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1型神经纤维瘤病中的先天性桡骨假关节:一个不容错过的病症!

Congenital pseudoarthrosis of the radius in Neurofibromatosis Type 1: An entity not to be missed!

作者信息

Narayana Kurup Jayakrishnan K, Shah Hitesh H

机构信息

Department of Orthopedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India.

出版信息

J Orthop. 2020 Sep 23;22:427-430. doi: 10.1016/j.jor.2020.09.013. eCollection 2020 Nov-Dec.

Abstract

Congenital pseudoarthrosis of the radius is exceedingly rare. We report an isolated pseudoarthrosis of the radius in a five-year-old girl child with Neurofibromatosis type-I (NF-I). She underwent excision of the pseudoarthrosis tissue, ulna osteotomy, iliac crest cortico-cancellous bone grafting, and K wire fixation of both bones. A sound union of the radius and the ulna was achieved after 4 months. At two-year follow-up, mild shortening of the forearm persisted with maintenance of sound union of both bones. Accurate diagnosis of the condition is central in avoiding complications and the need for complex surgical procedures.

摘要

先天性桡骨假关节极为罕见。我们报告一例患有Ⅰ型神经纤维瘤病(NF-Ⅰ)的5岁女童孤立性桡骨假关节。她接受了假关节组织切除、尺骨截骨、髂嵴皮质松质骨移植以及两根骨头的克氏针固定。4个月后桡骨和尺骨实现了牢固愈合。在两年的随访中,前臂仍有轻度缩短,但两根骨头保持牢固愈合。准确诊断该疾病对于避免并发症以及避免复杂手术操作至关重要。

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Descriptive analysis of tibial pseudarthrosis in patients with neurofibromatosis 1.1型神经纤维瘤病患者胫骨假关节的描述性分析
Am J Med Genet. 1999 Jun 11;84(5):413-9. doi: 10.1002/(sici)1096-8628(19990611)84:5<413::aid-ajmg5>3.0.co;2-1.
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Congenital pseudarthrosis of the ulna.先天性尺骨假关节
J Hand Surg Br. 1994 Apr;19(2):238-43. doi: 10.1016/0266-7681(94)90176-7.

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