Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: report of a case.

Surgery Today
Feza KarakayaliMehmet Haberal

Abstract

We report a case of Klippel-Trenaunay syndrome (KTS) with serious morbidity caused by the rupture of hemangiomas of the spleen and inferior epigastric artery (IEA). A 40-year-old woman, who had suffered from edema and varicose veins in her left leg and toes since birth, underwent emergency laparotomy and splenectomy for a spontaneous splenic rupture. Pathological examination revealed hemangiomatosis of the spleen. She presented again 40 days later with a rectus muscle hematoma, which computed tomography revealed to be actively bleeding. Arteriography confirmed a bleeding IEA, which was then embolized. Hematological investigation revealed a heterozygous form of factor VIII and fibrinogen deficiency. The patient recovered well and was asymptomatic at her 1-year follow-up. We report this case to reinforce that investigations for KTS should involve all organ systems, and include detailed hematologic tests. By defining coagulation and vascular abnormalities, life-threatening bleeding episodes may be prevented.

References

Nov 1, 1985·Annals of Surgery·P A BaskervilleN L Browse
Jan 1, 1974·Radiology·J Bliznak, T W Staple
Jan 27, 1998·Mayo Clinic Proceedings·A G JacobP Gloviczki
Nov 20, 1998·The American Journal of Gastroenterology·K Darwish, B L Bleau
May 31, 2002·Dermatology : International Journal for Clinical and Investigative Dermatology·Nicolas J MuellerThonas Enzler
Feb 24, 2006·Obstetrical & Gynecological Survey·Susan R SteinShirley K Sawai
Apr 13, 2006·Medicine·W Brett Cherry, Paul S Mueller
Apr 18, 2006·Journal of Vascular Surgery·Ravul JindalNicholas J W Cheshire

❮ Previous
Next ❯

Citations

Aug 7, 2013·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Arivarasan KarunamurthyMiguel Reyes-Múgica
Aug 25, 2010·Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine·Irving T WellsSimon Freeman

❮ Previous
Next ❯

Related Concepts

Related Feeds

Blood Clotting Disorders

Thrombophilia includes conditions with increased tendency for excessive blood clotting. Blood clotting occurs when the body has insufficient amounts of specialized proteins that make blood clot and stop bleeding. Here is the latest research on blood clotting disorders.