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http://dx.doi.org/10.3345/kjp.2012.55.1.29

PHACE association with intracranial, oropharyngeal hemangiomas, and an atypical patent ductus arteriosus arising from the tortuous left subclavian artery in a premature infant  

Kim, Do-Hyun (Department of Pediatrics, Dongguk University Ilsan Hospital)
Choi, Jang-Hwan (Department of Pediatrics, Dongguk University Ilsan Hospital)
Lee, Jung-Ha (Department of Pediatrics, Dongguk University Ilsan Hospital)
Kim, Hee-Sup (Department of Pediatrics, Dongguk University Ilsan Hospital)
Publication Information
Clinical and Experimental Pediatrics / v.55, no.1, 2012 , pp. 29-33 More about this Journal
Abstract
PHACE association is a rare neurocutaneous condition in which facial hemangiomas associate with a spectrum of posterior fossa malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. We reported a case of PHACE association in a premature infant showing facial, intracranial, and oropharyngeal hemangiomas with evidence of the Dandy-Walker variant and complicated cardiovascular anomalies, including a right-sided aortic arch and an atypical patent ductus arteriosus arising from a tortuous left subclavian artery. To our knowledge, intracranial hemangiomas are rare in PHACE association, and a concomitant oropharyngeal hemangioma has not been previously reported in the PHACE association literature. In infants presenting with large, plaque-like facial hemangiomas, it is important to conduct active cardiovascular and neurological evaluations. Special attention should be given to the laryngoscopic examination to search for additional hemangiomas in the airway.
Keywords
PHACE association; Central nervous system cavernous hemangioma; Patent ductus arteriosus; Premature infants;
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1 Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996;132:307-11.
2 Bhattacharya JJ, Luo CB, Alvarez H, Rodesch G, Pongpech S, Lasjaunias PL. PHACES syndrome: a review of eight previously unreported cases with late arterial occlusions. Neuroradiology 2004;46:227-33.
3 Heyer GL, Millar WS, Ghatan S, Garzon MC. The neurologic aspects of PHACE: case report and review of the literature. Pediatr Neurol 2006;35: 419-24.
4 Metry DW, Dowd CF, Barkovich AJ, Frieden IJ. The many faces of PHACE syndrome. J Pediatr 2001;139:117-23.
5 Metry DW, Haggstrom AN, Drolet BA, Baselga E, Chamlin S, Garzon M, et al. A prospective study of PHACE syndrome in infantile hemangiomas: demographic features, clinical findings, and complications. Am J Med Genet A 2006;140:975-86.
6 Metry DW, Garzon MC, Drolet BA, Frommelt P, Haggstrom A, Hall J, et al. PHACE syndrome: current knowledge, future directions. Pediatr Dermatol 2009;26:381-98.
7 Tortori-Donati P, Fondelli MP, Rossi A, Bava GL. Intracranial contrastenhancing masses in infants with capillary haemangioma of the head and neck: intracranial capillary haemangioma? Neuroradiology 1999;41:369-75.
8 Poetke M, Frommeld T, Berlien HP. PHACE syndrome: new views on diagnostic criteria. Eur J Pediatr Surg 2002;12:366-74.
9 Ersoy S, Mancini AJ. Hemifacial infantile hemangioma with intracranial extension: a rare entity. Pediatr Dermatol 2005;22:309-13.
10 Judd CD, Chapman PR, Koch B, Shea CJ. Intracranial infantile hemangiomas associated with PHACE syndrome. AJNR Am J Neuroradiol 2007;28:25-9.
11 Poindexter G, Metry DW, Barkovich AJ, Frieden IJ. PHACE syndrome with intracerebral hemangiomas, heterotopia, and endocrine dysfunction. Pediatr Neurol 2007;36:402-6.
12 Oza VS, Wang E, Berenstein A, Waner M, Lefton D, Wells J, et al. PHACES association: a neuroradiologic review of 17 patients. AJNR Am J Neuroradiol 2008;29:807-13.
13 Rao RP, Drolet BA, Holland KE, Frommelt PC. PHACES association: a vasculocutaneous syndrome. Pediatr Cardiol 2008;29:793-9.
14 Pascual-Castroviejo I. Vascular and nonvascular intracranial malformation associated with external capillary hemangiomas. Neuroradiology 1978;16:82-4.
15 Pascual-Castroviejo I, Viano J, Moreno F, Palencia R, Martinez Fernandez V, Pascual-Pascual SI, et al. Hemangiomas of the head, neck, and chest with associated vascular and brain anomalies: a complex neurocutaneous syndrome. AJNR Am J Neuroradiol 1996;17:461-71.
16 Burrows PE, Robertson RL, Mulliken JB, Beardsley DS, Chaloupka JC, Ezekowitz RA, et al. Cerebral vasculopathy and neurologic sequelae in infants with cervicofacial hemangioma: report of eight patients. Radiology 1998;207:601-7.
17 Drolet BA, Dohil M, Golomb MR, Wells R, Murowski L, Tamburro J, et al. Early stroke and cerebral vasculopathy in children with facial hemangiomas and PHACE association. Pediatrics 2006;117:959-64.