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Vol. 58. Núm. 4.
Páginas 339-349 (Abril 2003)
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Vol. 58. Núm. 4.
Páginas 339-349 (Abril 2003)
DOI: 10.1016/S1695-4033(03)78068-0
Acceso a texto completo
Hemangiomas y malformaciones vasculares cutáneas y patología asociada. Un nuevo síndrome neurocutáneo
Cutaneous hemangiomas, vascular malformations and associated disorders. a new neurocutaneous syndrome
Visitas
...
I. Pascual-Castroviejoa,
Autor para correspondencia
pascas@inves.es

Correspondencia: Servicio de Neurología Pediátrica. Hospital Univesitario La Paz. P.° de la Castellana, 261. 28046 Madrid. España.
, J.C. López-Gutiérrezb, S.I. Pascual-Pascuala, S. Rafiaa, J. Alarcón-Palacioc
a Servicio de Neurología Pediátrica. Hospital Universitario La Paz
b Unidad de Cirugía Plástica Infantil. Hospital Universitario La Paz
c Unidad de Neurología. Hospital San Rafael. Madrid. España
Información del artículo
Objetivo

Mostrar las características de este nuevo síndrome neurocutáneo, un trastorno sistémico que es el más frecuente de los síndromes neurocutáneos descritos hasta ahora y que se presenta con mayor frecuencia en mujeres.

Material y métodos

Lo constituyen el gran número de pacientes con alteraciones vasculares cutáneas en cualquier parte del cuerpo que son estudiados continuadamente por los autores desde los puntos de vista clínico y de imagen por sus problemas estéticos, funcionales y/o neurológicos.

Resultados

Los estudios clínicos han mostrado una amplia escala en la importancia de las lesiones vasculares cutáneas y su relación con diversas alteraciones internas, localizadas casi siempre en zonas subyacentes a las lesiones vasculares cutáneas, siendo las que afectan a la cabeza, cara, cuello y tórax la primera descrita y la mejor conocida. Como hallazgos más frecuentes se encuentran malformaciones del cerebelo, persistencia de la arteria trigeminal, ausencia de arteria carótida y/o vertebral, coartación aórtica y cardiopatía congénita. Una gran parte de los sujetos con este trastorno presentan retraso mental o nivel límite.

Conclusiones

Este trastorno se presenta con mayor frecuencia en mujeres y es el síndrome neurocutáneo más recientemente conocido y el de mayor prevalencia. Se asocia con una amplia patología que puede aparecer en zonas subyacentes a las alteraciones vasculares cutáneas de cualquier parte del cuerpo. Los hemangiomas cutáneos y los tumores hemangiomatosos subyacentes, al igual que otras alteraciones vasculares asociadas, pueden crecer e involucionar en paralelo de forma espontánea y natural, sin influencia terapéutica, hasta quedar los vasos completamente ocluidos mientras que las malformaciones vasculares no sufren ningún cambio evolutivo ni involutivo. Este nuevo trastorno neurocutáneo ya ha recibido diversas denominaciones, como síndrome de Pascual-Castroviejo tipo II y PHACE.

Palabras clave:
Síndrome neurocutáneo
Trastorno neurocutáneo
Hemangiomas
Malformaciones vasculares
Síndrome de Pascual-Castroviejo tipo II
PHACE
Malformaciones del cerebelo
Retraso mental
Objective

Cutaneous hemangiomas and vascular malformations constitute a systemic disease that is the most frequent of the neurocutaneous syndromes reported to date. It appears more frequently in women than in men. The aim of this study was to describe the characteristics of this new neurocutaneous syndrome.

Material and methods

We studied a large number of patients who presented cutaneous hemangiomas and/or vascular malformations located anywhere on the body. The patients underwent periodic clinical follow-up and imaging studies due to their aesthetic, functional and/or neurological problems.

Results

Clinical evaluation revealed a wide range in the size and severity of cutaneous vascular lesions and in their relationship with various internal anomalies, which were almost always located in the areas underlying the cutaneous vascular lesions. Those situated in the head, face, neck and chest were first discovered and are the best known. The most frequent findings were cerebellar malformations, persistence of the trigeminal artery, absence of the carotid or vertebral arteries and congenital heart disease. Many of the patients with this syndrome had mental retardation or borderline mental retardation.

Conclusions

This disorder appears more frequently in females than in males. It is the most recently described neurocutaneous syndrome and is also the most prevalent. This syndrome is associated with numerous disorders that can be located in the areas underlying the cutaneous vascular lesions anywhere on the body. Cutaneous hemangiomas and subjacent hemangiomatous tumors, like other associated vascular abnormalities, can show a parallel, spontaneous increase or decrease in size without treatment. Complete occlusion of the vessels can occur while vascular malformations show no progression or involution. This new neurocutaneous syndrome is also known as Pascual- Castroviejo type II syndrome and PHACE.

Key words:
Neurocutaneous syndrome
Neurocutaneous disease
Hemangiomas
Vascular malformations
Pascual-Castroviejo type II syndrome
PHACE
Cerebellar malformations
Mental retardation
El Texto completo está disponible en PDF
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