SHM Converge Scientific Abstract ePoster Gallery-Weathering Storms: When Thrombosis Begets Thrombosis

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This case describes a 5-year-old girl admitted with fatigue, fever, weight loss, abdominal pain, bilateral anterior uveitis, and optic disc edema. Initial workup was unrevealing, but cross-sectional imaging showed extensive thrombosis in multiple unusual vascular beds: dural venous sinuses with cerebral edema and midline shift, bilateral subsegmental pulmonary emboli, hepatic and portal vein thrombosis, and bilateral renal vein thromboses.

The presentation met criteria for <strong>thrombotic storm</strong>, a rare, acute, extreme prothrombotic state marked by multiple arterial or venous thrombotic events occurring over a short period, often in unusual locations. A triggering event such as infection, trauma, surgery, or pregnancy may be present, and about half of cases are associated with antiphospholipid antibodies. Diagnosis is often difficult and relies heavily on imaging, especially when thrombosis occurs in more than one site or appears progressive or recurrent despite treatment.

The clinical team transferred the patient to the ICU and started <strong>bivalirudin</strong>. She was also treated with high-dose steroids, infliximab, and azathioprine while specialty services evaluated for underlying causes. Her thromboses stabilized without hemorrhage, and she was later transitioned to therapeutic enoxaparin. The differential diagnosis included catastrophic antiphospholipid syndrome, heparin-induced thrombocytopenia, paraneoplastic syndromes, Behçet disease, Kimura disease, and CMV infection. Extensive autoimmune, hematologic, and genetic testing did not identify a definitive cause.

Key takeaways:
- Maintain a low threshold to search for additional thromboses when an unusual clot is found.
- Thrombotic storm should be suspected with multiple acute clots in diverse or unusual vascular beds.
- Rapid anticoagulation is the mainstay of therapy and is critical to reduce morbidity and prevent fatal outcomes.

Asset Subtitle

Elizabeth Petersen

Meta Tag

Author List Elizabeth Petersen

Category Clinical Vignettes

Concept Thrombotic Storm

Concept Thrombosis

Concept Acute Thrombotic Event

Concept Catastrophic Antiphospholipid Antibody Syndrome

Concept Anticoagulation

Distinguished Finalist

Presenter Organization Boston Children's Hospital and Brigham and Women's Hospital

Presenting Author Elizabeth Petersen

Track Pediatric

Keywords

thrombotic storm

multiple thromboses

unusual vascular beds

dural venous sinus thrombosis

pulmonary emboli

hepatic vein thrombosis

portal vein thrombosis

renal vein thrombosis

bivalirudin

anticoagulation

Thrombotic Storm

Thrombosis

Acute Thrombotic Event

Catastrophic Antiphospholipid Antibody Syndrome

Anticoagulation