Combined lymphangioma and hemangioma of the spleen in a patient with Klippel-Trénaunay syndrome.
BACKGROUND
Klippel-Trenaunay syndrome (KTS) is a very rare congenital anomaly of blood vessels, characterized by the following clinical triad: varicose superficial veins, port-wine stain and usually bony and soft tissue hypertophy of extremities, most often located in the lower extremities. It is often accompanied by visceral manifestations, and rarely combined with splenomegaly.


METHODS
A 30-year-old female patient came to the Surgery Clinic because of occasional left hypochondrial pain. After she was diagnosed with KTS combined with splenomegaly, splenectomy was performed. Macroscopic and microscopic spleen examination indicated the presence of tumor of vascular origin, presenting a combination of lymphangioma and hemangioma.


CONCLUSIONS
Diagnosed KTS demands a thorough clinical examination of the patient because of the potential presence of visceral manifestations. When splenomegaly is present, even though being often benign, splenectomy is usually performed to alleviate accompanying symptoms which occur as a result of organ enlargement and compression, to prevent rupture and consequential bleeding when the vascular spleen tumor is large, and finally to avoid a possibility of malignant transformation.
Surgery Clinic, Clinical Center Kragujevac, Kragujevac, Serbia. drmspasic@gmail.com
Marko
M
Spasić
Dragce
D
Radovanović
Dragan
D
Canović
Goran
G
Azanjac
Predrag
P
Djurdjević
Slobodanka
S
Mitrović
D000328:Adult; D005260:Female; D006391:Hemangioma / Q000150:complications*; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications*; D008202:Lymphangioma / Q000150:complications*; D013160:Splenic Neoplasms / Q000150:complications*