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一例坎塔普特拉型中肢发育不全——新发现与新诊断方法

A case of mesomelic dysplasia Kantaputra type--new findings and a new diagnostic approach.

作者信息

Siwicka Karolina Anna, Kitoh Hiroshi, Nishiyama Masaki, Ishiguro Naoki

机构信息

Department of Hand Surgery, Medical University of Gdansk, Gdansk, Poland.

出版信息

J Pediatr Orthop B. 2008 Sep;17(5):271-6. doi: 10.1097/BPB.0b013e32830cc3c8.

Abstract

Mesomelic dysplasia is a heterogeneous group of rare bone diseases characterized by disproportionate shortness of middle segments of limbs and short stature. This study reports a male patient with an early diagnosed mesomelic dysplasia Kantaputra type (MIM*156232), characterized by symmetric, bilateral forearm and lower leg shortening with feet malformations and ankle-tarsal synostoses. As a result of several diagnostic biases in defining the mesomelic syndromes, we attempted to categorize symptoms and to clarify hitherto difficult discrimination between mesomeliae. Given that specific ankle joint alignment distortions seem to be pathognomonic for Kantaputra type (especially a fibulo-calcanear and, to a lesser extent, a tibio-talar synostosis), but not detectable with plane radiography, we encouraged use of MRI evaluation as an indispensable modality for proper diagnosis and further preoperative planning.

摘要

中肢发育不全是一组罕见的骨骼疾病,其特征是四肢中段不成比例地短小和身材矮小。本研究报告了一名早期诊断为中肢发育不全坎塔普特拉型(MIM*156232)的男性患者,其特征为双侧前臂和小腿对称性缩短,伴有足部畸形和踝-跗骨融合。由于在定义中肢综合征时存在多种诊断偏差,我们试图对症状进行分类,并澄清迄今中肢发育不全之间难以区分的情况。鉴于特定的踝关节排列扭曲似乎是坎塔普特拉型的特征性表现(尤其是腓骨-跟骨融合,在较小程度上还有胫-距骨融合),但平片无法检测到,我们鼓励使用MRI评估作为正确诊断和进一步术前规划必不可少的手段。

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