LATE DIAGNOSED PHACE SYNDROME BY ANEURYSMAL SUBARACHNOID HEMORRHAGE. CASE REPORT

Authors

  • Dana Mihaela TURLIUC “Grigore T. Popa” University of Medicine and Pharmacy Iasi
  • B. COSTACHESCU “Grigore T. Popa” University of Medicine and Pharmacy Iasi
  • I. POEATA “Grigore T. Popa” University of Medicine and Pharmacy Iasi
  • N. DOBRIN “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi
  • A.I. CUCU “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi
  • Anca SAVA “Grigore T. Popa” University of Medicine and Pharmacy Iasi
  • Gabriela DUMITRESC “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi
  • Claudia Florida COSTEA “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi

Abstract

PHACE syndrome is a neurocutaneous disorder coined in 1996, but the clear criteria of diagnostic were established in 2009. Even though the age of the diagnostic for the disease is during childhood, we present the case of a patient with PHACE syndrome with late diagnostic, at the age of 59-year-old by aneurysmal subarachnoid hemorrhage. The particularity of this case is represented by the paucity of the diagnostic criteria, respectively the presence of facial hemangioma and the dysplasia of the cerebral arteries at the level of Willis’s circle, associated with two aneurysms: ruptured aneurysm of the right posterior communicating artery and unruptured aneurysm of junction P1-P2 of the left posterior cerebral artery. The aim of the article is to height line the importance of early diagnostic of PHACE syndrome and the prevention of complications.

Author Biographies

  • Dana Mihaela TURLIUC, “Grigore T. Popa” University of Medicine and Pharmacy Iasi

    Faculty of Medicine
    Department of Surgery (II)
    “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi
    Department of Neurosurgery

  • B. COSTACHESCU, “Grigore T. Popa” University of Medicine and Pharmacy Iasi

    Faculty of Medicine
    Department of Surgery (II)
    “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi
    Department of Neurosurgery

  • I. POEATA, “Grigore T. Popa” University of Medicine and Pharmacy Iasi

    Faculty of Medicine
    Department of Surgery (II)
    “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi
    Department of Neurosurgery

  • N. DOBRIN, “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi

    Department of Neurosurgery

  • A.I. CUCU, “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi

    Department of Neurosurgery

  • Anca SAVA, “Grigore T. Popa” University of Medicine and Pharmacy Iasi

    Faculty of Medicine
    Department of Morpho-Functional Sciences (I)
    “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi
    Department of Pathology

  • Gabriela DUMITRESC, “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi

    Department of Pathology

  • Claudia Florida COSTEA, “Prof. Dr. N. Oblu” Emergency Clinical Hospital Iasi

    Department of Ophthalmology

References

1. Frieden IJ, Reese V, Cohen D. PHACE syndrome: the association of posterior fossa brain malfor-mations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye ab-normalities. Arch Dermatol 1996; 132(3): 307-311.
2. Cannady SB, Kahn TA, Traboulsi EI, Koltai PJ. PHACE syndrome: report of a case with a glioma of the anterior skull base and ocular malformations. Int J Pediatr Otorhinolaryngol 2006; 70(3): 561-564.
3. Dobrin I, Zapuhlih Gr, Dobrin N, Poeata I, Ianovici I, Chiriac A, Semiology of neurosurgical skull base – notes. Romanian Neurosurgery 2008; XV(2): 26-29.
4. Metry DW, Siegel DH, Cordisco MR et al. Comparison of disease severity among affected male versus female patients with PHACE syndrome. J Am Acad Dermatol 2008; 58(1): 81-87.
5. Siegel DH, Shieh JT, Kwon EK et al. Copy number variation analysis in 98 individuals with PHACE syndrome. J Invest Dermatol 2013; 133(3): 677-684.
6. Pascual-Castroviejo I. Vascular and nonvascular intracranial malformation associated with external capillary hemangiomas. Neuroradiology 1978; 16: 82-84.
7. Metry D, Heyer G, Hess C et al. PHACE Syndrome Research Conference. Consensus Statement on Diagnostic Criteria for PHACE Syndrome. Pediatrics 2009; 124(5): 1447-1456.
8. Metry DW, Dowd CF, Barkovich AJ, Frieden IJ. The many faces of PHACE syndrome. J Pediatr 2001; 139: 117-123.
9. Heyer GL, Millar WS, Ghatan S, Garzon MC. The Neurologic Aspects of PHACE: Case Report and Review of the Literature. Pediatric Neurology 2006; 35(6): 419-424.
10. Popescu E, Trandafir V, Negru D, Dobrin N, Trandafir D. Rev Med Chir Soc Med Nat Iasi 2013; 117(1): 227-232.
11. Haggstrom AN, Lammer EJ, Schneider RA, Marcucio R, Freiden IJ. Patterns of infantile hemangio-mas: New clues to hemangioma pathogenesis and embryonic facial development. Pediatrics 2006; 117: 698-703.
12. Heyer GL, Dowling MM, Licht DJ et al. The cerebral vasculopathy of PHACES syndrome. Stroke 2008; 39(2): 308-316.
13. Burrows PE, Robertson RL, Mulliken JB et al. Cerebral vasculopathy and neurologic sequelae in infants with cervicofacial hemangioma: report of eight patients. Radiology 1998; 207: 601-607.

Additional Files

Published

2017-09-30

Issue

Vol. 121 No. 3 (2017): The Medical-Surgical Journal

Section

SURGERY

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