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A full-term, 7-month-old male infant presented to an outpatient pediatric otolaryngology clinic with a firm, irregularly shaped posterior neck mass. He had been diagnosed prenatally with cerebellar hypoplasia, so a magnetic resonance imaging study of the brain was performed at 1 month of age. This study demonstrated an incidental solid, homogenous mass in the subcutaneous fat of the right occiput with irregular borders and infiltration into the paraspinal musculature (Figure, A). On examination, the upper posterior neck mass was deep to palpation without overlying skin changes. The patient had no discomfort, and neck movement was not restricted.
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D. Glial heterotopia
Glial heterotopia occurs when normal glial tissue abnormally appears at a location distant from the central nervous system (CNS). These lesions arise when fragments of embryonic neuroglial tissue become separated from the developing CNS.1 Although glial heterotopia lesions may be connected to the cranium via a fibrous stalk, complete separation (rather than herniation or invagination) from the CNS is what differentiates lesions such as glial heterotopia from meningoceles, encephaloceles, or meningoencephaloceles. While glial heterotopia in the head and neck area often presents in the nasal region (frequently referred to as nasal glioma), these masses may occur in other locations along the developing nervous system, including the paranasal sinuses, orbits, nasopharynx, scalp, face, neck, lungs, and gluteal regions.1,2 In many cases, glial heterotopia is asymptomatic and discovered incidentally.3 The incidence of nasal glioma is 1 in 20 000 to 40 000 births, with nonnasal glial heterotopia presenting even less commonly.4 Glial heterotopia may be associated with other congenital abnormalities, including cleft palate, congenital heart defects, Pierre Robin sequence, or posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies syndrome.5,6
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Corresponding Author: Andrew R. Scott, MD, Division of Otolaryngology and Facial Plastic Surgery, Floating Hospital for Children at Tufts Medical Center, 800 Washington St, Boston, MA 02111 (firstname.lastname@example.org).
Published Online: October 17, 2019. doi:10.1001/jamaoto.2019.2861
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient’s parents for granting permission to publish this information. We would also like to thank Julie Tse, MD, a pathologist at Tufts Medical Center, for providing the histopathologic images that were used in this article. She was not compensated for her contributions.
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