- tion of small bowel infarction.2-7 Portomesen - teric vein gas is most commonly caused by mesenteric ischemia but may have a variety of other causes. Main causes of this complication include: complicated diverticulitis, appendici- tis, urinary infections, pelvic infections, biliary diseases, inflammatory bowel diseases, necro- tizing pancreatitis, infections with unknown source (Table 1). Primary factors that favor the development of this pathologic entity are intestinal wall alterations, bowel distention, and sepsis. Although the pathogenesis of portomesenteric venous gas and pneumatosis intestinalis in acute bowel ischemia has not been estab- lished, it may be related to the ulcers and rup- tures in the continuity of the bowel wall in necrotic mucosa where normal resistance to intraluminal gas pressure is lost.7 Extension of the thrombophlebitis into larger veins leads to septic thrombophlebitis of the mesenteric vein, which can extend further to involve the portal vein. Symptoms and diagnostic imaging findings of small bowel ischemia have been described. Pylephlebitis is a severe clinical entity, having a mortality of 11-32%. Clinical manifestations are usually confusing, varying from asymptomatic through minimal symp- toms related to the primary infection site, to acute abdomen. In the subacute form of venous mesenteric thrombosis, patients may have abdominal pain for days or weeks, with- out bowel infarction. In 88% of patients there is bacteriemia, with E. coli and B. fragilis as the main pathogens. Liver involvement can cause hepatomegaly, elevation of liver enzyme levels, abscesses and jaundice. Seldom, there is a hypercoagulable state associated.8 Imaging findings of pylephlebitis are related to venous involvement with presence of intravascular thrombi or air, the primary source of infection and intrahepatic anomalies, such as unopaci- fied branches of the portal vein, transient parenchymal attenuation differences and intrahepatic abscesses.8 At CT, portal vein gas appears as tubular areas of decreased attenua- tion in the liver, predominantly in the left lobe. These low-attenuation areas are caused by the accumulation of gas in the intrahepatic portal veins, where it is carried by centrifugal blood to the hepatic periphery. Portal vein gas can be visualized at conventional radiography, but substantial amounts must be present for detec- tion.7 The radiographic criterion for portal vein gas is a branching area of low attenuation extending to 2 cm of the liver capsule.3 Intrahepatic portal vein gas must be differenti- ated from air in the biliary tree (pneumobilia). In latter condition, the air is located centrally Emergency Care Journal; volume 9:e12 Correspondence: Ennio Bruschi, Radiology Department, Sant’Antonio Abate Hospital, via Morgagni Giobatta 18, 33028 Tolmezzo, Italy. Tel. +39.347.9904714 - Fax: +39.0433.48833. E-mail: bruschie73@gmail.com Key words: pylephlebitis, strangulated umbilical hernia, small bowel ischemia. Contributions: the authors contributed equally. Conflict of interests: the authors declare no potential conflict of interests. Received for publication: 21 March 2013. Revision received: 29 May 2013. Accepted for publication: 30 May 2013. This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0). ©Copyright E. Bruschi et al., 2013 Licensee PAGEPress, Italy Emergency Care Journal 2013; 9:e12 doi:10.4081/ecj.2013.e12 Non co mmerc ial us e o nly [page 34] [Emergency Care Journal 2013; 9:e12] (i.e. it does not extend to 2 cm of the liver cap- sule) and a confluence of air is seen in the common hepatic duct. Pneumobilia also has a left lobe predilection at CT due to its more ven- tral location. Gas in the small mesenteric veins appears as tubular or branched areas of decreased attenuation in the mesenteric border of the bowel. At CT, pneumatosis may manifest with small isolated gas bubbles within an ischemic bowel wall or as broad rims of air dis- secting the entire bowel wall into two layers.3 The reported specificities of pneumatosis and portal venous gas for acute bowel ischemia usu- ally approach 100%.3 In agreement with the lit- erature,7 not only is the presence of full-thick- ness bowel wall necrosis, but also the extent of transmural bowel infarction that determines the clinical outcome of affected patients. Computed tomography findings of pneumatosis intestinalis and portomesenteric venous gas due to bowel ischemia do not generally allow prediction of transmural bowel infarction, because they may be observed in patients with only partial ischemic bowel wall damage. The clinical outcome of patients with bowel ischemia with these CT findings seems to depend mainly on the severity and extent of their underlying disease. Pylephlebitis remains an entity with high morbidity and mortality, sel- dom having subtle signs and symptoms. Still, early recognition of suggestive radiologic find- ings with the combination of findings of small bowel infarction and multiple venous thrombo- sis and/or gas bubbles in the region drained by the portal system, thanks to modern imaging modalities, may improve the prognosis of patients. Conclusions Computed tomography findings of pneumato- sis intestinalis and portomesenteric venous gas due to bowel ischemia do not generally allow prediction of transmural bowel infarction, because they may be observed in patients with only partial ischemic bowel wall damage. The clinical outcome of patients with bowel ischemia with these CT findings seems to depend mainly on the severity and extent of their underlying disease.6 The presence of portomesenteric venous gas has been previously indicative of an unfavorable clinical outcome, with a poor prog- nosis in patients having bowel ischemia. We agree with the theory that it is not only the pneu- matosis, the severity of bowel ischemia and the presence of a full-thickness bowel wall necrosis, but also the extent of transmural bowel infarc- tion that mainly determine the clinical outcome of affected patients.6 However, urgent laparoto- my is mandatory in patients at risk of ischemic bowel, with CT findings of portomesenteric venous gas and bandlike pneumatosis. Case Report Table 1. Reported causes of portomesenteric venous gas. Necrotic bowel SMA syndrome Cholangitis Organ transplant (liver, kidney, bone marrow) Inflammatory bowel disease Bowel obstruction Acute suppurative cholecystitis Graft-vs-host reaction Peptic ulcer Blunt abdominal trauma Colonic diverticulitis Pulmonary disease (chronic obstructive pulmonary disease, bronchopneumonia, asthma) Acute necrotizing pancreatitis Child abuse Colitis steroid and cytostatic therapy Intraperitoneal tumor Barotraumas Abdominal tuberculosis Seizures Severe enteritis ERCP Intra-abdominal abscess Colonoscopy Retroperitoneal abscess Barium enema Gangrene Portal phlebitis SMA, superior mesenteric artery syndrome; ERCP, endoscopic retrograde cholangio-pancreatography. Figure 1. Computed tomography scan. Preliminary scout view demonstrates intra- hepatic gas (a). Contrast material- enhanced computed tomography scans obtained at the top of the liver show tubu- lar areas of decreased attenuation in the periphery of the liver (b, c; white arrows). These findings are consistent with the pres- ence of gas in the intrahepatic portal veins. Non co mmerc ial us e o nly [Emergency Care Journal 2013; 9:e12] [page 35] References 1. Plemmons RM, Dooley DP, Longfield RN. Septic thrombophlebitis of the portal vein: (pylephlebitis): diagnosis and manage- ment in the modern era. Clin Infect Dis 1995;21:1114-20. 2. Feczko PJ, Mezwa DG, Farab MC, White BD. Clinical significance of pneumatosis of the bowel wall. Radiographics 1992;12: 1069-78. 3. Wiesner W, Khurana B, Ji H, Ros PR. CT of acute bowel ischemia. Radiology 2003;226: 635-50. 4. Sebastià C, Quiroga S, Espin E, et al. Portomesentericvein gas: pathologic mechanisms, CT findings, and prognosis. Radiographics 2000;20:1213-24. 5. Bradbury MS, Kavanagh PV, Bechtold RE, et al. Mesenteric venous thrombosis: diag- nosis and noninvasive imaging. Radiogra - phics 2002;22:527-41. 6. Wiesner W, Mortelé KJ, Glickman JN, et al. Pneumatosisintestinalis and portomesen- teric venous gas in intestinal ischemia: correlation of CT findings with severity of ischemia and clinical outcome. Am J Roentgenol 2001;177:1319-23. 7. Lai WH, Hwang TL, Chen HW. Portome - senteric venous gas in acute bowel ischemia: report of a case. Surg Today 2008;38:656-60. 8. García Figueiras R, Liñares Paz M, Baleato González S, Villalba Martín C. Case 158: pylephlebitis. Radiology 2010;255:1003-7. Case Report Figure 2. Contrast-enhanced computed tomog- raphy scan showing tubular or branched areas of decreased attenuation as expression of mesenteric vein gas (yellow arrows). Figure 3. Bubble-like pneumatosis in small bowel. Enhanced computed tomography scan showing intramural bowel gas (white arrow). Figure 4. a) Humbilical hernia loop of the small intestine; b) sagittal view. White arrow in a) and b) indicates dilatation of the upstream part of small bowel. Non co mmerc ial us e o nly