1. Teaching NeuroImages

2. Vignette
F/40
Symptoms: horizontal diplopia, right proptosis Onset: 1 month ago
Signs:
Esotropia / Right abducens nerve palsy
Pupil: small, isocoric / RAPD (-/-)
Cutaneous capillary malformation
Hypertrophy of bone and soft tissue
Varicose vein on lower extremities
Kim et al.

3. Imaging Kim et al. Figure 2. Figure 1.
Figure 1. The clinical features of Klipple-Trenaunay syndrome. Cutaneous capillary malformation on her right hand, hemihypertrophy of the left lower extremity, bony and soft tissue hypertrophy of right toe and varicose vein on right leg confirmed the diagnosis of Klippel-Trenaunay syndrome.
Figure 2. The orbital CT images of the patient. Orbital CT demonstrates multiple giant fusiform intracranial aneurysms, maximal diameter of 34mm, from bilateral internal carotid artery, basilar artery and right posterior cerebral artery.
Figure 2.
Figure 1.
Kim et al.

4. Multiple giant intracranial aneurysms in Klippel-Trenaunay syndrome (KTS)
KTS is a rare congenital mesodermal phakomatosis, characterized by combinations of varicose vein, cutaneous capillary malformation and hypertrophy of bone and soft tissue.
Vascular malformations combined with KTS usually originate from slow flow systems, such as capillary, lymphatic, and venous systems. However, arterial aneurysms are reported in the literature, though only 8 cases of intracranial aneurysms have been reported so far
Our patient had mild ophthalmic symptoms, associated with potentially life-threatening multiple giant intracranial aneurysms, unlike previous cases
The present case emphasizes the importance of prompt cerebrovascular imaging with suspicion in KTS patients with neurological or ophthalmic symptoms.
References
Cohen MM Jr. Klippel-Trenaunay syndrome. Am J Med Genet. 2000;93:
Star A, Fuller CE, Landas SK. Intracranial aneurysms in Klippel-trenaunay/weber syndromes: case report. Neurosurgery. 2010;66:E ; discussion E8.
Kim et al.