Abstract
Vascular lesions in Von Recklinghausen’s disease also known as Neurofibromatosis type 1 (NF1), are rare but have a fatal and potentially life threatening complications such as spontaneous hemothorax. An emergent thoracotomy is indicated when there is an active bleeding associated unstable hemodynamic status. Despites surgery is laborious and unpredictable but it have a merit to stop hemorrhage. A conservative management with endovascular embolization or non-operative approach have also been reported in case of hemodynamic stability. We report two case report of spontaneous hemothorax in patient with Recklinghausen disease. A chest tube was immediately inserted for two patients. Due to continuous bleeding and hemodynamic instability (Patient 1), and the increase of pleural effusion volume (Patients 1 & 2), emergent surgery of thorax was done with favourable post-operative follow up.
Highlights
Neurofibromatosis type 1 (NF1), or Von Recklinghausen’s disease, is autosomal dominant disorder and has an incidence of 1/3000 [1, 2]
We report two case report of spontaneous hemothorax in patient with Recklinghausen disease
This disease is characterized by ‘tache café au lait spots’, it can manifest as any type of neurofibroma, characteristic bone lesions, optic nerve glioma [2,3,4]
Summary
Neurofibromatosis type 1 (NF1), or Von Recklinghausen’s disease, is autosomal dominant disorder and has an incidence of 1/3000 [1, 2]. The physical examination noted a blood pressure (10/6 mm Hg), polypnea (24c/min) and a syndrome of left fluid effusion. He had a skin and mucous membrane paleness, several café-au-lait spots and plexiform neurofibromas on the trunk, limbs. The chest X-ray showed moderate left pleural effusion. We have removed 1000 ml of black blood fluid with electrocoagulation of the lesion which was at the origin of the bleeding This lesion was located opposite the 5th intercostal space on the parietal pleura, it was a vascular lesion at the expense of the intercostal artery (Figure 3) which could be a vascular dysplasia or pseudoaneurysm. The patient was seen after 16 months of the operation with a correct follow-up X-ray
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