Gorham-Stout disease presenting as chest wall defect: A case report and review of literature.

IF 0.9 4区 医学 Q4 SURGERY
Athish Kannan Karur, Dinesh Kumar Sathanantham, Jayakumar Thanathu Krishnan Nair, Adinarayanan Kishore Nochur, Samuel Olaniyan
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引用次数: 0

Abstract

Gorham-Stout disease (GSD), also known as vanishing bone syndrome, is an extremely rare, non-malignant condition marked by progressive osteolysis due to abnormal intraosseous lymphatic and vascular proliferation. Extensive chest wall involvement is particularly uncommon and can present without symptoms, complicating diagnosis and management. In this study, a review of similar cases in the literatures is tabulated with comparison of this case to other articles is presented. A 24-year-old male presented with an incidental finding of "hollowness" in his right chest wall following a muscle strain, but remained asymptomatic otherwise. Imaging revealed near-total disappearance of the right 4th to 6th ribs anterolaterally, and partial resorption of the 7th and 8th ribs laterally. MRI confirmed extensive osteolysis with prominent lymphatic and vascular malformations. Histopathologic evaluation showed degenerative bony trabeculae with fibrous tissue and lymphovascular proliferations, confirming GSD. The patient was managed conservatively-vitamin D and calcium supplementation. No progression of disease was detected at subsequent follow-ups. This case underscores the need for high clinical suspicion and comprehensive evaluation in unexplained rib osteolysis. While various medical (bisphosphonates, sirolimus, interferon-α2b) and surgical interventions have been employed, no standard therapy is established. In asymptomatic patients without life-threatening complications, conservative management may be appropriate. The treatment should be personalized based on disease course and complications. The unpredictable prognosis mandates long-term monitoring.

Gorham-Stout病表现为胸壁缺损:1例报告及文献复习。
Gorham-Stout病(GSD),也称为消失骨综合征,是一种极其罕见的非恶性疾病,其特征是骨内淋巴和血管增生异常导致进行性骨溶解。胸壁广泛受累尤其罕见,可无症状表现,使诊断和治疗复杂化。在本研究中,回顾了文献中的类似病例,并将该病例与其他文章进行了比较。一名24岁男性患者在肌肉拉伤后,在右胸壁偶然发现“空洞”,但除此之外仍无症状。影像学显示右侧前外侧第4至第6根肋骨几乎完全消失,外侧第7和第8根肋骨部分吸收。MRI证实广泛的骨溶解伴明显的淋巴和血管畸形。组织病理学检查显示退行性骨小梁伴纤维组织和淋巴血管增生,证实GSD。对患者进行保守治疗——补充维生素D和钙。在随后的随访中未发现疾病进展。本病例强调了对不明原因的肋骨骨溶解需要高度的临床怀疑和综合评估。虽然采用了各种药物(双膦酸盐、西罗莫司、干扰素-α2b)和手术干预措施,但尚未建立标准治疗方法。对于无危及生命并发症的无症状患者,保守治疗可能是合适的。治疗应根据病程和并发症个性化。不可预测的预后要求长期监测。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
1.00
自引率
0.00%
发文量
98
审稿时长
3-8 weeks
期刊介绍: The Turkish Journal of Thoracic and Cardiovascular Surgery is an international open access journal which publishes original articles on topics in generality of Cardiac, Thoracic, Arterial, Venous, Lymphatic Disorders and their managements. These encompass all relevant clinical, surgical and experimental studies, editorials, current and collective reviews, technical know-how papers, case reports, interesting images, How to Do It papers, correspondences, and commentaries.
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