Association of Cutaneous Red-to-Purple Hemangiomas with Leptomeningeal Hemangiomas. A Clinical Study of Two Patients

Abstract

Cutaneous hemangioma is a benign vascular tumor of infancy with an initial proliferating period that appears between 1 to 2 weeks of life, extends during 18 months to 2 years of life, and then slowly regresses during several years until it disappears completely. They are characterized by endothelial cell proliferation followed by diminishing hyperplasia and progressive fibrosis. Vascular malformations are present at birth, grow commensurately with the child, and are characterized histologically by a normal rate of endothelial cell turnover, flat endothelium, thin (normal) basal membrane and normal mast cells. These cutaneous anomalies are commonly associated with cerebellar malformations, main cerebral arteries anomalies, congenital cardiac anomalies and/or coarctation of the aorta and persistence of embryonic arteries. Cutaneous hemangiomas can be associated with intracranial or extracranial hemangiomas that regress at the same time as the cutaneous hemangiomas. Cutaneous hemangiomas may show different types of color. Cutaneous red-to-purple hemangiomas are uncommon and their bright-red color is evident from the first weeks of life and remains unaltered until the hemangioma disappears. The intracranial angiographic studies in our series of more than 50 cases with facial hemangioma showed that patients with red-to-purple hemangiomas are commonly associated with localized leptomeningeal hemangiomas either in the ipsilateral or contralateral side. These leptomingeal hemangiomas were visualized only by MR enhanced with gadolinium. Involution of the cutaneous and leptomeningeal hemangiomas seems to occur simultaneously as in other types of external and internal hemangiomas.

Key words

Pascual-Castroviejo type II syndrome (P-CIIS) - PHACES - cutaneous red-to-purple hemangioma - leptomeningeal hemangioma - neurocutaneous disorders - vascular malformations

References

• 1 Bélanger-Quintana A, Aparicio Meix JM, Quintana-Castilla A. Síndrome de Pascual-Castroviejo II: asociación de hemangioma facial, alteración de fosa posterior y cardiopatía congénita.  An Esp Pediatr. 2002;  57 582-594
• 2 Burrows PE, Robertson RL, Mulliken JB. et al . Cerebral vasculopathy and neurologic sequealae in infants with cervicofacial hemangioma: Report of eight patients.  Radiology. 1998;  207 601-607
• 3 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
• 4 Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics.  Plast Reconstr Surg. 1982;  69 412-420
• 5 Oza VS, Wang E, Berenstein A. et al . PHACES association: a neuroradiologic review of 17 patients.  AJNR Am J Neuroradiol. 2008;  29 807-813
• 6 Pascual-Castroviejo I. Vascular and nonvascular intracranial malformations associated with external capillary hemangiomas.  Neuroradiology. 1978;  16 82-84
• 7 Pascual-Castroviejo I, Viaño J, Moreno F. et al . Hemangiomas of the head, neck, and chest with associated vascular and brain anomalies: A complex neurocutaneous syndrome.  AJNR Am J Neuroradiol. 1996;  17 461-471
• 8 Pascual-Castroviejo I, Pascual-Pascual SI, Rafia S. et al . Hemangiomas y malformaciones vasculares cutáneas e intracraneales (síndrome de Pascual-Castroviejo tipo II).  Presentación de un caso. Rev Neurol. 2002;  35 1034-1036
• 9 Pascual-Castroviejo I, Viaño J, Pascual-Pascual SI. et al . Do cutaneous hemangiomas and internal vascular anomalies follow the same evolution?.  Neurology. 2003;  61 140-141
• 10 Pascual-Castroviejo I, Pascual-Pascual SI, Garcia-Guereta L. et al . Cutaneous thoracic hemangioma and internal vascular anomalies.  J Pediatr Neurol. 2005;  3 103-106
• 11 Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez Fragua R. et al . Hemangiomas y malformaciones vasculares cutáneas y patología asociada (síndrome de Pascual-Castroviejo tipo II). Presentación de 41 pacientes.  Rev Neurol. 2005;  41 223-236
• 12 Pascual-Castroviejo I, Pascual-Pascual SI, Velazquez-Fragua R. et al . Sturge-Weber syndrome. Study of 55 patients.  Can J Neurol Sci. 2008;  35 301-307
• 13 Torres-Mohedas J, Verdú A, Vidal B. et al . 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. 2001;  32 50-54
• 14 Tortori-Donati P, Fondelli MP, Rossi A. et al . Intracranial contrast enhancing masses in infants with capillary hemangioma of the head and neck: intracranial capillary haemangioma?.  Neuroradiology. 1999;  41 369-375
• 15 Taveras JM. Multiple progressive intracranial arterial occlusions: A syndrome of children and young adults.  Am J Roentgenol Rad Ther Nucl Med. 1969;  106 235-268
• 16 Wright TL, Bresnan MJ. Radiation-induced cerebrovascular disease in children.  Neurology. 1978;  26 540-543

Correspondence