Abstract
Background
Osteochondromas, also known as exostoses, are the most common benign tumors of bone and can be classified into isolated and multiple osteochondromas. A great majority of osteochondromas is asymptomatic, painless, slow-growing mass, and incidentally found. However, osteochondromas occurring in adolescence or in adult patients can grow in size and become symptomatic as a result of mechanical irritation of the surrounding soft tissues or peripheral nerves, spinal cord compression, or vascular injury.
Case presentation
We present a case of a 13-year-old girl with spontaneous hemothorax, the cause of which was identified by limited thoracotomy with the aid of video-assisted thoracic surgery to be bleeding from a diaphragmatic laceration incurred by a costal exostosis on the left sixth rib. Preoperative chest computed tomography (CT) depicted a bony projection arising from the rib and bloody effusion in the intrathoracic cavity, but was unable to discern the bleeding cause from the lung or the diaphragm. This case will highlight our awareness that costal exostosis possibly results in bloody pleural effusion. Meanwhile, English literatures about solitary costal exostosis associated with hemothorax were searched in PubMed and nineteen case reports were obtained. Combined our present case with available literature, a comprehensive understanding of this rare disease entity will further be strengthened.
Conclusions
Injury to the diaphragm is the primary cause of hemothorax caused by costal osteochondroma, including the present case. Thoracic CT scan can help establish a diagnosis of preoperative diagnosis of costal osteochondroma. Surgical intervention should be considered for those patients with symptomatic osteochondroma of the rib. Combined with our case and literature, prophylactic surgical removal of intrathoracic exostosis should be advocated even in asymptomatic patients with the presentation of an inward bony spiculation.
Similar content being viewed by others
Explore related subjects
Find the latest articles, discoveries, and news in related topics.Background
Osteochondromas represent the most common bone tumor accounting for 20–50% of all benign osseous tumors [1]. They develop during bone maturation at the metaphysis in the period from early childhood through the late teens, which can be sessile or pedunculated. Osteochondromas may be solitary or multiple. A percentage of 85% of osteochondromas present as solitary lesions, while 15% occur in the context of hereditary multiple exostoses or familial osteochondromatosis, a genetic disorder that is inherited in an autosomal dominant manner [2]. Osteochondromas tend to occur at the metaphyseal region of the long bones of the extremities, but have also been reported to exist in other areas such as the scapula, pelvis, clavicle, rib and vertebra. The key radiological features of osteochondroma are cortical and marrow continuity between the lesion and the parent bone, and a cartilage cap [3]. When these tumors are found within the thoracic skeleton, clinicians face unusual diagnostic and therapeutic dilemmas. Radiologists must be aware of the potential life-threatening secondary complications from rib osteochondromas and the role of CT scan for full diagnostic work-up. Nowadays, the mainstay of conservative treatment is observed with plain radiographs initially and subsequently by clinical examination. Indicators for surgical therapy include pain, complications, cosmetic reasons, increased risk of malignant transformation, and uncertain diagnosis. Here, a costal osteochondroma manifesting as spontaneous hemothorax is reported and treated successfully via limited thoracotomy with the aid of VAT. Meanwhile, similar English literature about hemothorax caused by solitary rib osteochondromas is also reviewed and incorporated into our case so as to better highlight this disease entity.
Case presentation
On August 12, 2021, a 13-year-old girl presented to our hospital with persistent left shoulder pain for more than 2 months, which was accompanied by left chest pain for 2 days. She denied the history of any familial diseases, recent chest trauma or having anticoagulant drugs. Physical examination revealed reduced respiratory movement and decreased breathing sounds in the left lung field. Percussion sounds of the left chest were solid. Besides, blood hemoglobin levels on admission was 108 g/L(reference range, 110–150 g/L) and clotting parameters were also normal. Chest CT demonstrated a left pleural effusion associated with passive atelectasis of the lung and a 2 cm long spear-shaped osseous proliferation originating from the visceral side of the left sixth rib (Fig. 1). The osseous proliferation showed medullary continuity with the parent rib-bone which prompted the probable diagnosis of osteochondroma. For further diagnosis and treatment of the pleural effusions, the patient underwent ultrasound-guided thoracentesis, and 350 ml of dark blood fluid was aspirated in the intrathoracic cavity, which was indicative of hemothorax. Subsequently, a contrast-enhanced multi-slice computed tomography (MSCT) scan of the thorax ruled out potential pulmonary vascular malformation and neoplasms. The osseous projection abutted the left diaphragm downward (Fig. 2), but there was no visible extravasation of intravenous contrast in the left intrathoracic cavity.
Surprisingly, the patient's hemoglobin level dropped to 88 g/L at the interval of 1 day, a clue of mild anemia from the probability of chronic active bleeding. After homotypic packed red blood cells of 2U were given, the patient was hemodynamically stable. Based on the shape and growth pattern of the bony protrusion on CT and relevant clinical data by now, the hemothorax was probably associated with this costal lesion. Therefore, surgical intervention was necessary to clarify the cause of hemothorax and certain diagnosis of costal lesion.
On August 25, 2021, exploratory VAT revealed that a bony protrusion spanned from the medial aspect of the left sixth rib toward the chest cavity, in close contact with the diaphragm. The diaphragm had been lacerated by the bony protrusion (Fig. 3) and was locally covered with residual clotted blood and fibrin layers, suggesting the aetiology of bloody pleural effusion. There was no obvious injury to the visceral pleura, but heavy adhesion of pleural space was noticed. Then the bony protrusion was excised along with partial anterior sixth rib through limited thoracotomy with the aid of VAT. After the closure of wounds, a chest tube was inserted and left until the postoperative 7th day. The gross specimen consisted of rib part and an attached bony protrusion (Fig. 3). Pathology confirms the osseous proliferation consistent with an osteochondromatous proliferation with no signs of dysplasia or concern of malignancy, namely osteochondroma. The patient was discharged home on postoperative 8th day in good clinical condition. At a 6-month follow-up, there was no evidence of any residual or recurrent exostosis.
Discussion
Osteochondromas, also known as exostoses, are the most common benign tumors of bone and can be classified into isolated and multiple osteochondroma, and they can be observed in 1–2% of the population [4]. The multiple form is an autosomal dominant syndrome referred to as hereditary multiple exostosis or familial osteochondromatosis, accounting for 55% of rib osteochondromas [5]. Osteochondromas are lesions on the surface of the bone composed of both cortical and medullary bone with hyaline cartilage caps, which tend to grow away from the joint through tensile forces of tendons and ligaments. The presence of cortical and medullary continuity of the tumor with the underlying bone is a pathognomonic feature that establishes the diagnosis.
Osteochondromas tend to occur at the metaphyseal region of the long bones of the extremities, but have also been reported to exist in other areas such as the scapula, pelvis, clavicle, rib and vertebra. Among primary chest wall tumors primarily occurring in the rib, fibrous dysplasia is the most common cause of a benign rib growth, followed by osteochondromas [6]. Osteochondromas of the ribs generally originate from the costochondral junction but may also occur at the costovertebral junction. Despite our patient coming in with an osteochondroma slightly more lateral to the costochondral junction, it was histopathologically confirmed to be consistent with a benign osteochondromatous proliferation. A great majority of osteochondromas is asymptomatic, painless, slow-growing mass, and incidentally found. Those symptomatic osteochondromas usually present in younger patients, 75–80% are discovered before the 20th year of age. However, osteochondromas occurring in adolescence or in adult patients can grow in size and become symptomatic as a result of mechanical irritation of the surrounding soft tissues or peripheral nerves, spinal cord compression, or vascular injury. In the case of a patient with a symptomatic osteochondroma of the rib, symptoms may range from hemothorax or pneumothorax to cardiac symptoms, diaphragmatic rupture and spinal nerve injuries due to extrinsic compression, and a palpable lump or malignant transformation. The diagnosis of the patient is osteochondroma located on the rib and manifests as spontaneous hemothorax owing to the diaphragmatic injury. In addition, our patient reported her repeated left shoulder pain lasting for more than 2 months, which was probably a clue of left diaphragm friction by the exostosis. The mechanisms underlying diaphragmatic injury due to exostosis include direct force by a sharp bony spur or repetitive erosion by particularly pointed bony extrusions during respiratory movements. Injury to the diaphragm, pleura, heart, and lung have all been reported rarely [7,8,9,10,11,12,13,14,15,16,17,18,19,20,21], and they could cause a life-threatening condition if untreated timely. Most previous reports have also not addressed any significant traumatic event or impact prior to the occurrence of symptoms [7,8,9,10, 13,14,15,16,17,18, 21,22,23], including the case of our patient. Literatures concerning solitary rib osteochondroma leading to hemothorax are listed in the Table 1, which demonstrates different organs injury including the diaphragm, lung, pleura and pericardium in turn. Occasionally it is unable to judge the exact cause of hemothorax, but a favorable outcome after the resection of solitary rib exostosis.
Osteochondromas are most often diagnosed depending on radiographic evidence. Radiographs are often diagnostic, and however, cross-sectional imaging may be indicated to assess for complications, assess the cartilage cap or in some challenging cases establish the presence of medullary continuity. CT and magnetic resonance imaging (MRI) may be of some use in defining the extent of tumor spread locally. Compared with plain X-ray film, cross-sectional imaging offers a more accurate tool in the diagnosis of this condition. Recognition of the radiologic spectrum of osteochondroma and its variants usually allows prospective diagnosis and differentiation of the numerous potential complications, thus helping guide therapy and improving patient management [26]. As described in literature review and our patient, currently CT investigation has become the main method. Its reconstructed image and 3D volume rendering clearly described the morphology, extent and growth pattern of solitary osteochondroma.
Surgical removal of osteochondromas is not usually indicated, especially in childhood. However, surgical resection is indicated for osteochondromas developing in adolescence after puberty or in adult patients with pain, increased size, mechanical complications and malignant transformation. Surgical management of thoracic osteochondroma, with excision for painful, symptomatic, malignant lesions or lesions adjudged to be at risk of intrathoracic complications, yields good outcomes in terms of symptom control, establishing histologic diagnosis, and prevention of thoracic complications [27]. Cases of rib exostosis were surgically approached using different techniques. Minimally invasive thoracoscopic techniques are the preferred method for surgical management of patients with symptomatic costal osteochondromas, but mini-incision thoracotomy is needed depending on factors including dimension of the rib lesion and localization involving the rib [9, 10, 13, 14, 16, 18, 19, 21]. In our case, costal osteochondroma was resected safely via limited thoracotomy with the aid of VAT. VAT can help visualize the internal thoracic structures at locations that are hard to reach and inaccessible through other imaging modalities. Various studies have shown that the surgical resection of osteochondroma is necessary to avoid further complications of hemothorax, pneumothorax or intercostal neuralgia. However, osteochondroma of the ribs can even be conserved if not associated with complications and patient does not need to undergo unnecessary surgery [28].
Conclusions
Osteochondromas are a very common entity, but rarely occur at the ribs. Osteochondroma of the rib may cause non-specific, occasionally serious complications for mechanical frictions or mass effect. Thoracic CT scan can help establish a diagnosis. Surgical intervention should be considered for those patients with symptomatic osteochondroma of the rib. Combined with our case and literature, prophylactic surgical removal of intrathoracic exostosis should be advocated even in asymptomatic patients with the presentation of an inward bony spiculation.
Availability of data and materials
We declared that materials described in the manuscript, including all relevant raw data, will be freely available to any scientist wishing to use them for non-commercial purposes, without breaching participant confidentiality.
Abbreviations
- CT:
-
Computed tomography
- VAT:
-
Video-assisted thoracic surgery
- MSCT:
-
Multi-slice computed tomography
- MRI:
-
Magnetic resonance imaging
- M:
-
Male
- F:
-
Female
- CXR:
-
Chest X-ray
- US:
-
Ultrasound
References
Tepelenis K, Papathanakos G, Kitsouli A, et al. Osteochondromas: an updated review of epidemiology, pathogenesis, clinical presentation, radiological features and treatment options. In Vivo. 2021;35(2):681–91.
Bovée JV. Multiple osteochondromas. Orphanet J Rare Dis. 2008;3:3.
Alyas F, James SL, Davies AM, Saifuddin A. The role of MR imaging in the diagnostic characterisation of appendicular bone tumours and tumour-like conditions. Eur Radiol. 2007;17(10):2675–86.
Vasseur MA, Fabre O. Vascular complications of osteochondromas. J Vasc Surg. 2000;31(3):532–8.
Glass RB, Norton KI, Mitre SA, Kang E. ribs: P A spectrum of abnormalities. Radiographics. 2002;22:87–104.
Nam SJ, Kim S, Lim BJ, Yoon C-S, Kim TH, Suh J-S, Yoon C-S, Suh J-S, et al. Imaging of primary chest wall tumors with radiologic-pathologic correlation. Radiographics. 2011;31:749–70.
Propper RA, Young LW, Wood BP. Hemothorax as a complication of costal cartilaginous exostoses. Pediatr Radiol. 1980;9(3):135–7.
Reynolds JR, Morgan E. Haemothorax caused by a solitary costal exostosis. Thorax. 1990;45(1):68–9.
Tomares SM, Jabra AA, Conrad CK, Beauchamp N, Phoon CK, Carroll JL. Hemothorax in a child as a result of costal exostosis. Pediatrics. 1994;93(3):523–5.
Simansky DA, Paley M, Werczberger A, Bar Ziv Y, Yellin A. Exostosis of a rib causing laceration of the diaphragm: diagnosis and management. Ann Thorac Surg. 1997;63:856–7.
Buchan KG, Zamvar V, Mandana KM, et al. Juxtacardiac costal osteochondroma presenting as recurrent haemothorax. Eur J Cardiothorac Surg. 2001;20(1):208–10.
Waseem M, Hajjar F, Yasser M, et al. Unusual presentation of rib exostosis. Ann Thorac Surg. 2003;75(2):575–7.
Bini A, Grazia M, Stella F, et al. Acute massive haemopneumothorax due to solitary costal exostosis. Interact Cardiovasc Thorac Surg. 2003;2:614–5.
Pham-Duc ML, Reix P, Mure PY, et al. Hemothorax: an unusual complication of costal exostosis. J Pediatr Surg. 2005;40:e55-57.
Jin W, Hyun SY, Ryoo E, et al. Costal osteochondroma presenting as haemothorax and diaphragmatic laceration. Pediatr Radiol. 2005;35:706–9.
Matsushima K, Matsuura K, Kayo M, Gushimiyagi M. Periosteal chondroma of the rib possibly associated with hemothorax: a case report. J Pediatr Surg. 2006;41(10):E31–3.
Martino A, Fabrizzi G, Costarelli L, et al. Haemothorax caused by isolated costal exostosis. Eur J Pediatr Surg. 2007;17:129–31.
Matsuno Y, Mori Y, Umeda Y, Imaizumi M, Takiya H. Thoracoscopic resection for costal exostosis presenting with hemothorax in a child. Eur J Pediatr Surg. 2009;19(4):253–4.
Nakano T, Endo S, Nokubi M, et al. Hemothorax caused by a solitary costal exostosis. Ann Thorac Surg. 2009;88(1):306.
Patel M, Bauer TW, Santoscoy T, et al. Osteochondroma of the fifth rib resulting in recurrent hemothorax. Skeletal Radiol. 2015;44:1853–6.
Arunachalam P, Sam C, Lakshmi K, et al. A rare case of pediatric osteochondroma presenting as hemothorax. J Indian Assoc Pediatr Surg. 2020;25:316–8.
Harrison NK, Wilkinson J, O’Donohue J, et al. Osteochondroma of the rib: an unusual cause of hemothorax. Thorax. 1994;49:618–9.
Marlowe GS, Arensman R, Price MR. Spontaneous hemothorax associated with costal exostoses. Am Surg. 2011;77(9):E190–2.
Huang HR, Lin TY, Wong KS. Costal exostosis presenting with hemothorax: report of one case. Eur J Pediatr. 2006;165(5):342–3.
Graham J, Winterson R, Grovell J, Boon RL. An unusual cause of a pleural effusion. Emerg Med J. 2008;25(11):749.
Murphey MD, Choi JJ, Kransdorf MJ, et al. Imaging of osteochondroma: variants and complications with radiologic-pathologic correlation. Radiographics. 2000;20:1407–34.
Bakhshi H, Kushare I, Murphy MO, et al. Chest wall osteochondroma in children: a case series of surgical management. J Pediatr Orthop. 2014;34(7):733–7.
Nakano T, Endo S, Tsubochi H, et al. Thoracoscopic findings of an asymptomatic solitary costal exostosis: is surgical intervention required? Interact Cardiovasc Thorac Surg. 2012;15(5):933–4.
Acknowledgements
None.
Funding
Predicting pathologic response for those resectable stage III non-small cell lung cancer patients with neoadjuvant therapy through artificial intelligence method (JLSWSRCZX2021-054). Predicting pathologic response and prognosis for those resectable stage III non-small cell lung cancer patients with neoadjuvant therapy through computed tomography deep learning and radiomic features (JDYY11202110).
Author information
Authors and Affiliations
Contributions
DBC was the radiologist who diagnosed the disease and helped perform the analysis with constructive discussions. RNP and XQL wrote the initial draft and reviewed the literature, they contributed equally to the work. ZJW was the surgeon who performed the procedure and confirmed the validity of our paper. LJD completed the collection of relevant literature. All authors have read and approved the final manuscript.
Corresponding author
Ethics declarations
Ethics approval and consent to participate
The study was approved by the Medical Ethics Committee of the First Hospital of Jilin University.
Consent for publication
Informed written consent was obtained from the patient for publication of this case, including all individual details and accompanying images. Written consent form is available for review upon request.
Competing interests
The authors declare that they have no competing interests.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
About this article
Cite this article
Pan, R., Lu, X., Wang, Z. et al. Hemothorax caused by costal exostosis injuring diaphragm: a case report and literature review. J Cardiothorac Surg 17, 230 (2022). https://doi.org/10.1186/s13019-022-01984-7
Received:
Accepted:
Published:
DOI: https://doi.org/10.1186/s13019-022-01984-7