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Clinical Problem Solving | Radiology

A Scalp Lesion With Intracranial Extension QUIZ

David S. Bick, MD1; John J. Brockland, BS, MS2; Andrew R. Scott, MD3
[+] Author Affiliations
1Department of Otolaryngology, Tufts Medical Center, Boston, Massachusetts
2Department of Surgery, University of Missouri, Columbia
3Divisions of Pediatric Otolaryngology and Facial Plastic & Reconstructive Surgery, Floating Hospital for Children–Tufts Medical Center, Boston, Massachusetts
JAMA Otolaryngol Head Neck Surg. 2015;141(3):289-290. doi:10.1001/jamaoto.2014.3479.
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In this case report, a 6-month-old girl has presented with a lesion at the midline vertex of her skull that had been present since birth. The lesion was noted to have an annular dark tuft of hair with central clearing emanating from an underlying plaque directly over the posterior fontanel. Results from initial genetic testing, including microarray and single gene testing for branchio-oto-renal syndrome, were negative for abnormalities. What is your diagnosis?

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Figure.

A, Photograph of lesion at the midline vertex of the skull. B, Magnetic resonance image demonstrating a T1-hypointense, T2-hyperintense lesion with mild central enhancement. C, Magnetic resonance image showing embryonic vertical positioning of the straight sinus. D, Intraoperative photograph.

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