Kinoshita Hayato, Miyakoshi Naohisa, Kobayashi Takashi, Abe Toshiki, Kikuchi Kazuma, Shimada Yoichi
Akita Kosei Medical Center, 1-1-1 Nishibukuro Iijima, Akita, 011-0948, Japan.
Department of Orthopedic Surgery, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita, 010-8543, Japan.
BMC Surg. 2019 Apr 25;19(1):44. doi: 10.1186/s12893-019-0502-z.
Neurofibromatosis type 1 is an autosomal dominant genetic disease with characteristic café-au-lait spots, neurofibroma, and dystrophic changes in the bones. However, complications involving atlanto-axial dislocation are rare.
We report a case of neurofibromatosis with atlanto-axial dislocation. The chief complaints were numbness of the upper limb and gait disturbance. We performed short fusion using the Brooks method. However, recurrence of the dislocation was found after 5 months recovery, and the patient underwent posterior fusion from the occipital bone to C4. Thereafter, she had a good postoperative course.
Neurofibromatosis patients often exhibit a low bone mineral density because of dystrophic changes, and are prone to fragile bones. In the present case, the use of long fusion at the first surgery may have helped to form a strong fusion of fragile bone.
1型神经纤维瘤病是一种常染色体显性遗传病，具有特征性的咖啡牛奶斑、神经纤维瘤和骨骼的营养不良性改变。然而，涉及寰枢椎脱位的并发症很少见。
我们报告一例患有寰枢椎脱位的神经纤维瘤病病例。主要症状为上肢麻木和步态障碍。我们采用布鲁克斯方法进行了短节段融合。然而，在恢复5个月后发现脱位复发，患者接受了从枕骨到C4的后路融合术。此后，她术后恢复良好。
神经纤维瘤病患者常因营养不良性改变而骨密度较低，且易发生骨质脆弱。在本病例中，首次手术时采用长节段融合可能有助于脆弱骨骼形成牢固融合。
