RESUMEN
Bone involvement is relatively rare in vascular malformations. Gorham-Stout disease, also referred to as vanishing bone disease, is characterized by osteoclast activation and osteolysis caused by proliferating lymphatic endothelial cells. We present the case of a 12-year-old boy who had Gorham-Stout disease at the age of 8 years. The clinical course was complicated by pathological fractures and localized intravascular consumption coagulopathy. Sclerotherapy and embolization therapy led to normalization of the coagulation parameters and significant improvement of the clinical findings. We speculate that this effect may be attributable to the elimination of lymphatic endothelial cells.
RESUMEN
An infant presented with right upper eyelid ptosis and was subsequently diagnosed with acquired Horner syndrome. Further evaluation revealed a right-sided cervicothoracic lymphatic malformation. At 13 weeks of age, the child underwent percutaneous intracystic sclerotherapy with a mixture of sodium tetradecyl sulphate and ethanol. Twenty-one weeks after initial treatment, ophthalmic examination showed complete resolution of the blepharoptosis and pupillary miosis. Percutaneous sclerotherapy not only effectively treated the space-occupying lymphatic malformation but also reversed the Horner syndrome that was presumably induced by neural tension (more likely) or compression.
Asunto(s)
Síndrome de Horner/diagnóstico por imagen , Síndrome de Horner/terapia , Anomalías Linfáticas/diagnóstico por imagen , Anomalías Linfáticas/terapia , Escleroterapia/métodos , Blefaroptosis/diagnóstico por imagen , Blefaroptosis/terapia , Etanol/administración & dosificación , Humanos , Lactante , Recién Nacido , Tetradecil Sulfato de Sodio/administración & dosificaciónRESUMEN
An infantpresented with right upper eyelid ptosis and was subsequently diagnosed with acquired Horner syndrome. Further evaluation revealed a right-sided cervicothoracic lymphatic malformation. At 13 weeks of age, the child underwent percutaneous intracystic sclerotherapy with a mixture of sodium tetradecyl sulphate and ethanol. Twenty-one weeks after initial treatment, ophthalmic examination showed complete resolution of the blepharoptosis and pupillary miosis. Percutaneous sclerotherapy not only effectively treated the space-occupying lymphatic malformation but also reversed the Horner syndrome that was presumably induced by neural tension (more likely) or compression.