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Vol. 58. Núm. 6.
Páginas 599-603 (junio 2003)
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Vol. 58. Núm. 6.
Páginas 599-603 (junio 2003)
Acceso a texto completo
Heterogenicidad del síndrome de Gorham-Stout: asociación a malformaciones linfáticas y venosas
Heterogenicity of gorham-stout syndrome: association with lymphatic and venous malformations
Visitas
17237
I. Somoza Argibay**
Autor para correspondencia
isomoza@canalejo.org

Correspondencia: Servicio de Cirugía Pediátrica. Hospital Materno-Infantil Teresa Herrera.Complejo Hospitalario Juan Canalejo.As Xubias, 84. 15006 A Coruña. España.
, M. Díaz González, L. Martínez Martínez, Z. Ros Mar, J.C. López-Gutiérrez
Unidad de Cirugía Plástica Infantil. Servicio de Cirugía Pediátrica.Hospital Materno Infantil La Paz. Madrid. España.
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Información del artículo
Introducci ón

El síndrome de Gorham-Stout constituye una rara enfermedad de etiología desconocida que se caracteriza por osteólisis rápidamente progresiva y proliferación microscópica de vasos anormales. Presentamos 2 casos clínicos asociados a malformaciones linfaticovenosas.

Casos clínicos

El primer caso es un varón de 5 años afecto de linfangiomatosis generalizada de evolución desfavorable, con importante afectación pleural y lesiones osteolíticas. El segundo caso se trata de una niña de 5 años diagnosticada de síndrome de Klippel-Trénaunay con importante afectación ósea en miembros inferiores y fractura patológica secundaria.

Conclusiones

El síndrome de Gorham-Stout puede presentarse ocasionalmente asociado a distintas malformaciones linfaticovenosas. La afectación linfática del hueso puede provocar osteólisis y resorción ósea.

Palabras clave:
Síndrome de Gorham-Stout
Malformaciones linfaticovenosas
Resorción ósea
Osteólisis
Interferón alfa 2b(IFN-a2b)
Introduction

Gorham-Stout syndrome is a rare disorder of unknown etiology characterized by osteolysis and microscopic proliferation of abnormal vessels. We report two cases of this syndrome associated with lymphatic and venous malformations.

Case reports

The first case is a 5-year-old boy with disseminated lymphangiomatosis of poor prognosis, with significant pleural involvement and osteolytic lesions. The second case is a 5-year-old girl with a diagnosis of Klippel-Trenaunay syndrome with significant skeletal involvement of the lower extremities and secondary pathological fracture.

Conclusions

Gorham-Stout syndrome may occasionally be associated with various lymphatic and venous malformations. Osteolysis and bone resorption may be induced by lymphatic bone involvement.

Key words:
Gorham-Stout syndrome
Lymphatic venous malformations
Bone resorption
Osteolysis
Alpha-2b interferon(IFN-a2b)
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