Department of Neurosurgery, Gulhane School of Medicine, University of Health Sciences, Etlik, 06010, Ankara, Turkey.
Department of Pathology, Gulhane School of Medicine, University of Health Sciences, Ankara, Turkey.
Childs Nerv Syst. 2024 Jul;40(7):2223-2225. doi: 10.1007/s00381-024-06372-1. Epub 2024 Mar 28.
Cystic degeneration of the fibrous dysplasia is a very rare clinical condition and may present with loss of vision when it involved the skull base. A 12-year-old female child presented with an enlargement of the skull. She was diagnosed as large skull base and skull vault tumor. She underwent partial removal of the tumor, and custom-made titanium implant was inserted. The diagnosis was fibrous dysplasia. Two years after the initial diagnosis, she presented with total loss of vision at her right eye. Radiological imaging confirmed the cystic degeneration within the tumor. She re-operated and the cyst fluid was evacuated in association with the removal of cyst wall. The diagnosis was the cystic degeneration of the fibrous dysplasia. Her vision was improved a few days after the surgery. Fibrous dysplasia of the skull base should be closely followed-up in order to prevent severe visual complications.
纤维结构不良的囊性变是一种非常罕见的临床情况，当涉及颅底时可能会导致视力丧失。一位 12 岁的女性患儿出现头颅增大。她被诊断为颅底和颅盖大肿瘤。她接受了肿瘤的部分切除，并植入了定制的钛植入物。诊断为纤维结构不良。初次诊断两年后，她的右眼完全失明。放射影像学证实肿瘤内发生囊性变。她再次手术，切除囊壁并排出囊液。诊断为纤维结构不良的囊性变。手术后几天，她的视力有所改善。为了防止严重的视力并发症，应密切随访颅底的纤维结构不良。
