International Journal of Surgery Case Reports 99 (2022) 107657 Contents lists available at ScienceDirect International Journal of Surgery Case Reports journal homepage: www.elsevier.com/locate/ijscr Case report A case of pylephlebitis complicating an acute appendicitis: Uncommon cholangitis-like situation Mar Dalmau a, *, Carlos Petrola a, Pablo Lopez a, Ramon Vilallonga a, b, Amador Garcia Ruiz de Gordejuela a, b, Manel Armengol a, b a Department of General and Digestive Surgery, Hospital Universitari Vall d’Hebron, Barcelona, Spain b Universidad Autónoma de Barcelona, Spain A R T I C L E I N F O A B S T R A C T Keywords: Introduction: Pylephlebitis represents an uncommon but serious condition with significant mortality which can Phylephlebitis complicate intrabdominal sepsis of any etiology. One of the most common predisposing infections is appendicitis. Appendicitis Presentation of case: A 21-year-old male with 4 days of epigastric and right upper quadrant pain with associated Cholangitis presentation fever and chills with hyperbilirubinemia and leukocytosis in blood test was orientated as cholangitis at first diagnostic. Poor response to antibiotic treatment with persistent fever and bacteriemia with E. coli and S. con­ stellatus isolated in blood cultures led to complete the study with a CT scan which revealed an acute appendicitis complicated with thrombosis of the superior mesenteric vein (SMV) up to the splenoportal confluence. Appen­ dectomy, treatment with broad-spectrum antibiotic and anticoagulation treatment led to full recovery. Follow-up after 6 months showed almost complete SMV patency. Discussion: Pylephlebitis can present as a clinical cholangitis-like picture with hyperbilirubinemia with or without liver abscess formation. CT scan seems to be the most sensitive diagnostic test as it identifies the underlying focus of infection, the extension of the thrombosis and detects liver abscesses. Surgical removal of the source of infection as appendectomy and adequate antibiotic treatment adjusted by culture should be initiated promptly. Anticoagulant treatment should be considered in the case of poor clinical outcome or thrombosis progression. Conclusion: Pylephlebitis should be suspected mainly in patients with appendicitis and diverticulitis with erratic behavior despite surgical removal and/or antibiotic treatment with abnormal liver tests and persistent bacter­ iemia. CT scan is the preferred image study. 1. Introduction 2. Case presentation Pylephlebitis, or infective thrombosis of the portal vein system, re­ A 21-year-old male with no history of interest presented with a 4-day sults from intrabdominal sepsis of any etiology and represents a serious history of epigastric and upper quadrant abdominal pain, vomiting and condition due to subacute presentation and delayed diagnosis with fever with chills accompanied with dark tone urine. On admission, he significant morbidity and mortality from 11 to 32 % [1–3]. Outcome in had a high temperature (38.5 ◦ C), his blood pressure was 117/80 reports over the last 20 years have improved because of earlier diagnosis mmHg, his pulse rate 110/min and his respiratory rate was 20/min. with sensitive image technics and prompt treatment [1]. Physical examination noted right upper quadrant pain and tenderness A case of thrombophlebitis of the superior mesenteric vein as a without muscle guarding. Blood test results showed normal leukocytes complication of acute appendicitis with a clinical cholangitis-like debut (9.250/mm3; NV 4.000–11.000/mm3), elevate bilirubin (2.5 times is described. It was successfully treated with surgical removal of the first normal value), increased cholestasis enzymes (alkaline phosphatase infection site, antibiotics, and anticoagulation therapy. The work has slightly elevated, and γ-GT 3 times elevated) and an elevated C-reactive been reported in line with the SCARE criteria [2]. protein (21.65 mg/dL; NV 0.03–0.5 mg/dL). Abdominal ultrasound described a minimal ectasia of the intrahepatic bile duct with no apparent obstructive cause and cholelithiasis. Under diagnostic * Corresponding author at: Hospital Universitari Vall Hebron, Universidad Autónoma de Barcelona, Paseo de la Vall d’Hebron, 119-129, 08035 Barcelona, Spain. E-mail addresses: mardalmau6@gmail.com (M. Dalmau), manel.armengol@vallhebron.cat (M. Armengol). https://doi.org/10.1016/j.ijscr.2022.107657 Received 30 July 2022; Received in revised form 11 September 2022; Accepted 11 September 2022 Available online 15 September 2022 2210-2612/© 2022 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). M. Dalmau et al. International Journal of Surgery Case Reports 99 (2022) 107657 orientation of acute cholangitis in a patient with cholelithiasis, he was received oral anticoagulation with acenocoumarin and completed 6 treated with amoxicillin clavulanic after extracting blood cultures and weeks of intravenous antibiotic therapy. The follow-up 6th month CT admitted to the Hospital. Blood cultures were positive for Escherichia coli scan demonstrated patency of the SMV, finishing anticoagulant treat­ and Streptococcus constellatus. During his stay, the patient remained ment by then. feverish despite antibiotic therapy for 48 h. The treatment was switched to piperacillin-tazobactam, and new blood cultures were obtained. An 3. Discussion extended study with magnetic resonance cholangiopancreatography (MRCP) showed hepatosplenomegaly, bile duct within the normal limits Pylephlebitis begins with the thrombophlebitis of small veins, and hepatic periportal and subcapsular edema suggesting hepatitis. The draining an area of infection and leading to septic thrombophlebitis of microbiological study was extended with serology of hepatotropic vi­ the portal and mesenteric vein by extension. Diverticulitis and appen­ ruses, which resulted negative. In the context of acute infection with dicitis are the most common predisposing infections [1,3] although it persistent fever and bicytopenia with lymphopenia and thrombocyto­ has also been associated with pancreatitis [3], cholangitis and infected penia, a CT abdominal scan was performed, with findings of acute choledocholithiasis [4,5], inflammatory bowel disease [6] and pelvic appendicitis complicated with an abscess at the root of the mesenterium sepsis [7,8]. (37 × 26 mm) and superior mesenteric vein (SMV) thrombosis (Fig. 1). A Bacteriemia associated with pylephlebitis occurs in 44–88 % of cases gangrenous appendix was removed, and the abscess was drained. Anti­ and is usually polymicrobial, E. coli and B. fragilis being the most com­ coagulation treatment with low molecular weight heparin (LMWH) was mon isolations [1,4]. In the case presented, blood cultures in admission started, with prophylactic doses reaching complete treatment when resulted positive for E. coli and Strepctococcus constellatus. However, two platelet recount was normalized. He had erratic progress with fever on days after initial antibiotic treatment, in the surgical liquid only E. coli the 7th postoperative day with a CT scan that showed a surgical site fluid BLEE was present. collection of 87x32mm with almost complete thrombus of the SMV. The most common presenting symptoms are abdominal pain and Initially, a percutaneous drainage was placed, resulting insufficient and fever. Clinical signs may include right upper quadrant or generalized requiring subsequent laparoscopic surgical drainage. Later surgical abdominal tenderness and hepatomegaly. Although pylephlebitis usu­ cultures resulted positive for E. coli BLEE, so the treatment spectrum was ally presents as an acute illness, the course of the primary focus of extended with ertapenem. Eventually, his postoperative progress was infection is subacute and silent [9,10]. adequate, and he was discharged on the 24th postoperative day. He Leukocytosis and abnormal liver function tests, especially an Fig. 1. Coronal section of abdominal CT-scan with thrombosis of the superior mesenteric vein (arrow) secondary to an appendicitis complicated with an ab­ scess (asterix). 2 M. Dalmau et al. International Journal of Surgery Case Reports 99 (2022) 107657 increase of cholestasis values, are common findings in blood tests. Grant information Clinical jaundice appears to be an unusual condition in pylephlebitis unless it is complicated with hepatic abscesses [1,11,12]. In the above None. case, an increase of bilirubin and mild abnormalities in liver function suggested an incipient cholangitis, which was later seen to have over­ Funding lapped and masked the clinical picture of an acute appendicitis with thrombophlebitis, in the absence of liver abscess formation. Moreover, This research had no funding. the inciting intrabdominal sepsis may also contribute to hepatic disfunction. Ethical approval In terms of complications, liver abscesses are reported in a third of cases, resulting from septic emboli release within the liver [3,11]. This study was exempt from ethical approval. Mesenteric vein involvement, present in 42 % of cases could lead to bowel ischemia and infarction. However, bowel ischemia requiring Consent resection is a rare condition described in just one case [13]. In the case presented, the thrombus involved the SMV up to the splenoportal Written informed consent was obtained from the patient for publi­ confluence without intrahepatic implication or signs of bowel ischemia cation of this case report and accompanying images. A copy of the in surgical revision. Portal hypertension is a rare and long-term written consent is available for review by the Editor-in-Chief of this complication [14]. journal on request. The diagnosis is reached by demonstration of portal vein thrombosis in a patient with bacteriemia. As it is an uncommon condition, the Author contribution diagnosis is usually delayed because symptoms are unspecific and clin­ ical signs from the first infection site are typically subacute. In the past, Mar Dalmau: Managed the case, Writed the original manuscript, Got the diagnosis was often done at laparotomy or in the autopsy in patients informed consent. with severe sepsis. Carlos Petrola: Managed the case, Reviewed and edited the manu­ Demonstration of thrombosis in the portal and mesenteric vein can script, Got informed consent. be done by CT and ultrasonography. The advantage of the CT study is Pablo López: Managed the case, Reviewed and edited the manu­ identification of the underlying focus of infection, extent of pyle­ script, Got informed consent. phlebitis, and intrahepatic abnormalities, such as liver abscesses [15]. Ramón Vilallonga: Managed the case, Reviewed and edited the It is also remarkable that in this case both abdominal US and MRCP manuscript. were not able to find the real cause of the infection. It’s likely that the Amador Garcia Ruiz de Gordejuela: Managed the case, Reviewed and biliary signs and symptoms led the radiologist to focus primarily on the edited the manuscript. biliary tract. It’s possible that in such a severe case of sepsis an Manel Armengol Carrasco: Reviewed and edited the manuscript, abdominal CT-scan should have been done earlier. Supervised the process. The principal treatment for the infection is by using empiric broad gram-negative and anaerobic spectrum antibiotics until culture results Guarantor are available [1]. The treatment, which takes at least 4 to 6 weeks, can be completed by oral agents after a significant clinical response and Amador Garcia Ruiz de Gordejuela. availability, depending on the microorganisms isolated. Surgery is needed when the main infective cause is an appendicitis, Research registration number although Stizenberg et al. [16], reported that interval laparoscopic ap­ pendectomy could be delayed 3 months after treatment. Surgical or Not applicable. radiological percutaneous drainage of intrabdominal abscess associated may be needed. The role of anticoagulant treatment is controversial, as there are no Provenance and peer review randomized controlled studies and no consensus on its use in throm­ bophlebitis. Extensive thrombosis to the mesenteric vein, progression of Not commissioned, externally peer-reviewed. thrombosis in CT control, persistent bacteremia despite antibiotic treatment or presence of hypercoagulable state, are all reasonable in­ Declaration of competing interest dications for anticoagulation [14,17–19]. The most common regimen is initially anticoagulation with LMWH None of the authors have conflicts of interest. [14]. On discharge, it can be switched to an oral anticoagulant. Treat­ ment duration is uncertain. It is reasonable to stop when image CT References control suggests that the thrombus disappears or remains stable. [1] R.M. Plemmons, D.P. Dooley, R.N. Longfield, Septic thrombophlebitis of the portal vein (pylephlebitis): diagnosis and management in the modern era, Clin. Infect. 4. 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