Chest
Postgraduate Education CornerPulmonary and Critical Care PearlsSpontaneous Hemothorax and Recurrent Hemoptysis in a 26-Year-Old Man With Skin Lesions
Section snippets
Physical Examination
On physical examination, the patient appeared comfortable and in no acute respiratory distress. Vital signs on presentation were significant for a heart rate of 100, a respiratory rate of 30, and oxygen saturation of 96% on 2-L nasal cannula. His body habitus was remarkable for short stature (5 ft 1 in), and his facial examination was significant for flattened nasal bridge and slightly low-set ears. Chest examination revealed dullness to percussion and decreased air entry over the lower half of
Discussion
In most cases, hemothorax is the result of trauma or a complication of a procedure, such as placement of a central venous catheter or thoracentesis. Hemothorax, as a result of tearing of adhesions between pleural layers or rupture of highly vascularized bullae, can be a complication of spontaneous pneumothorax. Although rare, spontaneous hemothorax does occur (Table 1). After ruling out pneumothorax and necrotizing infections, the differential diagnosis of spontaneous hemothorax in an otherwise
Clinical Pearls
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The presence of spontaneous hemothorax in a young, healthy patient should prompt an investigation for inherited coagulopathies, neoplastic disease, and vascular and connective tissue anomalies.
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The vascular type of EDS is a rare but serious heritable defect in type 3 collagen expression encoded by the COL3A1 gene.
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Despite a penetrance of 100%, the age of detection of type 4 EDS may vary from early childhood into adulthood.
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During the physical examination, the vascular type of EDS does not usually
Acknowledgments
Financial/nonfinancial disclosures: The authors have reported to the CHEST the following conflicts of interest: Dr Siner receives funding from the Yale Clinical and Translational Service Award to investigate the role of growth factors in human sepsis. Drs Selim, Lane, and Rubinowitz have reported no conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.
References (0)
Cited by (12)
Respiratory manifestations of Ehlers-Danlos syndromes
2023, Revue des Maladies RespiratoiresA 19-year-old man with relapsing bilateral pneumothorax, hemoptysis, and intrapulmonary cavitary lesions diagnosed with vascular ehlers-danlos syndrome and a novel missense mutation in COL3A1
2015, ChestCitation Excerpt :The clinical diagnosis of vascular EDS usually relies on two or more of the following criteria: characteristic facial features with a pinched nose, narrow lips and prominent eyes; easy bruising and thin skin with visible veins; or tissue fragility with easy rupture of arteries and internal organs5,7 (Table 1). The rare presentation of vascular EDS with pneumothorax, intrapulmonary hematomas, or hemothorax seems to occur more often in young adults.1,3,8–15 The biochemical abnormality leading to vascular EDS is in type III collagen, which is a key element in the structure of arterial walls, viscous organs, and lung parenchyma.3
Waxing and waning pulmonary nodules and cavities
2022, Zhonghua Jiehe he Huxi Zazhi / Chinese Journal of Tuberculosis and Respiratory DiseasesSpontaneous pneumothorax and hemothorax frequently precede the arterial and intestinal complications of vascular Ehlers–Danlos syndrome
2019, American Journal of Medical Genetics, Part AVascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection
2018, Vascular and Endovascular Surgery
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).
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Drs Selim and Lane contributed equally to this work.