The diagnosis is usually obvious on account of a congenital facial cutaneous hemangioma also known as port wine stain or facial nevus flammeus. Sturge weber syndrome ct monday september 10 2012 hemiatrophy neurocutaneous syndromes neuroradiology sturge weber syndrome tram track calcification 35 year male presented with h o frequent fall seizures abnormal cognitive state.
Sturge weber syndrome can be classified into.
Tram track sturge weber syndrome radiology. It is one of the phakomatoses and is often associated with port wine stains of the face glaucoma seizures intellectual disability and ipsilateral leptomeningeal angioma cerebral malformations and tumors. In a child with developmental delay presenting with seizures macrocephaly presence of port wine stains on the body and imaging features of subcortical tram track like pattern of calcifications parenchymal volume loss enlarged choroid plexus and calvarial hyperostosis are diagnostic of sturge weber syndrome. Sturge weber syndrome is a rare syndrome with an incidence estimated at 1 case in 20 000 50 000 persons 11.
Intracranial calciﬁcation a common radiologic manifestation. Sturge weber syndrome diagnostic imaging. Sturge weber syndrome sws also known as encephalotrigeminal angiomatosis is a rare neurocutaneous syndrome characterized by a facial capillary vascular malformation a port wine stain pws with ipsilateral leptomeningeal angiomas lma.
It is caused by calcifications along the cerebral sulcus reflecting vascular malformations in the pia mater. However the tram track sign is not present in many patients who are younger than 2 years old. The tram track sign is a well known manifestation of sturge weber syndrome at cerebral ct.
It may or may not be associated with ipsilateral glaucoma 1. 1 department of radiology hacettepe university 06100 sihhiye ankara turkey. Tram track of cortex dilated medullary vein draining into dilated deep vein enlarged choroid plexus.
15128996 indexed for medline. This feature is almost always present and usually involves the ophthalmic division v1 of the. In patients with sturge weber syndrome x ray ﬁndings in the skull usually show a tram track pattern of calciﬁcation that is caused by calciﬁcation in opposing gyri on either side of an intervening dilated sulcus.
Sturge weber syndrome sometimes referred to as encephalotrigeminal angiomatosis is a rare congenital neurological and skin disorder. 1 a case of 55 year old man with first ever generalized seizure diagnosed with sturge weber syndrome type iii by characteristic mri findings. Review of literature with case illustration romanian neurosurgery vol.
Sturge weber syndrome encephalotrigeminal angiomatosis radiology discussion including radiology cases.