CAJ | 학술논문

目的：提高对Gorham-Stout综合征的认识。方法报告1例下颌骨、肋骨、双手（足）多骨及皮肤受侵的Gorham-Stout综合征患者，结合文献对其临床特征进行探讨。结果 DR片示右侧下颌支、多根肋骨、双手（足）多发中远节指（趾）骨质吸收溶解，残端呈“削笔尖”状，溶骨区无骨膜反应；CT 结合二维及三维重建直观地显示了骨质破坏的范围、周围软组织萎缩；M RI见残留的右侧下颌小头周围少许长T1、T2异常信号渗出影环绕。肋骨及胸膜穿刺活检病理见成骨细胞明显减少，而血管和纤维组织增生。结论 Gorham-Stout综合征是一种以大块骨质溶解为特征的罕见病，影像学及病理结合临床有助于发现该病。
목적：제고대Gorham-Stout종합정적인식。방법보고1례하합골、륵골、쌍수（족）다골급피부수침적Gorham-Stout종합정환자，결합문헌대기림상특정진행탐토。결과 DR편시우측하합지、다근륵골、쌍수（족）다발중원절지（지）골질흡수용해，잔단정“삭필첨”상，용골구무골막반응；CT 결합이유급삼유중건직관지현시료골질파배적범위、주위연조직위축；M RI견잔류적우측하합소두주위소허장T1、T2이상신호삼출영배요。륵골급흉막천자활검병리견성골세포명현감소，이혈관화섬유조직증생。결론 Gorham-Stout종합정시일충이대괴골질용해위특정적한견병，영상학급병리결합림상유조우발현해병。
Objective To improve the knowledge of Gorham-Stout syndrome .Methods This paper reported one case of mandibular ,ribs ,hand (foot) in patients with multiple bone and skin involvement of Gorham-Stout syndrome ,and the lit-erature was reviewed .Results X-ray showed that right mandibular ramus ,ribs ,hand (foot) multiple finger (toe) bone were absorption and dissolved ,stump was "sharpening the pencil"shape ,osteolytic area had periosteal reaction .CT with 2D and 3D reconstruction displayed the range around the bone destruction ,soft tissue atrophy .MRI showed residual right mandibular head around a little long T1 and T2 signal abnormalities effusion shadows surround .The ribs and pleural biopsy in bone cells decreased ,while the blood vessels and fibrous tissue presented with hyperplasia .Conclusion Gorham-Stout syndrome is a very rare disease characterized by massive osteolysis .Imaging and appearance of pathology may help to final diagnosis .