Surgical treatment of Gorham-Stout disease combined with scoliosis: a case report and literature review

被引:0
作者
Jiao, Yang [1 ]
Sun, Heng [2 ]
Huang, Yizhen [2 ]
Zhao, Junduo [2 ]
Huang, Xu'an [2 ]
Cai, Haoyu [2 ]
Shen, Jianxiong [2 ]
机构
[1] Chinese Acad Med Sci, Peking Union Med Coll Hosp, Peking Union Med Coll, Dept Breast Surg,Dept Surg, Beijing, Peoples R China
[2] Chinese Acad Med Sci, Peking Union Med Coll Hosp, Dept Orthopaed Surg, Peking Union Med Coll, Beijing, Peoples R China
基金
中国国家自然科学基金;
关键词
Gorham-stout disease; Scoliosis; Surgery; Sirolimus; Diagnosis; Case report;
D O I
10.1186/s12891-024-08217-z
中图分类号
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学(修复外科学)];
学科分类号
摘要
BackgroundGorham-Stout disease (GSD) is a rare disease characterized by osteolysis and lymphatic malformations. GSD involving the spine is exceptionally rare and lacks a standard cure. The aim of this article was to report a case of GSD with scoliosis treated via corrective surgery and medication. Clinical features, imaging data, treatment, and published GSD cases are discussed.Case Presentation. We report the case of a 14-year-old male with GSD (confirmed by pathology and genetic analysis of bone tissue due to a previous fracture), which mainly involved the spine and caused progressive scoliosis. Scoliosis was defined as a right upper thoracic curve with a Cobb angle of 61 degrees and a left major thoracic curve with a Cobb angle of 50 degrees. Preoperative magnetic resonance imaging of the spine in the T2-weighted phase showed wedge-shaped changes in T4-9, with substantial high signal and no intradiscal abnormalities. The patient successfully underwent scoliosis correction from T2-L2. After surgery, the patient was treated with sirolimus for osteoporosis, and the effect of the scoliosis correction remained stable after 2 years of follow-up. This case documented the rare phenomenon of spinal lamina chylous leakage caused by GSD. Corrective surgery combined with sirolimus achieved good results in the treatment of scoliosis caused by GSD.ConclusionThis study provides an important reference for the diagnosis and treatment of GSD involved with spine.
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页数:7
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