Department of Neurosurgery, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki, 852-8501, Japan.
Childs Nerv Syst. 2022 Sep;38(9):1817-1820. doi: 10.1007/s00381-022-05472-0. Epub 2022 Jul 5.
McCune-Albright syndrome (MAS) and fibrous dysplasia (FD) have been reported to cause Chiari type I malformation (CM1) and skull base invagination (BI).
A 6-year-old girl was diagnosed with MAS and FD. She was diagnosed with CM1 at age 8 years, and the syringomyelia had gradually increased by age 20 years. We performed foramen magnum decompression and C1 laminectomy, and the syringomyelia stopped spreading after surgery.
This patient underwent long-term radiological observation and morphological evaluations, which revealed that the skull thickening was progressing, while the posterior cranial fossa volume (PCFV) remained unchanged for 14 years. Therefore, although PCFV did not decrease, it was considered to be relatively inadequate due to the increase in brain volume with growth, resulting in posterior fossa overcrowding, causing CM1.
In patients with FD/MAS, long-term evaluation of bone thickening, odontoid position, and PCFV is necessary.
McCune-Albright 综合征（MAS）和纤维结构不良（FD）可导致 Chiari Ⅰ型畸形（CM1）和颅底凹陷（BI）。
一名 6 岁女孩被诊断为 MAS 和 FD。她在 8 岁时被诊断为 CM1，20 岁时脊髓空洞症逐渐加重。我们进行了枕骨大孔减压和 C1 椎板切除术，术后脊髓空洞停止扩张。
该患者进行了长期的影像学观察和形态学评估，结果显示颅骨增厚在进展，而后颅窝容积（PCFV）在 14 年内保持不变。因此，尽管 PCFV 没有下降，但由于脑体积随生长而增加，导致后颅窝拥挤，考虑到相对不足，也会导致 CM1。
在 FD/MAS 患者中，需要对骨增厚、齿状突位置和 PCFV 进行长期评估。
