Use of Rivaroxaban for Prophylaxis of Superficial Venous Thrombosis in Klippel-Trenaunay- Weber Syndrome - Case Report and Systematic Review
Bibliographic record
Abstract
Abstract INTRODUCTION: We present a case of a young man with Klippel Trenaunay Weber Syndrome (KTWS) characterized by recurrent superficial vein thrombosis (SVT) complicated by an episode of disseminated intravascular coagulation (DIC) who ultimately was maintained on prophylactic rivaroxaban to prevent recurrent thrombotic events. We performed a literature search to identify similar cases and to summarize common presenting features and attempted treatment modalities. CASE DESCRIPTION: An 18 year old man with KTWS presented with a large vascular malformation over his left chest and arm developed acute worsening SVT post sodium tetradecyl injection. Further investigation revealed a severe DIC was also present. On exam, the venous malformation and left arm were extremely tender with significant bruising. Presenting labs: Hg 24, platelet 98, bilirubin 588 (unconjugated 212), LDH 1597, PTT 32, INR 1.9, fibrinogen 1.1, Ddimer >20. He was treated with aggressive supportive care for DIC and routine deep vein thrombosis (DVT) prophylaxis with low-molecular-weight heparin (LMWH). Symptoms improved after anticoagulation was started. Ultrasound of his left arm revealed old SVT with recanulization within the venous malformation. He subsequently developed recurrent painful crises in his arm secondary to recurrent SVT. These were usually treated with short term LMWH. Imaging studies ruled out any deep vein thrombotic events. We eventually elected to commence indefinite duration anticoagulation with rivaroxaban 10mg daily with significant decrease in further acute flares. METHODS: To review how thrombotic manifestations of KTWS are managed, we performed a comprehensive literature search on PubMed using MESH terms: Klippel Trenaunay Weber Syndrome, Thrombosis, DIC and Kasabach-Merritt coagulopathy. RESULTS: We found 99 abstracts. 87 were excluded for non thrombotic complications of KTWS. 13 case reports focusing on treatment of thrombotic manifestations of KTWS were analyzed further. These included 12 case reports reviewing management of DVT in KTWS and 1 case report reviewing management of SVT in KTWS however with no DIC. 3 of these abstracts also reviewed management of coagulopathy (DIC or local intravascular coagulopathy). Of the 12 case reports, 5 described patients with deep vein thrombosis in KTWS treated with therapeutic warfarin; 4 patients improved however 1 died of cor pulmonale. An additional 5 cases of DVT in KTWS were successfully managed with therapeutic LMWH. 2 of these cases were also complicated by DIC. One case report described use of therapeutic rivaroxaban (20mg daily) to manage DVT in KTWS. This case was also complicated by DIC. There were no case reports describing the prevention of SVT in KTWS. CONCLUSION: To our knowledge this is the first case report to describe successful off-label use of prophylactic rivaroxaban to prevent further thrombotic events in a patient with KTWS. Disclosures Off Label Use: RIVAROXABAN -- PROPHYLACTIC ANTI-THROMBOSIS FOR SUPERFICIAL VEIN THROMBOSIS. Wu:Leo Pharma: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Pfizer: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.007 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.002 | 0.002 |
| Bibliometrics | 0.007 | 0.007 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.002 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".