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British Journal of Radiology (2009) 82, e102-e104
© 2009 British Institute of Radiology
doi: 10.1259/bjr/36297676
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British Journal of Radiology 82 (2009),e102-e104 ©2009 The British Institute of Radiology

Klippel–Trenaunay syndrome with hemimegalencephaly, retroperitoneal lymphangioma and double inferior vena cava

S VURUCU, MD 1 B BATTAL, MD 2 M KOCAOGLU, MD 2 and R AKIN, MD 1

Departments of 1 Pediatric Neurology and 2 Radiology, Gulhane Military Medical School, Ankara, Turkey

Correspondence: Murat Kocaoglu, Department of Radiology, Gulhane Military Medical School, 06018 Etlik, Ankara, Turkey. E-mail: kocaoglumurat{at}yahoo.com; kocaoglumurat{at}gata.edu.tr

Klippel–Trenaunay syndrome (KTS) is a rare disorder characterised by congenital vascular hamartomas, limb hypertrophy, lymphangiomas and atresia of lymph vessels with non-pitting oedema. A 6-year-old girl with KTS was referred to our hospital for evaluation of intractable seizures. In addition to findings consistent with KTS, we also found hemimegalencephaly, retroperitoneal lymphangioma and double inferior vena cava. All of these associations in the same patient with KTS are unique in the English literature. We report on the multidedector CT and MRI features of such an unusual case.






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