H-Index
14
Scimago Lab
powered by Scopus
eISSN: 1941-5923
call: +1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Logo


Get your full text copy in PDF

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

Floriane D.A. Degbelo, Giovanni Cito, Boumédiène Guendil, Michel Christodoulou, Ziad Abbassi

(Department of Surgery, Sion Hospital, Sion, Switzerland)

Am J Case Rep 2019; 20:674-678

DOI: 10.12659/AJCR.915810


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.

This paper has been published under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.
I agree