Peter J. Koltai MD, FACS, FAAP

Publication Details

  • PHACE syndrome: Report of a case with a glioma of the anterior skull base and ocular malformations INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY Cannady, S. B., Kahn, T. A., Traboulsi, E. I., Koltai, P. J. 2006; 70 (3): 561-564

    Abstract:

    PHACE syndrome consists of the constellation of manifestations including Posterior fossa anomalies of the brain (most commonly Dandy-Walker malformations), Hemangiomas of the face and scalp, Arterial abnormalities, Cardiac defects, and Eye anomalies. We present the case of a patient who presented with respiratory distress at birth secondary to a large nasal glioma. She was subsequently found to have a ventricular septal defect (VSD), a facial hemangioma, and a malformation of the eye and optic nerve head. The nasal glioma, which extended to the cribriform plate, has not been described in this syndrome. The tumor was resected through a coronal incision, midline nasal bone osteotomy, and a retrograde dissection from the nasal bones to the anterior skull base. Glioma of the skull base is a novel and serious manifestation of this uncommon condition.

    View details for DOI 10.1016/j.ijporl.2005.047.014

    View details for Web of Science ID 000235354700025

    View details for PubMedID 16144720

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