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Retroperitoneal Laparoscopic Debridement and Drainage for Pancreatic Abscess
Published on: March 15, 2024
CAPS: a rare acute abdomen
John-Paul Smith1, Mark Woodward, Oliver Tunstall
1Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK. jposmith@gmail.com
BMJ Case Reports
|August 2, 2013
Summary
A rare pediatric case of catastrophic antiphospholipid syndrome (CAPS) involved extensive blood clots, leading to organ damage. Prompt multidisciplinary medical and surgical intervention resulted in a successful recovery for the young patient.
Area of Science:
- Vascular Medicine
- Pediatric Thrombosis
- Autoimmune Disorders
Background:
- Catastrophic Antiphospholipid Syndrome (CAPS) is a rare, life-threatening condition characterized by widespread organ thrombosis.
- Childhood CAPS is exceptionally rare and presents significant diagnostic and management challenges.
- Early recognition and aggressive treatment are crucial for improving outcomes in pediatric thrombotic events.
Observation:
- An 11-year-old girl presented with severe abdominal pain, vomiting, and gastrointestinal bleeding.
- Vascular imaging revealed extensive arterial thrombosis, including the celiac axis, splenic artery, and superior mesenteric artery (SMA), with associated splenic and bilateral renal infarcts.
- Laboratory investigations confirmed the presence of lupus anticoagulant, leading to a diagnosis of CAPS.
Findings:
- The patient was treated with anticoagulation, immunosuppressive therapy, and surgical intervention including SMA embolectomy, small bowel resection, and stoma formation.
- Successful stoma closure and establishment of enteral feeding were achieved at three months post-operatively.
- This case highlights the critical role of a multidisciplinary approach in managing complex pediatric thrombotic emergencies.
Implications:
- Successful management of childhood CAPS requires a coordinated effort between hematology, rheumatology, radiology, and surgery.
- Aggressive anticoagulation and immunosuppression, coupled with timely surgical intervention, can lead to favorable outcomes even in severe presentations of pediatric CAPS.
- This case underscores the importance of considering rare thrombotic disorders in pediatric patients presenting with unexplained multi-organ ischemic events.
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