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Article ; Online: CAPS: a rare acute abdomen.

Smith, John-Paul / Woodward, Mark / Tunstall, Oliver / Ramanan, Athimalaipet

BMJ case reports

2013  Volume 2013

Abstract: ... performed at 3 months, and she was eventually established on full enteral feeds. Childhood CAPS is a rare ... CAPS). She was anticoagulated and started on combination immunosuppressive therapy. Her surgical ...

Abstract 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.
MeSH term(s) Abdomen, Acute/etiology ; Antiphospholipid Syndrome/complications ; Antiphospholipid Syndrome/diagnosis ; Catastrophic Illness ; Child ; Female ; Humans
Language English
Publishing date 2013-07-31
Publishing country England
Document type Case Reports ; Journal Article
ISSN 1757-790X
ISSN (online) 1757-790X
DOI 10.1136/bcr-2013-010081
Database MEDical Literature Analysis and Retrieval System OnLINE

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