Summary

An 11-year-old girl presented to the hospital with vomiting, left upper-quadrant pain and blood in the stool. An ultrasound scan showed no blood flow in the coeliac axis or the splenic artery. A contrast-enhanced CT scan then demonstrated no flow beyond the coeliac axis origin, a large clot in the distal superior mesenteric artery (SMA), a large splenic infarct and bilateral renal infarcts. Investigations revealed a positive lupus anticoagulant and she was given a presumptive diagnosis of catastrophic antiphospholipid syndrome (CAPS). She was anticoagulated and started on combination immunosuppressive therapy. Her surgical management was by SMA embolectomy, small bowel resection and proximal stoma formation. Stoma closure was performed at 3 months, and she was eventually established on full enteral feeds. Childhood CAPS is a rare condition with a very high mortality rate, and the successful outcome in this case reflects close multidisciplinary teamwork between medical and surgical specialties.