Home | About Us | Current Issue | Ahead of print | Archives | Search | Instructions | Subscription | Feedback | Editorial Board | e-Alerts | Login 
Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
 Users Online:222 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size

Table of Contents   
Year : 2022  |  Volume : 27  |  Issue : 1  |  Page : 115-117

Complicated pylephlebitis secondary to perforated appendicitis in an adolescent

1 Department of Pediatric Gastroenterology and Hepatology, Indraprastha Apollo Hospital, New Delhi, India
2 Department of Pediatrics, Apollo Hospital, Noida, Uttar Pradesh, India

Date of Submission20-Aug-2020
Date of Decision22-Nov-2020
Date of Acceptance09-Jan-2021
Date of Web Publication11-Jan-2022

Correspondence Address:
Dr. Sagar Jayesh Mehta
A 1304/Orchid Suburbia, New Link Road, Kandivali West, Mumbai - 400 067, Maharashtra
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_291_20

Rights and Permissions



Septic thrombophlebitis of the portal vein or pylephlebitis is a rare cause of morbidity and mortality in children. The common causes include infective intra-abdominal pathology (acute appendicitis or diverticulitis) or inflammatory conditions (acute pancreatitis and inflammatory bowel disease). Management involves a multidisciplinary team approach for favorable outcome. We present a case report of pylephlebitis secondary to perforated appendicitis with incomplete resolution of thrombosis.

Keywords: Acute abdomen, anticoagulation, appendicitis, pylephlebitis

How to cite this article:
Mehta SJ, Malhotra S, Panwar A, Sibal A. Complicated pylephlebitis secondary to perforated appendicitis in an adolescent. J Indian Assoc Pediatr Surg 2022;27:115-7

How to cite this URL:
Mehta SJ, Malhotra S, Panwar A, Sibal A. Complicated pylephlebitis secondary to perforated appendicitis in an adolescent. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2023 Jan 31];27:115-7. Available from: https://www.jiaps.com/text.asp?2022/27/1/115/335568

   Introduction Top

Pylephlebitis, resulting from appendicitis, is an uncommon life-threatening condition in children.[1] Due to its rarity, the overall incidence is unknown in children; however, adult studies estimate 2.7 per 100,000 person-years.[2],[3] The diagnosis relies on clinical features, imaging of the portal system and underlying abdominal pathology, and positive blood/tissue cultures.[1],[2],[3],[4] Prompt treatment with antibiotics with anticoagulation and surgical management is essential for patient survival.[1]

   Case Report Top

A 12-year-old boy presented with a high fever with chills and abdominal pain for 10 days. His abdominal pain was periumbilical in location initially but later localized to the right side. There was no significant past or family history. An ultrasound (USG) Doppler on the 10th day of disease showed thrombosis in the main portal vein, while no other abnormal pathology was found.

On examination, he was febrile and sick. Abdominal examination revealed tenderness in the right side of the abdomen and hepatomegaly. Investigations revealed that Hb was 11.1 g/dl; platelet count was 560 × 103/mm3; white blood cell count was 12.06 × 103/mm3 with 75% neutrophils, erythrocyte sedimentation rate (ESR) was 80 mm/h and C-reactive protein (CRP) 31 mg/l, procalcitonin was 2.33 ng/mL, aspartate aminotransferase was 45 U/l, and alanine aminotransferase was 51 U/l. USG of the abdomen showed heterogeneous liver echotexture and splenomegaly; portal vein showed a thrombus without cavernoma formation. The patient was treated with broad-spectrum antibiotics (piperacillin–tazobactam).

An abdominal contrast-enhanced computed tomography (CT) revealed perforated appendicitis and portal venous thrombosis [Figure 1].
Figure 1: Computed tomography of the abdomen on admission: Appendicitis and extraperitoneal rupture. Altered attenuation of the right lobe and portal venous thrombosis involving the main portal vein, superior mesenteric vein, left branch of the portal vein, and occluded right portal vein

Click here to view

He underwent an urgent laparotomy and appendectomy on the 2nd day of hospitalization. The adjacent bowel showed no ischemia. He was started on low-molecular weight heparin (LMWH), and metronidazole was added. The pus culture revealed  Escherichia More Details coli. He showed clinical improvement in the next 24 h and laboratory parameters normalized. The child was discharged after 2 weeks of hospital stay. Antibiotics were continued for 4 weeks, and LMWH was replaced with an oral factor Xa inhibitor (apixaban) that was continued for 12 months. He was reviewed periodically for 12 months using USG Doppler. On follow-up, CT of the abdomen showed an incomplete resolution of thrombus and resulting complication [Figure 2]. Liver chemistry and synthetic functions remained within normal limits.
Figure 2: Computed tomography of the abdomen on follow-up: Recanalization in the main portal vein, superior mesenteric vein, and left portal vein; the right portal vein showing total occlusion and attenuation of intrahepatic portal vein branches with right lobe atrophy and left lobe compensatory hypertrophy. The right hepatic vein is attenuated with peripheral veno-venous collaterals

Click here to view

Screening for underlying prothrombotic predisposition (activated protein C resistance, protein C and S, and antithrombin III) was normal. There was a heterozygous mutation detected in 1298 A > C MTHFR gene, while no mutations were found in prothrombin (G20210A) Factor II, Factor V (G1619 A), or 677 C > T MTHFR genes.

   Discussion Top

Very few case reports of pylephlebitis secondary to undiagnosed perforated appendicitis in children exist in literature. Other known etiologies include umbilical vein catheterization, necrotizing enterocolitis, acute and chronic pancreatitis, liver abscess, and inflammatory bowel diseases.[3]

The diagnosis of septic pylephlebitis was delayed by 12 days in this case. Yoon et al.[3] reported a delay in diagnosis of few days to 6 weeks, and the main reason was attributed to the nonspecific presentation. Clinical features in children usually include fever, abdominal pain, jaundice, and generalized weakness. The clinical features may be a mild form or severe with septic shock and death. Reported mortality in pediatric series is up to 50%.[1],[3]

Typical blood investigations show leukocytosis and elevated inflammatory markers such as thrombocytosis, ESR, CRP, or procalcitonin, similar to this case. Deranged liver biochemistry such as hyperbilirubinemia or transaminitis may be seen in some cases. Blood culture in pylephlebitis is most commonly polymicrobial in nature and may be seen in 23% to 88% of cases.[1],[3] With the correction of surgical pathology, these abnormalities normalized before discharge.

Classical features of portomesenteric thrombosis on Doppler sonography include a flow defect and dilation or absent compressibility of the portal venous system, while CT and magnetic resonance imaging commonly show a central hypodense venous thrombosis.[2] The ease of USG Doppler makes it the initial investigation of choice. However, contrast-enhanced CT scan reveals the filling defect in the portal vein and its tributaries, in addition to the underlying pathology (appendicitis, liver abscess, or pancreatitis), similar to our case.[3]

The first episode of deep-vein thrombosis or a life-threatening thrombotic event in a child warrants an evaluation of the underlying thrombotic condition.[1] Yoon et al. did not report an associated thrombophilic condition in most cases of portomesenteric thrombosis in their review.[3] In this case, we found a heterozygous mutation in (A > C) MTHFR gene that may have contributed to the extension of the thrombotic event.

The most important therapy of pylephlebitis is the eradication of the initial infectious process with appropriate broad-spectrum antibiotics. In our case, the initial blood culture was sterile because of early antibiotics treatment, but pus culture grew E. coli. The ideal duration of antibiotic therapy is unclear, but 4–6 weeks' duration is recommended. Both interval appendectomy and urgent laparotomy with appendectomy have been reported in treating acute appendicitis with pylephlebitis.[1]

The exact role of anticoagulation and duration in the management of septic pylephebitis is controversial.[1],[2],[3],[4] It prevents thrombus extension, improves recanalization, and reduces the risk of bowel ischemia. The usual recommended period is 4–6 months; however, prolonged anticoagulation may be needed in children with underlying thrombotic conditions. We initiated LMWH immediately after surgery and replaced it with oral apixaban (oral nonvitamin K anticoagulant) post discharge that has an adequate safety profile.[3] We decided to continue it for 12 months because of an associated thrombotic predisposition.

The most common complication of pylephlebitis is cavernomatous transformation, leading to portal hypertension and variceal bleeding.[5] We noted a complete resolution of thrombosis in mean platelet volume, left portal vein, and superior mesenteric vein; however, recanalization in the right portal vein did not occur. Subsequently, the right lobe of the liver showed atrophy and peripheral scarring. It resulted in right hepatic vein occlusion and veno-venous collateral formation. The patient remained asymptomatic because of involvement of a single hepatic vein.[6] In conclusion, septic pylephlebitis in a setting of acute appendicitis requires a high index of suspicion and careful evaluation. Close follow-up is essential in children with added complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Levin C, Koren A, Miron D, Lumelsky D, Nussinson E, Siplovich L, et al. Pylephlebitis due to perforated appendicitis in a teenager. Eur J Pediatr 2009;168:633-5.  Back to cited text no. 1
Choudhry AJ, Baghdadi YM, Amr MA, Alzghari MJ, Jenkins DH, Zielinski MD. Pylephlebitis: A review of 95 cases. J Gastrointest Surg 2016;20:656-61.  Back to cited text no. 2
Yoon SH, Lee MJ, Jung SY, Ho IG, Kim MK. Mesenteric venous thrombosis as a complication of appendicitis in an adolescent: A case report and literature review. Medicine (Baltimore) 2019;98:e18002.  Back to cited text no. 3
Granero Castro P, Raposo Rodríguez L, Moreno Gijón M, Prieto Fernández A, Granero Trancón J, González González JJ, et al. Pylephlebitis as a complication of acute appendicitis. Revista Espanola de Enfermedades Digestivas 2010;102:217-8.  Back to cited text no. 4
Degano LA, El Kik SA, Rizzi A. Pylephlebitis in pediatric patients. Arch Argent Pediatr 2014;112:e163-6.  Back to cited text no. 5
Hernández-gea V, De Gottardi A, Leebeek FW, Rautou P, Salem R, Garcia-pagan JC. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. J Hepatol 2019;71:175-99.  Back to cited text no. 6


  [Figure 1], [Figure 2]

This article has been cited by
1 Complicated pylephlebitis secondary to perforated appendicitis in a child- A rare case report
Soumya Pahari, Manju Shrestha, Sunil Basukala, Pooja Kafle, Kalpana Rai, Yugant Khand, Ojas Thapa, Anup Thapa
Annals of Medicine and Surgery. 2022; 82: 104744
[Pubmed] | [DOI]


Print this article  Email this article


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (768 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded35    
    Comments [Add]    
    Cited by others 1    

Recommend this journal

Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer | Privacy Notice

  2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

Online since 1st May '05