Neurofibromatosis 1 and massive hemothorax: a fatal combination

D’Errico, Stefano; Martelloni, Massimo; Cafarelli, Francesco Pio; Guglielmi, Giuseppe

doi:10.1007/s12024-018-0001-z

Cited by 9 publications

(4 citation statements)

References 8 publications

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“…Cardiovascular disease, haemorrhage, and embolism associated with vasculopathy are frequent causes of death in adult patients with NF1 (Zoller et al 1995 ). NF-V includes aneurysms, aneurysmal dilatation, stenosis, rupture, and arteriovenous malformations (D’Errico et al 2018 ; Hinsch et al 2008 ; Falcone et al 2010 ). Among these, aneurysmal dilatation is the most common form of vasculopathy (D’Errico et al 2018 ).…”

Section: Discussionmentioning

confidence: 99%

“…NF-V includes aneurysms, aneurysmal dilatation, stenosis, rupture, and arteriovenous malformations (D’Errico et al 2018 ; Hinsch et al 2008 ; Falcone et al 2010 ). Among these, aneurysmal dilatation is the most common form of vasculopathy (D’Errico et al 2018 ). Additionally, the involvement of multiple vessels is a common characteristic of NF-V (Friedman et al 2002 ).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Spontaneous rupture of the pancreatic arcade artery caused by neurofibromatosis type 1 successfully treated using emergency transcatheter arterial embolization, partial intra-aortic balloon occlusion, and stent graft placement: a case report and review of the literature

et al. 2020

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Background: Vascular abnormalities in neurofibromatosis type 1 (NF1) are rare, but are the second leading cause of death in persons with NF1. In NF1 vasculopathy (NF-V), fatal bleeding due to a spontaneous arterial rupture sometimes occurs. Ruptured extracranial arteries in patients with NF1 often involve thoracic vessels, such as the intercostal and subclavian arteries; very few reports exist regarding the abdominal region. Herein, we present the first case of intraperitoneal bleeding due to spontaneous pancreatic arcade artery (PAA) rupture associated with NF1, successfully treated by transcatheter arterial embolization (TAE) combined with stent-graft placement and partial intra-aortic balloon occlusion (IABO). Case presentation: A 40-year-old woman complained of back and abdominal pain. Upon admission, her blood pressure was 85/41 mmHg and heart rate was 129 beats/min. Computed tomography (CT) showed large intraperitoneal bleeding due to PAA rupture. After CT scanning, her systolic blood pressure decreased to 50 mmHg. Therefore, we performed emergency TAE with partial IABO. She was treated by TAE of the anterior superior pancreaticoduodenal artery, anterior inferior pancreaticoduodenal artery, and inferior pancreaticoduodenal artery. However, even after TAE, minor extravasation around the superior mesenteric artery continued, and her vital signs remained unstable. Stent-graft placement was selected to stop the haemorrhage, preserving normal blood flow of the superior mesenteric artery trunk. Excellent patency of the stent graft was confirmed on follow-up CT, and she was discharged on postoperative day 56.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Spontaneous rupture of the pancreatic arcade artery caused by neurofibromatosis type 1 successfully treated using emergency transcatheter arterial embolization, partial intra-aortic balloon occlusion, and stent graft placement: a case report and review of the literature

et al. 2020

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“…There were 3 out of 16 patients who died (19%) during care in the cases reviewed. Two died during thoracotomy and 1 patient died before any treatment [9,11,12].…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Hemothorax in a Patient with von Recklinghausen’s Disease: A Case Report and Review of the Literature

et al. 2019

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Patient: Male, 45 Final Diagnosis: Spontaneous hemorhorax Symptoms: Cough • worned dyspnea • yellow expectoration Medication: — Clinical Procedure: Chest tube application Specialty: Surgery Objective: Rare co-existance of disease or pathology Background: Von Recklinghausen’s disease, also known as Type 1 neurofibromatosis (NF1), is a genetic disorder characterized by skin tumors, neurofibromas of multiple organs and vascular abnormalities. Spontaneous thoracic hemorrhage is a rare but potentially fatal consequence of this disorder. After a review of the literature over the last 10 years and on the basis of a case study, the aim of this study was to report the challenges of management of this pathology. Case Report: We report a rare case of a 45-years-old male with a medical history of neurofibromatosis who complained of a 3-day history of progressive dyspnea. At his admission to the Emergency Department, the patient was hemodynamically stable. A chest computed tomography (CT) scan showed a large left hemothorax with mediastinal shift to the right without active bleeding. A chest tube was introduced, and conservative treatment was followed. Another CT scan performed 2 days later revealed a middle lobar pulmonary embolism on the opposite side. A full treatment of anticoagulation was administered, and the patient was released after 8 days of hospitalization. Three weeks later, a new chest CT scan indicated the absence of vascular aneurysm or source for hemothorax. Conclusions: Our systematic literature review found 15 articles which were described as early as 2005. To our knowledge, endovascular treatment produces the best immediate successful result (100%) and may be used in adjunction with video-assisted thorax surgery (VATS) or thoracic drainage to optimize outcomes. In the present case, conservative treatment showed a good result despite anticoagulation for pulmonary embolism. The endovascular approach seems to be the most promising, but treatment needs to be tailored to each individual patient.

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“…Hemothoraces have been reported as a rare complication of this disease, typically due to hemorrhage from neurofibromata invasion of thoracic vessels or direct bleeding from the tumors 4,5. Spontaneous hemothoraces due to aneurysmal rupture have been reported in the literature 6–9. An overview of the cases with extracranial vertebral artery aneurysms in patients with NF1, their presenting complaints, interventions, and outcome are provided in Table 1.…”

Section: Discussionmentioning

confidence: 99%

Fatal Tension Hemothorax Combined With Exanguination: A Rare Complication of Neurofibromatosis

Bidad

Hall

Blohm

2019

Clinical Practice and Cases in Emergency Medicine

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Neurofibromatosis (NF) is a common autosomal dominant disorder that can be subdivided into type 1, type 2, and schwannomatosis. Patients with NF1 typically develop café-au-lait spots, scoliosis, and benign neurofibromas. In addition, NF1 predisposes to vascular complications including stenosis, arterial ectasia, and aneurysms. Here, we report the case of an otherwise healthy 32-year-old man who developed a fatal tension hemothorax due to vertebral artery aneurysm rupture. Based on the available literature, we discuss the presentation, workup, and available therapeutic approaches to this complication of neurofibromatosis.

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Neurofibromatosis 1 and massive hemothorax: a fatal combination

Cited by 9 publications

References 8 publications

Spontaneous rupture of the pancreatic arcade artery caused by neurofibromatosis type 1 successfully treated using emergency transcatheter arterial embolization, partial intra-aortic balloon occlusion, and stent graft placement: a case report and review of the literature

Spontaneous rupture of the pancreatic arcade artery caused by neurofibromatosis type 1 successfully treated using emergency transcatheter arterial embolization, partial intra-aortic balloon occlusion, and stent graft placement: a case report and review of the literature

Spontaneous Hemothorax in a Patient with von Recklinghausen’s Disease: A Case Report and Review of the Literature

Fatal Tension Hemothorax Combined With Exanguination: A Rare Complication of Neurofibromatosis

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