Massive spontaneous hemothorax, giant intrathoracic meningocele, and kyphoscoliosis in neurofibromatosis type 1

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Massive Spontaneous Hemothorax, Giant Intrathoracic Meningocele, and Kyphoscoliosis in Neurofibromatosis Type 1

Neurofibromatosis type 1 (NF-1) is a heterogeneous autosomal dominant disease with an incidence ranging from 1 in 2500 to 1 in 3000. Rare intrathoracic vascular disorders resulting in massive spontaneous hemothorax with fatal consequences may occur in these patients, so also are various types of skeletal dysplasia which may result in dramatic presentations, posing management challenges to the a...

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Chest wall bleeding with giant intrathoracic meningocele in neurofibromatosis type 1.

We report the case of a 66-year-old woman with neurofibromatosis type 1 who developed chest wall bleeding with severe scoliosis and a giant intrathoracic meningocele. She was brought to the emergency department with acute-onset of left-sided chest pain and clinical signs of hypovolemia. Bleeding control was difficult in the first operation because the tissue was friable and there were multiple ...

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Spontaneous Hemothorax in a Patient with Neurofibromatosis Type 1: A Case Report

Background: Neurofibromatosis type1 (NF-1) is a hereditary autosomal dominant disease that is accompanied by complications, such as benign and malignant tumors and vascular involvement, including pulmonary hypertension, artery stenosis, and pulmonary artery aneurysm. Spontaneous hemothorax is a rare and lethal complication of NF-1 due to vasculopathy as stenosis or aneurysmal modifications of l...

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Giant Intrathoracic Meningocele and Breast Cancer in a Neurofibromatosis Type I Patient

Intrathoracic meningoceles are relatively rare entities found in patients with neurofibromatosis type I (NF1). Given that both the BRCA1 and NF-1 genes are located on the same long arm of chromosome 17, one would expect concurrence of neurofibromatosis and breast cancer. However, incidence of such co-disorders is very rare in the literature. Here, the authors report a case of a 50-year-old fema...

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Spontaneous Massive Hemothorax in a Patient with Neurofibromatosis Type 1 with Successful Transarterial Embolization

Vascular involvement in neurofibromatosis type 1 is rare but has the potential to be fatal. We report a case of a patient with spontaneous rupture of a left intercostal artery aneurysm, which presented as a massive left hemothorax and was successfully treated by transarterial coil embolization.

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ژورنال

عنوان ژورنال: Journal of Surgical Technique and Case Report

سال: 2014

ISSN: 2006-8808

DOI: 10.4103/2006-8808.135151