Overview Pylephlebitis or septic thrombophlebitis of the portal vein is an extremely rare and potentially fatal complication of appendicitis, which occurs secondary to infection drained by the portal venous system. We describe a case of pylephlebitis resulting from perforated appendicitis complicated by septic thrombosis. Diagnosis and progression was confirmed by computed tomography. The patient had a favorable outcome with medical and surgical therapy, prompting us to evaluate treatment of pylephlebitis.
Pylephlebitis With SMV Thrombosis After Perforated Appendicitis
Prateek K Gupta, MD, Brittany L Willer, MD, Tommy H Lee, MD, and Sumeet K Mittal, MD
Department of Surgery, Creighton University, Omaha, Nebraska.
Contact: Sumeet K Mittal, MD E-mail
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Citation: Gupta PK, Willer BL, Lee TH, and Mittal SK. Bile Leak After Laparoscopic Redo Fundoplication. J Surg Radiol. 2010 Oct 1;1(2).
Received June 29, 2010. Accepted July 29, 2010. Epub August 5, 2010. Copyright: © 2010 Surgisphere Corporation. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
Contents - Introduction- Discussion - Disclosures - References |
Introduction
Pylephlebitis is described as septic thrombophlebitis of the portal vein or one of its tributaries.1,2 The exact incidence of pylephlebitis is unknown, but its diagnosis may be obscured by the primary disease. The morbidity and mortality associated with portal and mesenteric pylephlebitis may be high, as this may be an overlooked condition secondary to an infection in the drainage of the portal venous system.Portal pylephlebitis is a described complication of appendicitis and other infective and inflammatory conditions affecting the small intestine, stomach, pancreas, and biliary tract.1 More infrequent causes include urogenital lesions, subphrenic abscesses, and various malignancies.3 Due to advances in management with earlier diagnosis of its underlying cause and the concurrent use of antibiotics, it is thought to be less common in modern era.4 Greater use of diagnostic radiological imaging may lead to increased diagnosis of this infection. In this article, we report a case of ruptured appendicitis complicated by development of pylephlebitis with superior mesenteric vein (SMV) thrombosis.
Case Report
A 33 year old male came to the emergency department for evaluation of abdominal pain. He presented with acute onset of generalized abdominal pain that began three days prior to admission and was localized to the right lower quadrant. He had associated anorexia, nausea, and emesis. During the initial evaluation, the patient was laying supine in the stretcher with his bilateral lower extremities flexed at the knee and complaining of increasing abdominal pain. The patient was febrile with a temperature of 103 oF. His routine blood work revealed white blood cell count (WBC) of 9,000/dl. A CT scan of the abdomen and pelvis was obtained, revealing a perforated appendix with associated phlegmon, additionally no abnormalities were found in the liver or portal system (Figure 1). After surgical evaluation, the patient received preoperative antibiotics and was taken to the operating room where an open appendectomy was performed. Intra-operative cultures were obtained and demonstrated group C Streptococcus and Klebsiella pneumoniae. The patient was treated with antibiotics for 5 days. His immediate post-operative course was uneventful and he was subsequently discharged home.
Figure 1. Computed tomography demonstrating intense right lower quadrant inflammatory reaction with changes consistent with likely perforated acute appendicitis and associated small fluid collection anterior to the rectum.
The patient returned to the emergency department four days later complaining of abdominal pain, fever, and drainage from the incision site. The previous incision was open and locally explored, noting no fascial defects. Blood work was obtained, which now demonstrated a leukocytosis of 12,000/dl and an elevated alkaline phosphatase level. A CT scan of the abdomen and pelvis was obtained to evaluate for a possible intraabdominal abscess (Figure 2), which was not evident. Surprisingly the CT scan demonstrated a dramatic increase in size of the liver compared to the pre-operative CT scan. Additionally, a filling defect was identified at the bifurcation of the right and left hepatic portal vein corresponding with a thrombus. Numerous lesions were seen throughout the liver and a focus defect measuring 7.7 cm by 5.7 cm was present in the posterior right lobe of the liver.
Figure 2. Computed tomography demonstrated abnormal filling defects within the left and right branches of the portal vein, which is more visible on the right than on the left. There are numerous lesions are seen throughout the liver.
With these new findings, the patient was hospitalized and was started on intravenous antibiotics and on intravenous heparin anticoagulation therapy. During the first week of hospitalization, the patient was treated for a partial bowel obstruction, which resolved with nonoperative management. He continued to have intermittent high fevers, which resolved by the end of the first week. He subsequently began having recurrent abdominal pain and fevers up to 103 oF. Repeat cultures were negative. A repeat CT scan of the abdomen and pelvis was obtained, which now demonstrated a thrombus within the superior mesenteric vein (Figure 3). Continuous heparin therapy and empiric broad-spectrum antibiotics were continued. By the end of his second week of hospitalization, the fevers and abdominal pain had resolved. The anticoagulation was transitioned and converted to Coumadin therapy for long-term anticoagulation, which was managed by his primary care physician. The patient was discharged home. Despite multiple attempts to contact him, we were unable to obtain any further follow-up.
Figure 3. Computed tomography demonstrating an apparent thrombus within the hepatic portion of the portal venous system and a visible clot within the superior mesenteric vein. The spleen remains enlarged while the pancreas, adrenals, and kidneys appear normal.
Discussion
Portal pylephlebitis is a rare complication of appendicitis and other inflammatory conditions that involve the portal drainage. The true incidence of pylephlebitis is unknown. Advancements in antibiotic therapy have presumably kept the incidence low in the modern era.1 Pylephlebitis should be considered in patients with evidence of intra-abdominal infection and bacteremia. A retrospective review of 18 cases in the literature reported the presence of associated bacteremia in 88% of the cases of confirmed pylephlebitis.4 However in our case, the presence of bacteremia was not confirmed as demonstrated by multiple negative blood cultures. Therefore, the absence of bacteremia should not deter one from postulating this diagnosis.
Pylephlebitis secondary to infection can be distinguished from thrombosis due to other causes by its typically non-occlusive nature.5 Septic emboli may result from pylephlebitis, which may distribute within the liver producing intrahepatic abscesses.2,6 This occurrence was demonstrated radiographically as multiple intrahepatic abscess and partial occlusions of the left and right portal veins were presented on CT (Figure 2), as these hepatic abscesses were most likely due to septic emboli. It has been reported that anticoagulation therapy may decrease the incidence of septic embolization from the infected portal thrombi to the liver, thus preventing liver abscesses.7 Anticoagulation therapy in a short duration can be used if thrombosis is associated with sepsis and not complicated by infarction or embolization.4
Although the benefit of adjunctive heparin therapy has not been clearly demonstrated in previous reports, some advocated its use as a primary treatment modality.1,4 An untreated portal or superior mesenteric vein (SMV) thrombosis has an associated mortality rate of up to 32%, and this uniformly feared complication could be catastrophic.8 Harsh recommended anticoagulation therapy as a first-line treatment, based on the presumption that the untreated embolism might further progress causing enteric ischemia. Once the bowel has been compromised, operative and radiological interventions in the form of thrombectomy and thrombolysis with direct intravascular infusion of thrombolytics have been advocated for mesenteric and portal vein non-suppurative thrombosis.9 In our case, there was improvement of symptoms and no progression to bowel compromise with anticoagulation therapy. Therefore, we present this case as an interesting diagnostic dilemma with radiographic findings consistent with portal pylephlebitis and thrombosis secondary to perforated appendicitis. Therefore, general surgeons should be attentive to the possibility of this diagnosis.
Disclosures
The authors have no disclosures or conflicts of interest related to this manuscript.
References
- Bolt RJ. Diseases of the hepatic blood vessels, Chapter 169. In: Bockus Gastroenterology, Fourth Edition. Vol 5. Ed-in-Chief, Berk JE. Philadelphia: WB Saunders; 1985, p3259–69.
- Klinefelter HF Jr, Grose WE, Crawford HJ. Pylephlebitis. Bull Johns Hopkins Hosp. 1960;106:65–73. PubMed
- Mchardy G. The Appendix, Chapter 144. In: Bockus Gastroenterology, fourth ed. Vol 4. Ed-in-Chief, Berk JE. Philadelphia: WB Saunders; 1985, p2609-24.
- Plemmons RM, Dooley DP, Longfield RN. Septic thrombophlebitis of the portal vein (pylephlebitis): Diagnosis and management in the modern era. Clin Infect Dis. 1995; 21:1114–20. PubMed
- Sakalkale R, Reeve P. Portal venous thrombophlebitis in a case of perforated appendicitis: lessons from a case. N Z Med J, 2006, 119:U1984
- Demertzis S, Ringe B, Gulba D, et al. Treatment of portal vein thrombosis by thrombectomy and regional thrombolysis. Surgery. 1994; 115:389–93. PubMed
- Harch JM, Radin RD, Yellin AE, et al. Pylethrombosis: serendipitous radiologic diagnosis. Arch Surg. 1987; 122:1116–9. PubMed
- Kader HA, Baldassano, Harty MP, et al. Ruptured retrocecal appendicitis in an adolescent presenting as portal-mesenteric thrombosis and pylephlebitis. J Ped Gastroenterol Nutr. 1998; 27:584–8. CrossRef
- Baril N, Wren S, Radin R, et al. The role of anticoagulation in pylephlebitis. Am J Surg. 1996; 172:449–53. CrossRef
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