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Published on: November 4, 2015

Catastrophic antiphospholipid syndrome and pulmonary embolism in a 3-year-old child

Carine Olivier1, Eleonore Blondiaux, Thierry Blanc

  • 1Department of Radiology, University Hospital of Rouen, 1 Rue de Germont, Rouen, 76031, France.

Pediatric Radiology
|June 13, 2006
PubMed

Insights

Catastrophic antiphospholipid syndrome (CAPS) is a rare condition causing widespread blood clots. This case highlights a severe pediatric instance with an unfavorable outcome despite prompt diagnosis and treatment.

Area of Science:

  • Pediatric Rheumatology
  • Hematology
  • Medical Imaging

Background:

  • Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis and pregnancy morbidity.
  • Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening variant with rapid onset of multiple organ system involvement.

Observation:

  • A 3-year-old girl with no prior medical history presented with extensive and recurrent thromboses.
  • The patient experienced cardiopulmonary embolism, indicative of severe thrombotic events.

Findings:

  • Diagnosis of CAPS was confirmed by high titers of autoantibodies against phospholipids and beta-2-glycoprotein 1.
  • Despite rapid diagnosis and multimodal treatment strategies, the patient's outcome was unfavorable.
  • Multimodality imaging, including ultrasonography and spiral CT, was crucial for monitoring thrombotic progression.

Implications:

  • This case underscores the critical need for early recognition and aggressive management of CAPS in pediatric populations.
  • The unfavorable outcome emphasizes the challenges in treating severe pediatric CAPS and the need for further research into effective therapeutic approaches.
  • Advanced imaging techniques play a vital role in the comprehensive management and follow-up of pediatric patients diagnosed with CAPS.

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