Nozawa Akifumi, Ozeki Michio, Hori Tomohiro, Kato Hiroki, Ohe Naoyuki, Fukao Toshiyuki
Department of Pediatrics, Gifu University Graduate School of Medicine, Japan.
Department of Radiology, Gifu University Graduate School of Medicine, Japan.
Intern Med. 2019 Jul 1;58(13):1929-1933. doi: 10.2169/internalmedicine.2118-18. Epub 2019 Feb 25.
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.
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。