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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 4  |  Issue : 2  |  Page : 35-38

Portal vein recanalization failed to improve chronic intestinal obstruction due to extrahepatic portal vein obstruction


Division of General Surgery, The First People's Hospital of Yunnan Province, Affiliated Hospital of Kunming University of Science and Technology, Kunming, China

Date of Submission01-Jul-2019
Date of Decision31-Aug-2019
Date of Acceptance20-Sep-2019
Date of Web Publication27-Nov-2019

Correspondence Address:
Kunmei Gong
Division of General Surgery, The First People's Hospital of Yunnan Province, Affiliated Hospital of Kunming University of Science and Technology, Kunming 650032
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ts.ts_9_19

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  Abstract 


Extrahepatic portal vein obstruction (EHPVO) refers to obstruction of the extrahepatic portal vein that is characterized by cavernous transformation, portal hypertension, and intestinal dysfunction. Radiological interventions on EHPVO are an extraordinary challenge, although being reported to be safe and effective in selected patients by pertinent experts. Chronic intestinal dysfunction is a rare complication of EHPVO; it is unknown whether portal vein re-canalization by radiological interventions can improve chronic intestinal dysfunction. We describe a 22-year-old male patient with chronic intestinal dysfunction due to EHPVO, which was not improved by portal vein re-canalization. The patient presented with acute abdominal pain and dyspepsia for 2 weeks without hematochezia in August 2016 and was diagnosed with EHPVO. Due to cavernous transformation, systemic anticoagulation therapy was administered, and although his abdominal pain was relieved, the patient still had dyspepsia and partial jejunum dysfunction. Intestinal segmentectomy was suggested but was refused, and the patient received catheter-directed thrombolysis in another hospital. Although the portal vein was partly recanalized, the intestinal obstruction was not alleviated. Four months after onset, an emergent enterectomy was performed due to severe hematochezia with pathological examination findings of necrosis, ulcer, and granulation formation. Unfortunately, the patient developed a serious systemic infection, severe thrombocytopenia and disseminated intravascular coagulation, which was assumed to be caused by intestinal bacterial translocation and serious malnutrition. The infection was subsequently controlled. In conclusion, in patients with chronic intestinal dysfunction due to EHPVO, portal vein re-canalization may not improve intestinal function. Timely enterectomy may prevent intestinal bacterial translocation and serious malnutrition.

Keywords: Case report, cavernous transformation, extrahepatic portal vein obstruction, jejunum dysfunction, portal vein thrombosis


How to cite this article:
Xiao L, Shang L, Xu X, Ouyang Y, Li L, Zhu Y, Gong K. Portal vein recanalization failed to improve chronic intestinal obstruction due to extrahepatic portal vein obstruction. Transl Surg 2019;4:35-8

How to cite this URL:
Xiao L, Shang L, Xu X, Ouyang Y, Li L, Zhu Y, Gong K. Portal vein recanalization failed to improve chronic intestinal obstruction due to extrahepatic portal vein obstruction. Transl Surg [serial online] 2019 [cited 2019 Dec 10];4:35-8. Available from: http://www.translsurg.com/text.asp?2019/4/2/35/271823




  Introduction Top


Extrahepatic portal vein obstruction (EHPVO) refers to obstruction of the extrahepatic portal vein that excludes portal vein thrombosis (PVT) developing concurrently with liver cirrhosis or hepatocellular carcinoma. EHPVO is characterized by cavernous transformation which results in the development of portal hypertension and intestinal dysfunction.[1]

Radiological interventions in patients with EHPVO include transjugular intrahepatic portosystemic shunt (TIPS), which is an extraordinary challenge due to cavernous transformation of the portal vein. However, it has been reported to be safe and effective in selected patients and specialized centers with pertinent expertise.[2]

Chronic intestinal dysfunction is a rare complication of EHPVO, which can lead to intestinal bacterial translocation and malnutrition. It is unknown whether portal vein recanalization by radiological interventions can improve intestinal function in these patients.


  Case Report Top


A 22-year-old male presented to our hospital due to acute abdominal pain and dyspepsia for 2 weeks without hematochezia in August 2016 and was diagnosed with EHPVO. His weight was 69 kg, and body mass index (BMI) was 22.5. The patient had no history of hepatic cirrhosis or alcohol addiction. Contrast-enhanced computed tomography (CT) revealed extra-and intrahepatic PVT with cavernous transformation [Figure 1]. Laboratory results showed no marked changes in infectious markers. The D-dimer level was 18.8 μg/mL. Immunological and tumor markers were negative. Protein C, protein S, and antithrombin III were slightly reduced. The physical examination showed no evidence of peritonitis. Anticoagulation therapy consisting of 5000 units of low molecular weight heparin twice a day was administered considering that cavernous transformation was a relative contraindication of intraluminal catheter-directed thrombolysis. The patient received simultaneous parenteral nutrition. Two weeks later, his abdominal pain was relieved, but the patient still complained of dyspepsia. Oral anticoagulation therapy with warfarin replaced low molecular weight heparin and the patient was found to have an international normalized ratio of 2–3. Upper gastrointestinal (UGI) series and CT revealed upper jejunum dysfunction with incomplete intestinal obstruction [Figure 2] and [Figure 3]. Parenteral and low-dose enteral nutrition was administered continuously with prokinetic therapy. Three weeks later, his intestinal dysfunction in the upper jejunum remained unrelieved and incomplete intestinal obstruction was identified by a second UGI series [Figure 4]. His body weight gradually decreased. Intestinal segmentectomy was suggested, but he refused it.
Figure 1: Contrast-enhanced computed tomography revealed extra-and intrahepatic portal vein thrombosis with cavernous transformation

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Figure 2: Five weeks after onset, contrast-enhanced computed tomography revealed edematous and dilated jejunum

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Figure 3: Five weeks after onset, an upper gastrointestinal series revealed upper jejunum dysfunction with incomplete intestinal obstruction

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Figure 4: Seven weeks after onset, intestinal dysfunction in the upper jejunum remained unrelieved with incomplete intestinal obstruction shown by a second upper gastrointestinal

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Two months after onset, he accepted percutaneous transhepatic catheter-directed thrombolysis in another hospital [Figure 5]. Angiography and CT revealed that the portal vein and superior mesenteric vein were partly recanalized and cavernous transformation was still present [Figure 6] and [Figure 7]. However, the intestinal obstruction was not alleviated after the intervention. Eight weeks after percutaneous transhepatic thrombolysis, an emergent enterectomy was performed in our hospital due to severe hematochezia. At this time, his weight had decreased from 69 to 41 kg, and his BMI had decreased from 22.5 to 13.4. At the site 70 cm distal to the Treitz ligament, the jejunum dilated to 10 cm, and the intestinal walls were edematous and rigid. A 60 cm segment of jejunum was resected [Figure 8]. Pathological examination findings of the resected jejunum showed necrosis and ulcer in intestinal wall with granulation formation, which was in accordance with chronic obstruction.
Figure 5: Eight weeks after onset, percutaneous transhepatic catheter-directed thrombolysis was performed in another institution

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Figure 6: Four months after onset, computed tomography revealed that the portal vein and superior mesenteric vein were partly recanalized, but still with cavernous transformation and edematous jejunum

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Figure 7: Four months after onset, computed tomography revealed that the portal vein and superior mesenteric vein were partly recanalized, but still with cavernous transformation and edematous jejunum

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Figure 8: At a site 70 cm distal to the Treitz ligament, the jejunum was dilated to 10 cm and the intestinal walls were edematous and rigid

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Unfortunately, the patient developed serious systemic infection whose temperature was 40°C 2 days after enterectomy. Stool culture revealed Pseudomonas aeruginosa infection, although blood and bone marrow cultures did not show positive results. The reason for this severe infection was assumed to intestinal bacterial translocation due to chronic intestinal obstruction and serious malnutrition. The patient developed thrombocytopenia (14 × 109/L) and disseminated intravascular coagulation 1 week after surgery. Following 2 weeks of high-dose anti-infective therapy, his infection was finally controlled. The patient was subsequently discharged to a rehabilitation center and has been taking warfarin. No recurrence of EHPVO and intestinal dysfunction were observed during 3 years of follow-up [Table 1].
Table 1: Timeline

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  Discussion Top


EHPVO refers to obstruction of the extrahepatic portal vein that excludes PVT developing concurrently with liver cirrhosis or hepatocellular carcinoma. EHPVO is characterized by cavernous transformation of the portal vein, and the collateral circulation is insufficient, which is clinically significant.[1] Portal cavernoma formation attempts to preserve hepatopetal flow; however, insufficient inflow results in the development of portal hypertension and intestinal dysfunction.

Radiological interventions in patients of EHPVO with portal hypertension include TIPS, partial splenic embolization, percutaneous embolization on splenic artery aneurysms.[3],[4] However, recanalization on the portal vein trunk is challenging due to cavernous transformation of the portal vein. Due to technical complexity and possible complications, radiological interventions on cavernous transformation of portal vein should be performed only in selected patients and in specialized centers with TIPS procedures.[5] In such centers, TIPS is safe and effective with success rate of approximate 98% in patients with cirrhosis and cavernous transformation.[2]

In addition, a Rex shunt is an effective approach for the treatment of children with cavernous transformation of the portal vein at an early stage who do not have additional liver lesions. The portal pressure is significantly decreased immediately after placement of the shunt (P < 0.01) and during the follow-up.[6] Variceal band ligation is another method to decrease the collateral circulation of portal vein, which is capable of partly reducing bleeding risk of EHPVO.[7]

However, almost all these procedures are aimed at patients with portal hypertension. In the present case, intestinal obstruction was the main clinical disorder without obvious portal hypertension; thus, it is unclear if these procedures are effective in such patients. If radiological intervention or a Rex shunt was performed in the early stage, would intestinal dysfunction be prevented? However, when intestinal obstruction is chronic, perhaps all approaches are useless.

In this case, although the portal vein trunk was partially recanalized by catheter-directed thrombolysis, this was insufficient for the treatment of chronic intestinal obstruction and recovery of small bowel function. Completely different from acute intestinal obstruction, pathophysiological changes in the chronic condition, including edema and dyskinesia cannot be improved by portal vein recanalization. Serious malnutrition and intestinal bacterial translocation will subsequently occur due to chronic obstruction, which can then induce severe infection.

The etiology of EHPVO differs in pediatric and adult populations, but hyper-coagulation states are commonly involved. Nevertheless, the vast majority of cases (up to 70%) may remain idiopathic despite thorough laboratory and clinical work-up.[8],[9] In our case, protein-C and protein–S deficiency may have been the cause of EHPVO. Patients with EHPVO often require prolonged oral anticoagulation therapy together with variceal band ligation to prevent thrombosis recurrence and portal hypertension-related bleeding.[7] Therefore, lifelong anticoagulation therapy is necessary in this patient.


  Conclusion Top


In patients with chronic intestinal dysfunction due to EHPVO, portal vein recanalization may not improve intestinal function. Timely enterectomy may prevent intestinal bacterial translocation and serious malnutrition.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This study was supported by Medical Leader's Fund of Yunnan Province, 2017, China.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Franceschet I, Zanetto A, Ferrarese A, Burra P, Senzolo M. Therapeutic approaches for portal biliopathy: A systematic review. World J Gastroenterol 2016;22 (45):9909-20.  Back to cited text no. 1
    
2.
Thornburg B, Desai K, Hickey R, Hohlastos E, Kulik L, Ganger D, Baker T, Abecassis M, Caicedo JC, Ladner D, Fryer J, Riaz A, Lewandowski RJ, Salem R. Pretransplantation portal vein recanalization and transjugular intrahepatic portosystemic shunt creation for chronic portal vein thrombosis: Final analysis of a 61-patient cohort. J Vasc Interv Radiol 2017;28 (12):1714-21.  Back to cited text no. 2
    
3.
Miraglia R, Maruzzelli L, Cortis K, Caruso S, Luca A. Interventional radiology procedures in pediatric patients with extra-hepatic portal vein obstruction and complications of portal hypertension. In: 2014 European Congress of Radiology. Vienna, Austria: European Society of Radiology; 2014.  Back to cited text no. 3
    
4.
Alberti D, Colusso M, Cheli M, Ravelli P, Indriolo A, Signorelli S, Fagiuoli S, D'Antiga L. Results of a stepwise approach to extrahepatic portal vein obstruction in children. J Pediatr Gastroenterol Nutr 2013;57 (5):619-26.  Back to cited text no. 4
    
5.
Klinger C, Riecken B, Schmidt A, De Gottardi A, Meier B, Bosch J, Caca K. Transjugular portal vein recanalization with creation of intrahepatic portosystemic shunt (PVR-TIPS) in patients with chronic non-cirrhotic, non-malignant portal vein thrombosis. Z Gastroenterol 2018;56 (3):221-37.  Back to cited text no. 5
    
6.
Wang RY, Wang JF, Sun XG, Liu Q, Xu JL, Lv QG, Chen WX, Li JL. Evaluation of Rex Shunt on Cavernous Transformation of the Portal Vein in Children. World J Surg 2017;41 (4):1134-42.  Back to cited text no. 6
    
7.
Guillaume M, Christol C, Plessier A, Corbic M, Péron JM, Sommet A, Rautou PE, Consigny Y, Vinel JP, Valla CD, Bureau C. Bleeding risk of variceal band ligation in extrahepatic portal vein obstruction is not increased by oral anticoagulation. Eur J Gastroenterol Hepatol 2018;30 (5):563-8.  Back to cited text no. 7
    
8.
Sarin SK, Khanna R. Non-cirrhotic portal hypertension. Clin Liver Dis 2014;18 (2):451-76.  Back to cited text no. 8
    
9.
Girolami A, Cosi E, Ferrari S, Girolami B. Heparin, coumarin, protein C, antithrombin, fibrinolysis and other clotting related resistances: Old and new concepts in blood coagulation. J Thromb Thrombolysis 2018;45 (1):135-41.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
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