Child's Nervous System

, Volume 27, Issue 4, pp 617–625 | Cite as

Pascual-Castroviejo type II syndrome (P-CIIS). Importance of the presence of persistent embryonic arteries

  • Ignacio Pascual-Castroviejo
  • Juan Alvarez-Linera
  • Juan Coya
  • Juan Viaño
  • Samuel-Ignacio Pascual-Pascual
  • Ramón Velázquez-Fragua
  • Juan-Carlos López-Gutiérrez
Original Paper

Abstract

Introduction

Cutaneous hemangioma and vascular malformation are two vascular abnormalities frequently associated with absence or hypoplasia of one or both carotid and/or vertebral arteries, presence of persistent embryonic arteries, especially the trigeminal, cerebellar malformations, and coarctation of the aortic arch and/or congenital cardiopathy. This disease is known as Pascual-Castroviejo type II syndrome (P-CIIS) and by the acronym PHACE.

Material and methods

Three patients (two females and one male) with facial hemangioma are studied during the first years of age by magnetic resonance angiography (MRA) and their vascular evolution to adult age followed through several MRA controls.

Results

All the three patients showed persistence of the trigeminal artery associated to other intra- and extracranial vascular abnormalities of type hemangioma or hemangiomatous arteries that presented progressive involution with decreased arterial caliber without appearing cerebrovascular stroke or hypoxic zones because, at the same time, collateral vascularization appeared through connections between the embryonic arteries and the peripheral branches of the internal carotids or connections between branches of the external and internal carotids. Only one patient had obstruction of a branch of the left middle cerebral artery after 3 days, with gastroenteritis with elevated fever at 17 months of life that caused parenchymal infarct in the left cerebral region supplied by the obstructed artery.

Conclusions

The presence of embryonic arteries, especially the trigeminal, and connections between branches of the internal and external carotids, mainly through the internal maxillary and ophthalmic arteries, ensure the cerebral supply in the P-CIIS despite the progressive involvement of the cerebral arteries.

Keywords

Pascual-Castroviejo type II syndrome (P-CIIS) PHACE Hemangioma Trigeminal artery Embryonic cerebral arteries Collateral cerebral supply 

References

  1. 1.
    Pascual-Castroviejo I (1978) Vascular and nonvascular intracranial malformations associated with external capillary hemangiomas. Neuroradiology 16:82–84PubMedCrossRefGoogle Scholar
  2. 2.
    Pascual-Castroviejo I (1985) The association of extracranial and intracranial vascular malformations in children. Can J Neurol Sci 12:139–148PubMedGoogle Scholar
  3. 3.
    Mulliken JB, Glowacki J (1982) Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg 69:412–420PubMedCrossRefGoogle Scholar
  4. 4.
    Pascual-Castroviejo I, Vélez A, Pascual-Pascual SI, Roche MC, Villarejo F (1991) Dandy–Walker malformation: analysis of 38 cases. Childs Nerv Syst 7:88–97PubMedGoogle Scholar
  5. 5.
    Pascual-Castroviejo I, Viaño J, Pascual-Pascual SI, Martínez V (1995) Facial hemangioma, agenesis of the internal carotid artery and dysplasia of cerebral cortex: case report. Neuroradiology 37:692–695PubMedCrossRefGoogle Scholar
  6. 6.
    Pascual-Castroviejo I, Viaño J, Moreno F, Palencia R, Martínez-Fernandez V, Pascual-Pascual SI et al (1996) Hemangiomas of the head, neck, and chest with associated vascular and brain anomalies: a complex neurocutaneous syndrome. AJNR Am J Neuroradiol 17:461–471PubMedGoogle Scholar
  7. 7.
    Frieden IJ, Reese V, Cohen D (1996) PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 132:307–311PubMedCrossRefGoogle Scholar
  8. 8.
    Léauté-Labréze C, de la Roque ED, Ubriche T, Boralevi F, Tambo JB, Tateb A (2008) Propanolol for severe hemangiomas of infancy. N Engl J Med 358:2649–2651PubMedCrossRefGoogle Scholar
  9. 9.
    Torres-Mohedas J, Verdú A, Vidal B, Jadraque R (2001) Presentación conjunta de hemangioma facial, malformación de fosa posterior e hipoplasia carótido-vertebral (síndrome de Pascual-Castroviejo tipo II): aportación de dos nuevos casos. Rev Neurol 32:50–54PubMedGoogle Scholar
  10. 10.
    Bélanger-Quintana A, Aparicio Meix JM, Quintana-Castilla A (2002) Síndrome de Pascual-Castroviejo II: asociación de hemangioma facial, alteración de fosa posterior y cardiopatía congénita. An Esp Pediatr 57:588–589PubMedGoogle Scholar
  11. 11.
    Mohammadi M, Mohebbi MR, Holden KR (2004) Facial hemangioma and associated malformations: a case report. Neuropediatrics 35:194–197PubMedCrossRefGoogle Scholar
  12. 12.
    Pascual-Castroviejo I, Santolaya JM, Lopez Martin V, Rodriguez-Costa T, Tendero A, Mulas F (1975) Cerebro-facio-thoracic dysplasia: report of three cases. Dev Med Child Neurol 17:343–351 (RE syndrome 3034POSSUM System)PubMedCrossRefGoogle Scholar
  13. 13.
    Pascual-Castroviejo I, Lopez-Gutierrez JC (2007) Cutaneous hemangioma associated with persistence of the trigeminal and both proatlantal arteries. J Child Neurol 22:337–340PubMedCrossRefGoogle Scholar
  14. 14.
    Pascual-Castroviejo I, Pascual-Pascual SI, Delgado J (2007) Cutaneous hemangiomas and vascular abnormalities: persistence of embryonic vascularization. AJNR Am J Neuroradiol 28:390–391PubMedCrossRefGoogle Scholar
  15. 15.
    Pascual-Castroviejo I, Pascual-Pascual SI, Lopez-Gutierrez JC, Velázquez-Fragua R, Viaño J (2007) Facial hemangioma and hemispheric migration disorder: presentation of 5 patients. AJNR Am J Neuroradiol 28:1609–1612PubMedCrossRefGoogle Scholar
  16. 16.
    Pascual-Castroviejo I, Pascual-Pascual SI, Viaño J, Lopez-Gutierrez JC, Palencia R (2010) Major and minor arterial malformations in patients with cutaneous vascular abnormalities. J Child Neurol 25:587–598PubMedCrossRefGoogle Scholar
  17. 17.
    Pascual-Castroviejo I, Viaño J, Pascual-Pascual SI, Quiñones D (2009) Congenital and evolving vascular disorders associated with cutaneous hemangiomas: case report. Neuropediatrics 40:148–151PubMedCrossRefGoogle Scholar
  18. 18.
    Yilmaz E, Erhan I, Dogan T (1995) Primitive persistent carotid-basilar and carotid-vertebral anastomose: a report of seven cases and a review of the literature. Clin Anat 8:36–43PubMedCrossRefGoogle Scholar
  19. 19.
    Teal TS (1972) Anomalous branches of the internal carotid arteries. Radiology 106:567–573Google Scholar
  20. 20.
    Pascual-Castroviejo I, Pascual-Pascual SI, Velázquez-Fragua R (2008) Origen anormal de la arteria cerebral media en la arteria basilar. Neurologia 23:536–537Google Scholar
  21. 21.
    Mall FP (1912) Determination of the age of the embryos and fetuses. In: Keibel VI and Mall FP (eds) Manual of human embryology.V.1. Philadelphia, 180–201Google Scholar
  22. 22.
    Streeter GL (1918) The development alterations in the vascular system of the brain of human embryo. Contrib Embryol 8:5–38Google Scholar
  23. 23.
    Congdon ED (1922) Transformation of aortic arch system during the development of the human embryo. Contrib Embryol 14:47–110Google Scholar
  24. 24.
    Padget DH (1948) The development of the cranial arteries in the human embryo. Contrib Embryol 32:205–262Google Scholar
  25. 25.
    Pascual-Castroviejo I, Viaño J, Pascual-Pascual SI, Martinez V (2003) Do cutaneous hemangiomas and internal vascular anomalies follow the same evolution? Neurology 61:140–141PubMedGoogle Scholar
  26. 26.
    Brouillard P, Vikkula M (2003) Vascular malformations: localized defects in vascular morphogenesis. Clin Genet 63:340–351PubMedCrossRefGoogle Scholar
  27. 27.
    Rissau W (1997) Mechanisms of angiogenesis. Nature 386:671–674CrossRefGoogle Scholar
  28. 28.
    Breugen CC, van der Horst CMAM, Hennekam RCM (2001) Progress toward understanding vascular malformations. Plast Reconstr Surg 107:1509–1523CrossRefGoogle Scholar
  29. 29.
    Folkman J, D’Amore PA (1996) Blood vessels formation: what is its molecular basis? Cell 87:1153–1155PubMedCrossRefGoogle Scholar
  30. 30.
    Hanahan D (1997) Signaling vascular morphogenesis and maintenance. Science 277:48–50PubMedCrossRefGoogle Scholar
  31. 31.
    Carmilet P (2000) Mechanisms of angiogenesis and arteriogenesis. Nat Med 6:389–395CrossRefGoogle Scholar
  32. 32.
    Poole TJ, Kinkelstein EB, Cox CM (2001) The role of FGF and VEGF in angioblast induction and migration during vascular development. Dev Dyn 220:1–17PubMedCrossRefGoogle Scholar
  33. 33.
    Jain RK (2003) Molecular regulation of vessel maturation. Nat Med 9:685–693PubMedCrossRefGoogle Scholar
  34. 34.
    Cohen MM Jr (2006) Vascular update: morphogenesis tumors, malformations, and molecular dimensions. Am J Med Genet 140:2013–2038PubMedCrossRefGoogle Scholar
  35. 35.
    Takahashi K, Mulliken JB, Kozakewich HPW, Rogers RA, Folkman J, Ezekowitz RAB (1994) Cellular markers that distinguish the phases of hemangioma during infancy and childhood. J Clin Invest 93:2357–2364PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Ignacio Pascual-Castroviejo
    • 6
    • 1
  • Juan Alvarez-Linera
    • 2
  • Juan Coya
    • 3
  • Juan Viaño
    • 4
  • Samuel-Ignacio Pascual-Pascual
    • 1
  • Ramón Velázquez-Fragua
    • 1
  • Juan-Carlos López-Gutiérrez
    • 5
  1. 1.Pediatric Neurology ServiceUniversity Hospital La PazMadridSpain
  2. 2.Imagen UnitSanatorio Ruber InternationalMadridSpain
  3. 3.Nuclear Medicine UnitUniversity Hospital La PazMadridSpain
  4. 4.Image UnitRosario ClinicMadridSpain
  5. 5.Pediatric Plastic Surgery SectionUniversity Hospital La PazMadridSpain
  6. 6.MadridSpain

Personalised recommendations