title: Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: report of a case.
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.
authors.affiliation: Department of General Surgery, Faculty of Medicine, Baskent University, 5 Sokak No: 48, Bahçelievler, Ankara, Turkey.
authors.forename: Feza
authors.initials: F
authors.lastname: Karakayali
authors.forename: Ceyla
authors.initials: C
authors.lastname: Basaran
authors.forename: Ebru Ayvazoglu
authors.initials: EA
authors.lastname: Soy
authors.forename: Sema
authors.initials: S
authors.lastname: Karakus
authors.forename: Hakan
authors.initials: H
authors.lastname: Yabanoglu
authors.forename: Gokhan
authors.initials: G
authors.lastname: Moray
authors.forename: Mehmet
authors.initials: M
authors.lastname: Haberal
mesh_terms: D000328:Adult; D019074:Epigastric Arteries / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006391:Hemangioma / Q000175:diagnosis / Q000209:etiology*; D006406:Hematoma / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications*; D011859:Radiography; D017568:Rectus Abdominis / Q000000981:diagnostic imaging; D012422:Rupture, Spontaneous; D013161:Splenic Rupture / Q000175:diagnosis / Q000209:etiology*; D016896:Treatment Outcome