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基纳畸形的临床和影像学表现。9例报告。

Clinical and radiological findings in Kirner's deformity. A report of nine cases.

作者信息

Beluffi G, Fiori P

机构信息

Unità Operativa Semplice di Radiologia Pediatrica, U.O.C. di Radiodiagnostica, IRCCS Policlinico San Matteo, Viale Golgi 19, I-27100, Pavia, Italy.

出版信息

Radiol Med. 2006 Apr;111(3):432-9. doi: 10.1007/s11547-006-0039-y. Epub 2006 Apr 11.

Abstract

PURPOSE

The purpose of this study is to report radiological findings and evaluate the frequency with which Kirner's deformity is found in a large paediatric population.

MATERIALS AND METHODS

We reviewed X-ray films of the hands taken over the past 23.5 years in order to assess not only bone age but all sorts of bone abnormalities (trauma, haematological disorders, bone syndromes and dysplasias, dysmorphic features, rheumatic disorders, etc.) to retrospectively evaluate all those presenting Kirner's deformity.

RESULTS

Among the 16,326 patients who underwent X-ray study of the hands, nine (six males and three females, 0.055% of the total) presented the typical radiological features of Kirner's deformity; no patient had a family history of the disorder except for one, in whom the suspicion of familiarity could not be verified radiologically. The diagnosis of Kirner's deformity was straightforward in most cases. In one case, a girl affected by Turner's syndrome, the abnormality was suspected, and its appearance, development and evolution was followed over 10 years.

CONCLUSIONS

Although rare, as also shown by our study, Kirner's deformity must be recognised and properly diagnosed in order to spare the subject unnecessary surgical procedures.

摘要

目的

本研究旨在报告影像学表现,并评估在大量儿科人群中发现柯纳畸形的频率。

材料与方法

我们回顾了过去23.5年拍摄的手部X线片,以评估骨龄以及各类骨骼异常情况(创伤、血液系统疾病、骨综合征和发育异常、畸形特征、风湿性疾病等),从而对所有出现柯纳畸形的病例进行回顾性评估。

结果

在接受手部X线检查的16326例患者中,9例(6例男性和3例女性,占总数的0.055%)呈现出柯纳畸形的典型影像学特征;除1例患者外,其他患者均无该病家族史,而该例患者的家族性怀疑无法通过影像学得到证实。大多数情况下,柯纳畸形的诊断较为直接。有1例患特纳综合征的女孩,疑似存在异常情况,对其异常表现、发展及演变过程进行了长达10年的跟踪观察。

结论

正如我们的研究所显示,尽管柯纳畸形罕见,但为避免患者接受不必要的外科手术,必须识别并正确诊断该畸形。

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