Daneshvar Kakhaki Abolghasem, Khodadad Kian, Pejhan Saviz, Karimi Shirin, Arab Mehrdad, Saghebi Reza, Behgam Shadmehr Mohammad, Farzanegan Roya
Tracheal Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, IR Iran.
Chronic Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, IR Iran.
Iran Red Crescent Med J. 2014 Nov 17;16(11):e12180. doi: 10.5812/ircmj.12180. eCollection 2014 Nov.
Gorham's disease is a rare disorder characterized by osteolysis and abnormal vascular growth within bones. Diagnosis of Gorham's disease is often delayed and for accurate and early diagnosis high clinical suspicion is crucial. No specific treatment is available. Management options include surgery, radiation therapy and medical therapy. We aimed to present the first case of Gorham's disease with chest wall involvement in Iran. By review of the literature we discussed important issues of this rare disease including clinical findings, diagnosis and treatment options.
We present a 48-year-old man with a history of dyspnea following a blunt chest trauma who was admitted to our clinic several times due to reaccumulation of pleural fluid and chylothorax. Gorham's disease was finally established according to clinical manifestations and radiological findings including massive osteolysis in his left ribs and also histological examination.
According to review of the literature and considering all treatment modalities the patients was successfully treated with a combination of radiotherapy, pamidronate and thalidomide. We suggest that this disease should be considered among differential diagnoses of patients with chest pain, pleural effusion and/or chylothorax with an unknown reason and more importantly history of chest trauma. In suspected cases, it is essential to examine biopsy specimens of the bone adjacent to the inflammated tissues in order to confirm diagnosis.
戈勒姆病是一种罕见的疾病，其特征为骨溶解和骨内异常血管生长。戈勒姆病的诊断常常延迟，对于准确早期诊断而言，高度的临床怀疑至关重要。目前尚无特效治疗方法。治疗选择包括手术、放射治疗和药物治疗。我们旨在介绍伊朗首例累及胸壁的戈勒姆病病例。通过文献回顾，我们讨论了这种罕见疾病的重要问题，包括临床表现、诊断和治疗选择。
我们报告一名48岁男性，有钝性胸部创伤后呼吸困难病史，因胸腔积液和乳糜胸反复积聚多次入住我院。根据临床表现、包括左肋骨大量骨溶解的影像学表现以及组织学检查，最终确诊为戈勒姆病。
根据文献回顾并考虑所有治疗方式，该患者通过放疗、帕米膦酸二钠和沙利度胺联合治疗获得成功。我们建议，对于原因不明的胸痛、胸腔积液和/或乳糜胸患者，尤其是有胸部创伤史的患者，应将此病纳入鉴别诊断。在疑似病例中，检查炎症组织相邻骨骼的活检标本以确诊至关重要。
