Nasal encephalocele in a child with Beckwith–Wiedemann syndrome

Case report

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✓ Beckwith–Wiedemann syndrome (BWS) is a rare congenital syndrome characterized by gigantism, macroglossia, exophthalmos, postpartum hypoglycemia, and multiple midline defects such as omphalocele. The authors describe, to the best of their knowledge, the first case of a child in whom BWS was diagnosed and who was subsequently treated for a nasal encephalocele.

Because the authors believe that this feature might not be an incidental finding in patients with BWS, intranasal masses in these patients should be carefully differentiated, as complications might be severe.

Abbreviations used in this paper: BWS = Beckwith–Wiedemann syndrome; MR = magnetic resonance; Shh = sonic hedgehog.
Article Information

Contributor Notes

Address correspondence to: Marike L. D. Broekman, M.D., Ph.D., Department of Neurosurgery, G03.124, University Medical Center Utrecht, P.O. Box 85500, 3508 GA Utrecht, The Netherlands. email: m.l.d.broekman-4@umcutrecht.nl.
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