Proteus syndrome with syringohydromyelia and arachnoid cyst.
BACKGROUND
Proteus syndrome (PS) is a rarely seen, sporadic disorder with a wide range of abnormalities including asymmetrical overgrowth, skin lesions, dysregulated adipose tissue, and vascular malformations. Brain and spinal malformations are extremely rare; syringohydromyelia and arachnoid cyst have not been reported previously.


METHODS
We present a 5-year-old girl with PS having severe central nervous system (CNS) abnormalities demonstrated by magnetic resonance imaging (MRI) including craniocutaneous lipomatosis, hemimegalencephaly, arachnoid cyst, and syringohydromyelia.
Anik
Yonca
Y
School of Medicine, Department of Radiology, University of Kocaeli, 41380 Umuttepe, Kocaeli, Turkey. yoncaanik@yahoo.com
Anik
Ihsan
I
Gonullu
Erdem
E
Inan
Nagihan
N
Demirci
Ali
A
D016080:Arachnoid Cysts / Q000209:etiology / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D054220:Malformations of Cortical Development / Q000209:etiology / Q000473:pathology; D019339:Port-Wine Stain / Q000209:etiology / Q000473:pathology; D016715:Proteus Syndrome / Q000150:complications / Q000473:pathology*; D013131:Spine / Q000473:pathology; D013595:Syringomyelia / Q000209:etiology / Q000473:pathology*