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Sturge Weber Syndrome

Betz MLB - MedPix (2006)

View Article: MedPix Image - MedPix Case

Affiliation: Childrens Hospital of Dayton, OH

ABSTRACT

Diagnosis: Sturge Weber Syndrome

History: This 8 yo girl presented at birth with large left sided facial nevus involving all three branches of the cutaneous trigeminal nerve.

Findings: Frontal and lateral views of the skull were obtained as a component of a shunt evaluation. These films show tram track intracranial calcifications over the posterior cerebral hemisphere. Non-contrast CT of brain demonstrated hydrocephalus, left sided intracranial calcifications of the cortex and atrophy of the left cerebral hemisphere, and hypertrophy of the overlying cranium. MR of brain was obtained when the patient experienced stroke like symptoms. These show lack of nomal left-sided venous drainage resulting in tortuous anomalous venous structures. The leptomeningeal angioma of left side meninges is best seen on the TRICKS MR angiogram.

Ddx: 1. Klippel-Trenaunay-Weber Syndrome: triad of cutaneous capillary and venous malformations (usually port-wine stain), venous varicosities, and hyperplasia of soft tissues and bone 2. Beckwith-Wiedemann Syndrome: may also have port-wine stain, but other features are macroglossia, omphalocele, macrosomia, and hypoglycemia 3. Sturge-Weber Syndrome: triad of cutaneous capillary and venous malformations (usually port-wine stain), ocular lesions (choroidal angioma and buphthalmos) and cerebral hemiatrophy.

Dxhow: Classic radiologic findings including the MR TRICKS images of the leptominingeal angioma and serpentine cortical signal drop out on the GRE sequences. CT of brain shows intracranial calcifications.

Exam: Pt is developmentally delayed. She is able to stand on her own, speak a few words and smile appropriately.

No MeSH data available.


The double contour  “gyriform” patterns of intracranial calcifications give “tram tracks” appearance on plain films (arrows).
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MPX2309_synpic29053: The double contour “gyriform” patterns of intracranial calcifications give “tram tracks” appearance on plain films (arrows).


Sturge Weber Syndrome

Betz MLB - MedPix (2006)

The double contour  “gyriform” patterns of intracranial calcifications give “tram tracks” appearance on plain films (arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX2309&req=5

MPX2309_synpic29053: The double contour “gyriform” patterns of intracranial calcifications give “tram tracks” appearance on plain films (arrows).

View Article: MedPix Image - MedPix Case

Affiliation: Childrens Hospital of Dayton, OH

ABSTRACT

Diagnosis: Sturge Weber Syndrome

History: This 8 yo girl presented at birth with large left sided facial nevus involving all three branches of the cutaneous trigeminal nerve.

Findings: Frontal and lateral views of the skull were obtained as a component of a shunt evaluation. These films show tram track intracranial calcifications over the posterior cerebral hemisphere. Non-contrast CT of brain demonstrated hydrocephalus, left sided intracranial calcifications of the cortex and atrophy of the left cerebral hemisphere, and hypertrophy of the overlying cranium. MR of brain was obtained when the patient experienced stroke like symptoms. These show lack of nomal left-sided venous drainage resulting in tortuous anomalous venous structures. The leptomeningeal angioma of left side meninges is best seen on the TRICKS MR angiogram.

Ddx: 1. Klippel-Trenaunay-Weber Syndrome: triad of cutaneous capillary and venous malformations (usually port-wine stain), venous varicosities, and hyperplasia of soft tissues and bone 2. Beckwith-Wiedemann Syndrome: may also have port-wine stain, but other features are macroglossia, omphalocele, macrosomia, and hypoglycemia 3. Sturge-Weber Syndrome: triad of cutaneous capillary and venous malformations (usually port-wine stain), ocular lesions (choroidal angioma and buphthalmos) and cerebral hemiatrophy.

Dxhow: Classic radiologic findings including the MR TRICKS images of the leptominingeal angioma and serpentine cortical signal drop out on the GRE sequences. CT of brain shows intracranial calcifications.

Exam: Pt is developmentally delayed. She is able to stand on her own, speak a few words and smile appropriately.

No MeSH data available.