Phace sendromu (Olgu)

Phace syndrome (Case)

Background.- Hemangiomas are the benign tumor of vascular endothelium. Unlike vascular malformations, they are rarely associated with structural abnormalities. A new neurocutaneous syndrome (PHACE syndrome) has been recently described in cases with large facial hemangioma associated with structural systemic abnormalities.Observation.- We report an infant with large facial hemangioma associated with posterior fossa brain malformations ( Dandy-Walker malformation and left cerebellar hemisphere hypoplasia), internal carotid artery anomalies, silent patent ductus arteriosus, strabismus and small hepatic caverneous hemangiomas. To our knowleged there was no documented case of PHECE syndrome in Turkey.

___

  • 1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: A classification based endothelial characteristic. Plast Reconstr Surg 1982; 69: 412-420.
  • 2. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: Clinical application of a new classification. J Pediatr Surg 1983; 18: 894-899.
  • 3. Burns AJ, Kaplan LC, Mulliken JB. Is there an association between hemangioma and syndromes with dysmorphic features. Pediatrics 1991; 88: 1257-1267.
  • 4. Enjolras O, Gelbert F. Superficial hemangiomas: associations and management. Pediatric Dermatology 1997; 14: 173-179.
  • 5. Reese V, Frieden IJ, Paller AS. Et al. Association of facial hemangiomas with Dandy-Walker and other posterior fossa malformations. J Pediatr 1993; 122: 379-384.
  • 6. Goldberg NS, Hebert AA, Esterly NB. Sacral hemangiomas and multipl congenital abnormalities. Arch Dermatol 1986; 122: 684-687.
  • 7. Schneeweiss A, Blieden LC, Shem-Tov A et al. Coarctation of the aorta with congenital hemangioma of the face and neck and aneurysm or dilatation of a subclavian or innominate artery. Chest 1982; 82: 186-187.
  • 8. 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: 307-311.
  • 9. Hırsch JF, Kahn AP, Renier D et al. The Dandy-Walker malformation. A review of 40 cases. J Neurosurg 1984; 61: 515-522.
  • 10. Opitz JM, Gilbert EF. Cns anomalies and the mid-line as a ‘developmental field’. Am J Med Genet. 1982; 12: 177-186.
  • 11. Pascual-Castroviejo I. The association of extracranial and intracranial vascular malformations in cheldren. Can J Neurol Sci 1985; 12: 139-148.
  • 12. Brett EM: Neurocutaneous syndromes. Paediatric neurology’de. Ed. E M Brett, 2nd edition. Edinburgh, Churchill Livingstone, 1991; 571-591.