Department of Radiology, National Center for Child Health and Development, Seatagaya-ku, Tokyo, 157-8535, Japan.
Pediatr Radiol. 2011 Aug;41(8):1013-9. doi: 10.1007/s00247-011-2132-1. Epub 2011 May 24.
The "coat-hanger" sign of the ribs with a bell-shaped thorax has been known as a radiological hallmark of the paternal uniparental disomy 14 (upd(14)pat).
To quantitatively determine the differences in thoracic deformity between upd(14)pat and other bone diseases with thoracic hypoplasia and to establish the age-dependent evolution.
The subjects comprised 11 children with upd(14)pat. The angle between the 6th posterior rib and the horizontal axis was measured (coat hanger angle; CHA). The ratio of the mid- to widest thorax diameter (M/W ratio) was calculated for the bell-shaped thorax.
CHA ranged from +28.5 to 45° (mean; 35.1° ± 5.2) in upd(14)pat, and from -19.8 to 21° (-3.3 ± 13°) in bone dysplasias (p < 0.01). The M/W ratio ranged from 58% to 93% (75.4 ± 10) in upd(14)pat, and from 80% to 92% (86.8 ± 3.3) in bone dysplasias (p < 0.05). Serial radiographs revealed that CHA remained constant during early childhood, while the M/W ratio gradually increased with age.
The "coat-hanger" sign of upd(14)pat provides a distinctive radiological gestalt that makes it possible to differentiate the disorder from other skeletal dysplasias. By contrast, the bell-shaped thorax is significant only in the neonatal period.
肋骨的“衣架”征伴钟形胸廓，这是父源单亲二体 14 号染色体（upd(14)pat）的放射学标志。
定量比较 upd(14)pat 与其他伴有胸廓发育不良的骨骼疾病的胸廓畸形差异，并建立年龄相关性演变。
本研究共纳入 11 例 upd(14)pat 患儿。测量第 6 后肋与水平线之间的夹角（衣架角；CHA）。计算钟形胸廓的中胸径与最宽胸径比（M/W 比）。
upd(14)pat 的 CHA 范围为+28.5°至 45°（平均值；35.1°±5.2°），而骨骼发育不良为-19.8°至 21°（-3.3±13°）（p<0.01）。upd(14)pat 的 M/W 比范围为 58%至 93%（75.4±10），骨骼发育不良为 80%至 92%（86.8±3.3）（p<0.05）。连续的 X 线片显示，CHAI 在幼儿期保持不变，而 M/W 比随年龄逐渐增加。
upd(14)pat 的“衣架”征提供了一种独特的影像学整体表现，使其能够与其他骨骼发育不良相区别。相比之下，钟形胸廓仅在新生儿期具有特征性。
