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Clinical Problem Solving
March 2015

A Scalp Lesion With Intracranial Extension

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

A 6-month-old girl 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, measuring 18 × 15 mm (Figure, A). The lesion was growing with the child.

The patient’s history was also significant for congenital left renal agenesis and failure of her newborn hearing screening test. On examination, she had epicanthal folds, upslanted palpebral fissures, and bilateral clinodactyly. Results from initial genetic testing, including microarray and single gene testing for branchio-oto-renal syndrome, were negative for abnormalities.

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