Arteriovenous shunting as a new feature of PHACES

Case report

Huan Wang M.D. 1 , Albert K. Oh M.D. 3 , and Darren B. Orbach M.D., Ph.D. 1 , 2
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  • 1 Departments of Neurosurgery and
  • 2 Radiology, Neurointerventional Radiology, Children's Hospital Boston, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts;
  • 3 Department of Surgery Plastic Surgery, Hasbro Children's Hospital, Providence, Rhode Island
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Patients with the congenital neurocutaneous disorder PHACES are at a markedly increased risk of ischemic infarction during childhood. Although intracranial arterial anomalies have been well described, venous abnormalities have not been documented. The authors report on a unique case of a 3-month-old girl with PHACES and a skull base osteodural arteriovenous fistula. A separate arteriovenous shunt at T-5 may also have been present. Imaging findings and treatment strategies are discussed.

Abbreviations used in this paper: AICA = anterior inferior cerebellar artery; AVF = arteriovenous fistula; BA = basilar artery; PHACES = posterior cranial fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, and sternal defects; VA = vertebral artery.

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Contributor Notes

Address correspondence to: Darren B. Orbach, M.D., Ph.D., Neurointerventional Radiology, Brigham & Women's Hospital, 75 Francis Street PBB 356, Boston, Massachussetts 02115. email: dorbach@partners.org.
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