Kim Junyoung, Park Young Chang, Moon Hyun-Soo, Do Woo Sung, Yang Kyu Hyun
Department of Orthopedic Surgery, Gangnam Severance Hospital, Yonsei University College of Medicine, Gangnam-gu, Seoul.
Department of Orthopedic Surgery, International St. Mary's Hospital, Catholic Kwandong University College of Medicine, Seo-gu, Incheon, Republic of Korea.
Medicine (Baltimore). 2020 Aug 7;99(32):e21648. doi: 10.1097/MD.0000000000021648.
Autosomal dominant type II (AD II) osteopetrosis is a rare inheritable metabolic bone disease characterized by hard but brittle bone and a narrow medullary canal. Intramedullary nailing (IMN) is a difficult but attractive option for the treatment of subtrochanteric fractures in patients with AD II osteopetrosis.
Two patients with AD II osteopetrosis sustained subtrochanteric fractures after a fall.
IMN was performed through the sequential use of instruments such as a 4.9-mm drill bit, small reamer, and larger reamer for over-reaming.
In the first case, IMN left some gap at the fracture site. Dynamization was performed to treat the delayed union at 6 months postoperatively. The fracture healed at 10 months after the dynamization. In the second case, IMN was successful without a gap, and the fracture healed at 8 months.
Although IMN is difficult to perform owing to partial obliteration of the medullary canal in AD II osteopetrosis, it can be performed with sequential widening of the medullary canal using various instruments. In addition, the fracture gap should not be left uncorrected during IMN to attain fracture union.
常染色体显性II型(AD II)骨硬化症是一种罕见的遗传性代谢性骨病,其特征是骨质坚硬但易碎,髓腔狭窄。髓内钉固定术(IMN)是治疗AD II骨硬化症患者转子下骨折的一种困难但有吸引力的选择。
两名AD II骨硬化症患者在跌倒后发生转子下骨折。
通过依次使用4.9毫米钻头、小型扩孔钻和大型扩孔钻进行过度扩孔等器械进行髓内钉固定术。
在第一例中,髓内钉固定术后骨折部位留有一些间隙。术后6个月进行动力化治疗延迟愈合。动力化治疗后10个月骨折愈合。在第二例中,髓内钉固定术成功且无间隙,骨折在8个月时愈合。
尽管由于AD II骨硬化症患者髓腔部分闭塞,髓内钉固定术操作困难,但可以使用各种器械依次扩大髓腔来进行。此外,在髓内钉固定术中不应让骨折间隙未得到纠正,以实现骨折愈合。