Özdemir Meltem, Kavak Rasime Pelin, Kaplanoğlu Hatice
University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Department of Radiology, Ankara, Turkey.
BJR Case Rep. 2020 Feb 12;6(1):20190073. doi: 10.1259/bjrcr.20190073. eCollection 2020 Mar.
Ulnar hemimelia, also referred to as post-axial longitudinal deficiency of the upper limb, is a very rare skeletal anomaly characterized by the partial or complete absence of the ulna. The majority of the reported cases are sporadic and more common in males. The disorder is mostly unilateral, right-sided and incomplete. A slight shortening of the forearm, radial bowing and ulnar-sided hand drift are the anomalies which often accompany ulnar hemimelia. Ulnar hemimelia may also be seen in association with complex wrist and hand anomalies. The absence of post-axial metacarpal and digital bones are frequent findings in patients with this rare disorder. Cases with additional digital abnormalities such as post-axial syndactyly and camptodactyly are also present in the literature. However, a case of ulnar hemimelia in association with mesoaxial synostotic syndactyly has never been reported to date.
尺骨半侧发育不全，也被称为上肢轴后纵向发育缺陷，是一种非常罕见的骨骼异常，其特征是尺骨部分或完全缺失。大多数报道的病例为散发性，且在男性中更为常见。这种疾病大多为单侧、右侧且不完全性。前臂轻度缩短、桡骨弓形弯曲和尺侧手部偏移是常伴随尺骨半侧发育不全的异常情况。尺骨半侧发育不全也可能与复杂的腕部和手部异常同时出现。轴后掌骨和指骨缺失是这种罕见疾病患者的常见表现。文献中也有伴有额外手指异常（如轴后并指和屈曲指）的病例。然而，迄今为止，尚未有尺骨半侧发育不全合并中轴骨性并指的病例报道。
