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伴颅底骨髓炎和延髓外侧综合征的戈谢病-斯托特病的致命进展

Fatal Progression of Gorham-Stout Disease with Skull Base Osteomyelitis and Lateral Medullary Syndrome.

作者信息

Nozawa Akifumi, Ozeki Michio, Hori Tomohiro, Kato Hiroki, Ohe Naoyuki, Fukao Toshiyuki

机构信息

Department of Pediatrics, Gifu University Graduate School of Medicine, Japan.

Department of Radiology, Gifu University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2019 Jul 1;58(13):1929-1933. doi: 10.2169/internalmedicine.2118-18. Epub 2019 Feb 25.

DOI:10.2169/internalmedicine.2118-18
PMID:30799352
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6663530/
Abstract

Gorham-Stout disease (GSD) is a rare condition in which spontaneous, progressive resorption of bone occurs. There are no previous reports of patients with fatal progression of GSD with skull base osteomyelitis (SBO) and lateral medullary syndrome (LMS). We present the case of a 27-year-old man diagnosed with GSD with involvement of the maxillofacial bones and skull base. The patient developed SBO; LMS resulted from progressive osteolysis, and the patient died of associated brainstem stroke. Careful follow-up with special emphasis on the early detection of intracranial complications is critical in patients presenting with progressive GSD with involvement of the skull base.

摘要

戈勒姆-斯托特病（GSD）是一种罕见的疾病，其特征为骨骼出现自发性、进行性吸收。此前尚无戈勒姆-斯托特病伴发颅底骨髓炎（SBO）和延髓外侧综合征（LMS）并出现致命进展的患者报告。我们报告一例27岁男性患者，诊断为戈勒姆-斯托特病，累及颌面骨和颅底。该患者发生了颅底骨髓炎；进行性骨质溶解导致了延髓外侧综合征，患者死于相关的脑干卒中。对于出现累及颅底的进行性戈勒姆-斯托特病的患者，进行仔细随访并特别强调早期发现颅内并发症至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/0d95f7d7c756/1349-7235-58-1929-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/dc745b6f4da5/1349-7235-58-1929-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/5939ca7a84f3/1349-7235-58-1929-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/f6f50c14daa2/1349-7235-58-1929-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/0d95f7d7c756/1349-7235-58-1929-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/dc745b6f4da5/1349-7235-58-1929-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/5939ca7a84f3/1349-7235-58-1929-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/f6f50c14daa2/1349-7235-58-1929-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1a84/6663530/0d95f7d7c756/1349-7235-58-1929-g004.jpg

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Efficacy and Safety of Sirolimus in the Treatment of Complicated Vascular Anomalies.西罗莫司治疗复杂血管异常的疗效与安全性。
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Gorham-Stout Disease of the Skull Base With Hearing Loss: Dramatic Recovery and Antiangiogenic Therapy.伴有听力损失的颅底骨肥大性骨关节病：显著恢复与抗血管生成治疗
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A Large Skull Defect Due to Gorham-Stout Disease: Case Report and Literature Review on Pathogenesis, Diagnosis, and Treatment.一例由戈谢病导致的巨大颅骨缺损：病例报告及关于发病机制、诊断和治疗的文献综述
Front Endocrinol (Lausanne). 2020 Feb 5;11:37. doi: 10.3389/fendo.2020.00037. eCollection 2020.
戈谢病的致命进展：一例病例报告及文献综述
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Bone. 2014 Jun;63:47-52. doi: 10.1016/j.bone.2014.02.011. Epub 2014 Feb 26.
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Gorham's disease of the maxilla: a case report.上颌骨戈谢病：一例报告。
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Skull base osteomyelitis leading to lateral medullary syndrome in a child.一名儿童因颅底骨髓炎导致延髓外侧综合征。
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