窒息性胸廓发育不良的影像表现(附4例报告)

The imaging features of asphyxiating thoracic dysplasia(report of four cases)

目的:加深对窒息性胸廓发育不良(ATD)影像表现的认识。方法:通过4例ATDX线和CT影像分析,结合临床资料及有关文献加以讨论。结果:①ATD是一种少见的常染色体隐性遗传性骨软骨发育不良疾病。②X线主要表现:胸廓狭长,肋骨短,肋骨头膨大;髂翼呈方形,股骨头骨骺提前骨化;长骨短,干骺端增宽;掌指骨短粗,锥形骨骺及赘生指。结论:对反复肺部感染的患儿,应进行影像学检查,早日明确诊断,为临床治疗提供依据。

Objective： To promote the understanding of the image features of asphyxiating thoracic dysplasia（ATD）. Methods： X-ray films and CT image features of four ATD were studied combining with clinical and relative literatures. Result： ATD is an uncommon autosomal recessive hereditary disorder of osteochondrodysplasia. The main findings of ATD are as follows： a narrow thorax, short ribs, the anterior ends of the ribs are widened and cupped; the pelvis also shows flaring ala of the ilium and early ossification of the centers for the capital femoral epiphyses; extremity deformities include shortening of the long tubular bone, metaphyseal irregularities and windening, the shortening of tubular bones of the hands with cone-shaped epiphyses and polydactylism. Conclusion： The children who suffer from pneumonia repeatedly should perform X-ray examination to help make correct diagnosis on time.