Hemotórax masivo espontáneo en pacientes con neurofibromatosis tipo 1 / Massive spontaneous hemothorax in patients with neurofibromatosis type 1

El hemotórax espontáneo es una entidad poco frecuente que en los pacientes con antecedentes de neurofibromatosis tipo 1 (NF1) puede ser debido a malformaciones vasculares intratorácicas que predisponen a formaciones aneurismáticas o bien a sangrado de tumores torácicos. Esto ocurre en muy raras ocasiones, con solo 53 casos reportados en la bibliografía desde 1975. Presentamos dos casos: el primero, un varón de 73 años con hemotorax derecho secundario a un neurofibroma intercostal; el segundo, una mujer de 35 años con hemotórax izquierdo secundario a un neurofibroma que lesionaba la arteria mamaria interna. Revisando la bibliografía, el 61,8% son mujeres con una edad media de 43,9 años, y con cierta predisposición a la localización en el hemitórax izquierdo (56,4%). El paquete intercostal es el más frecuentemente involucrado en el sangrado. La cirugía ha sido el tratamiento realizado con mayor frecuencia (58,2%), si bien la embolización a través de arteriografía selectiva es un tratamiento válido. Un 30,9% fallecieron durante el episodio y aquellos tratados con cirugía o embolización arteriográfica tienen una mayor supervivencia que aquellos pacientes a los que no recibieron tratamiento invasivo o bien solo se les colocó un drenaje torácico (p = 0,02)

Spontaneous hemothorax is an uncommon event that can occur in patients with a history of neurofibromatosis type 1 because of intrathoracic vascular malformations that predispose to aneurysms or bleeding from thoracic tumors. Only 53 cases of this rare association have been reported in the literature since 1975. We described 2 cases: one patient was a 73-year-old man with a right hemothorax secondary to an intercostal neurofibroma; the other was a 35-year-old woman with a left hemothorax secondary to a neurofibroma that compromised the internal mammary artery. Our review of the literature found that 61.8% of cases involved women with a mean age of 43.9 years. There was a certain tendency toward left-sided (56.4%) hemothorax, and the intercostal space was the most common site of bleeding. Treatment was most often surgical (58.2%) in reported cases, although selective artery embolization is also a valid choice. Exitus occurred during 30.9% of the reported episodes, and survival was higher in patients who were treated with surgery or arterial embolization than in those in whom only a thoracic drain was placed or who received no invasive treatment (P=.02)

Massive spontaneous hemothorax in patients with neurofibromatosis type 1. / Hemotórax masivo espontáneo en pacientes con neurofibromatosis tipo 1.

OBJECTIVES: Spontaneous hemothorax is an uncommon event that can occur in patients with a history of neurofibromatosis type 1 because of intrathoracic vascular malformations that predispose to aneurysms or bleeding from thoracic tumors. Only 53 cases of this rare association have been reported in the literature since 1975. We described 2 cases: one patient was a 73-year-old man with a right hemothorax secondary to an intercostal neurofibroma; the other was a 35-year-old woman with a left hemothorax secondary to a neurofibroma that compromised the internal mammary artery. Our review of the literature found that 61.8% of cases involved women with a mean age of 43.9 years. There was a certain tendency toward left-sided (56.4%) hemothorax, and the intercostal space was the most common site of bleeding. Treatment was most often surgical (58.2%) in reported cases, although selective artery embolization is also a valid choice. Exitus occurred during 30.9% of the reported episodes, and survival was higher in patients who were treated with surgery or arterial embolization than in those in whom only a thoracic drain was placed or who received no invasive treatment (P=.02).

ES: El hemotórax espontáneo es una entidad poco frecuente que en los pacientes con antecedentes de neurofibromatosis tipo 1 (NF1) puede ser debido a malformaciones vasculares intratorácicas que predisponen a formaciones aneurismáticas o bien a sangrado de tumores torácicos. Esto ocurre en muy raras ocasiones, con solo 53 casos reportados en la bibliografía desde 1975. Presentamos dos casos: el primero, un varón de 73 años con hemotorax derecho secundario a un neurofibroma intercostal; el segundo, una mujer de 35 años con hemotórax izquierdo secundario a un neurofibroma que lesionaba la arteria mamaria interna. Revisando la bibliografía, el 61,8% son mujeres con una edad media de 43,9 años, y con cierta predisposición a la localización en el hemitórax izquierdo (56,4%). El paquete intercostal es el más frecuentemente involucrado en el sangrado. La cirugía ha sido el tratamiento realizado con mayor frecuencia (58,2%), si bien la embolización a través de arteriografía selectiva es un tratamiento válido. Un 30,9% fallecieron durante el episodio y aquellos tratados con cirugía o embolización arteriográfica tienen una mayor supervivencia que aquellos pacientes a los que no recibieron tratamiento invasivo o bien solo se les colocó un drenaje torácico (p = 0,02).

Multidisciplinary management of Paget-Schroetter syndrome. A case series of eight patients.

Paget-Schroetter syndrome (PSS) in the context of upper extremity deep venous thrombosis (DVT) is an uncommon but potentially very serious condition affecting young, healthy adults, in which secondary post-thrombotic syndrome (PTS) can be a complication with major implications. The best treatment option remains controversial, with current guidelines recommending anticoagulation for at least 3 months. However, an incidence of PTS of approximately 50% after 6 months, 30% after 1 year and 25% after 2 years has been found using this therapeutic approach. Consequently, specialized units recommend local thrombolysis and early decompressive surgery. We describe a series of eight cases treated in this way. None of the patients showed signs of complications, and an early return to regular activities with no PTS was observed in 90% of cases.