Spontaneous spleen rupture and rectus sheath hematoma in a patient with Klippel-Trenaunay syndrome: report of a case.
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.
Karakayali
Feza
F
Department of General Surgery, Faculty of Medicine, Baskent University, 5 Sokak No: 48, Bahçelievler, Ankara, Turkey.
Basaran
Ceyla
C
Soy
Ebru Ayvazoglu
EA
Karakus
Sema
S
Yabanoglu
Hakan
H
Moray
Gokhan
G
Haberal
Mehmet
M
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