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AJNR - American Journal of Neuroradiology

Published ahead of print on October 5, 2007
doi: 10.3174/ajnr.A0711

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Posterior Fossa Dermoid Cysts in Association with Klippel-Feil Syndrome: Report of Three Cases

V.V. Pai^a, L.H. Lowe^a,b, M. Castillo^d, B.Y. Huang^d and L. Shao^a,c

^a From the University of Missouri-Kansas City School of Medicine, Kansas City, Mo
^b Departments of Radiology, Children's Mercy Hospital and Clinics, Kansas City, Mo
^c Pathology, Children's Mercy Hospital and Clinics, Kansas City, Mo
^d Department of Neuroradiology, University of North Carolina-Chapel Hill, Chapel Hill, NC

View larger version (104K): [in this window] [in a new window]   Fig 1. Posterior fossa dermoid and Klippel-Feil deformity in a 3-year-old boy. A, Lateral cervical spine radiograph shows a C2-C4 block vertebrae. B, Axial cranial CT without contrast demonstrates a midline posterior fossa mass (arrow), with mild surrounding low-attenuation halo of edema and a small focus of extension into the occipital bone (arrowhead). C, Axial T1-weighted MR image with contrast confirms the midline, cystic-appearing mass with peripheral enhancement (arrow) and extension through the midline occipital bone (arrowhead). D, Microscopic specimen of the benign dermoid cyst reveals keratinized squamous epithelial lining (arrowhead) with pilosebaceous glands and hair follicles with hair shafts (arrows). The cerebellar tissue (c) shows extensive inflammatory infiltrates (hematoxylin-eosin stain, original magnification X400).

View larger version (81K): [in this window] [in a new window]   Fig 2. Posterior fossa dermoid and Klippel-Feil deformity in a 12-month-old girl. A, Axial cranial CT with contrast shows a right heterogeneous posterior fossa mass with peripheral enhancement. Note that this mass (M) extends beyond the midline and posterior through the occipital bone (arrows) into the occipital soft tissues. B, Sagittal T1-weighted image with gadolinium shows obstructive hydrocephalus. Note that the posterior fossa dermoid cyst extends through the occipital bone by a sinus tract (arrows).

View larger version (118K): [in this window] [in a new window]   Fig 3. Posterior fossa dermoid and Klippel-Feil deformity in a 2-year-old boy. Axial T2-weighted image demonstrates a midline hyperintense intracranial mass with sinus tract (arrow) extending through the occipital bone forming an occipital scalp soft tissue mass (arrowhead).