0
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Acute intestinal infarction caused by initially unexplained splanchnic venous thromboses in a patient with protein C deficiency: A thought-provoking emergency case

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Highlights

          • Splanchnic venous thrombosis caused by superior mesenteric vein thrombosis is rare.

          • Delayed diagnosis and/or treatment can result in acute intestinal infarction.

          • Protein C deficiency is rare.

          • Insufficient awareness can result in mismanagement of splanchnic venous thrombosis.

          • Coagulation disorders contribute to acute intestinal infarction of unknown cause.

          Abstract

          Introduction and importance

          Splanchnic venous thrombosis (SVT) originating in the superior mesenteric vein (SMV) is rare and may cause acute intestinal infarction (AII). Protein C deficiency (PCD) results in thrombophilia.

          Presentation of case

          Acute unexplained SVT originating in the SMV and portal vein was detected in 68-year-old man. Pan-peritonitis and AII were diagnosed and emergency surgery performed. Part of the small intestine was necrotic and partial resection without anastomotic reconstruction was performed. Heparin was administered intravenously continuously from postoperative day (POD) 1. Hereditary, heterozygous, type 1 PCD was diagnosed postoperatively. The anastomosis was reconstructed on POD 16. Warfarin was substituted for heparin on POD 22. No recurrent thrombosis occurred during 2 years of follow-up.

          Clinical discussion

          Patients with the rare condition of SVT require prompt diagnosis and treatment and may have underlying disease. PCD can cause SVT even in intact veins and anticoagulation therapy should be administered immediately postoperatively. Misdiagnosis and/or delayed treatment of SVT can result in AII, a life-threatening condition with a high mortality rate. Insufficient clinician awareness can result in serious mismanagement of patients with PCD and SVT; emergency patients with AII caused by unexplained SVT should therefore be further investigated for prothrombotic states and assessment of coagulation–fibrinolysis profiles to clarify the underlying mechanism.

          Conclusion

          We here present a thought-provoking emergency case of AII associated with acute SVT caused by underlying PCD that was successfully treated by two-stage surgery and anticoagulation therapy. This case provides a timely reminder for emergency clinicians and gastrointestinal surgeons.

          Related collections

          Most cited references 11

          • Record: found
          • Abstract: found
          • Article: not found

          The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines

          The SCARE Guidelines were first published in 2016 and were last updated in 2018. They provide a structure for reporting surgical case reports and are used and endorsed by authors, journal editors and reviewers, in order to increase robustness and transparency in reporting surgical cases. They must be kept up to date in order to drive forwards reporting quality. As such, we have updated these guidelines via a DELPHI consensus exercise.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Liver sinusoidal endothelial cells: Physiology and role in liver diseases.

            Liver sinusoidal endothelial cells (LSECs) are highly specialized endothelial cells representing the interface between blood cells on the one side and hepatocytes and hepatic stellate cells on the other side. LSECs represent a permeable barrier. Indeed, the association of 'fenestrae', absence of diaphragm and lack of basement membrane make them the most permeable endothelial cells of the mammalian body. They also have the highest endocytosis capacity of human cells. In physiological conditions, LSECs regulate hepatic vascular tone contributing to the maintenance of a low portal pressure despite the major changes in hepatic blood flow occurring during digestion. LSECs maintain hepatic stellate cell quiescence, thus inhibiting intrahepatic vasoconstriction and fibrosis development. In pathological conditions, LSECs play a key role in the initiation and progression of chronic liver diseases. Indeed, they become capillarized and lose their protective properties, and they promote angiogenesis and vasoconstriction. LSECs are implicated in liver regeneration following acute liver injury or partial hepatectomy since they renew from LSECs and/or LSEC progenitors, they sense changes in shear stress resulting from surgery, and they interact with platelets and inflammatory cells. LSECs also play a role in hepatocellular carcinoma development and progression, in ageing, and in liver lesions related to inflammation and infection. This review also presents a detailed analysis of the technical aspects relevant for LSEC analysis including the markers these cells express, the available cell lines and the transgenic mouse models. Finally, this review provides an overview of the strategies available for a specific targeting of LSECs.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Protein C and protein S deficiency - practical diagnostic issues.

              Protein C (PC) and protein S (PS) are vitamin K-dependent glycoproteins, that act as natural anticoagulants. The proteolytic activation of PC by thrombin occurs on the surface of endothelial cells and involves thrombomodulin and endothelial PC receptor. In the presence of PS, phospholipids and calcium, activated PC (APC) inactivates membrane bound factors V (FVa) and FVIIIa by their cleavage at the specific arginine residues. PC and PS deficiencies are inherited as autosomal dominant disorders associated with recurrent venous thromboembolism (VTE) and, in most cases, derived from heterozygous missense mutations (78% and 63%, respectively). Heterozygous PC deficiency is found in 6% of families with inherited thrombophilia, in 3% of patients with a first-time deep vein thrombosis (DVT) and 0.2-0.3% of healthy individuals. The PS deficiency is detected more commonly than PC deficiency and its prevalence has been estimated with a less than 0.5% in the general European population and 2% to 12% of selected groups of thrombophilic patients. Approximately 75% of PC-deficient patients have type I deficiency and 95% of PS-deficient patients develop type I and type III of PS deficiency. The diagnosis of PC and PS deficiencies is challenging, many preanalytical and analytical factors may affect the PC/PS levels. Molecular analysis of the PC and PS genes (PROC and PROS1, respectively) involves direct gene sequencing and if negative, multiplex ligation-dependent probe amplification (MLPA) method. Patients with low PC and PS levels and the known mutation within PROC or PROS1 genes combined with other genetic or environmental thrombosis factors are at increased risk of recurrent thromboembolic events and require lifelong oral anticoagulation.
                Bookmark

                Author and article information

                Contributors
                Journal
                Int J Surg Case Rep
                Int J Surg Case Rep
                International Journal of Surgery Case Reports
                Elsevier
                2210-2612
                22 January 2021
                February 2021
                22 January 2021
                : 79
                : 390-393
                Affiliations
                Department of Surgery, Shiga General Hospital, 5-4-30 Moriyama, Moriyama, Shiga, 524-8524, Japan
                Author notes
                [* ]Corresponding author. horitomo55office@ 123456yahoo.co.jp
                Article
                S2210-2612(21)00073-0
                10.1016/j.ijscr.2021.01.071
                7848722
                33517210
                © 2021 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                Categories
                Case Report

                Comments

                Comment on this article