PMC4220418_01
Case 1. 6-year-old boy who presented with a short neck and bilateral lumps on his neck was diagnosed with bilateral Sprengel deformity. a) An AP X-ray of the thorax shows asymmetrical position of the scapulae and high position of scapular bases (arrows); compare the level of the glenoid cavity (asterisk) and infraglenoid tubercle (arrowhead) on each side. b) An AP X-ray of the cervical-upper thoracic spine demonstrates thoracic spine scoliosis (arrows) and fused ribs (asterisks). c) Lateral X-ray of the cervical-upper thoracic spine shows disturbance of the normal thoracic spine kyphosis (arrows).
JCIS-4-55-g002.jpg
PMC4220418_01_JCIS-4-55-g002.jpg
F1
The little boy was first examined with an anteroposterior X-ray of the thorax and anteroposterior and lateral X-rays of the cervical spine [Figure 1], which demonstrated asymmetrically high-positioned scapulae, in conjunction with cervical vertebrae synostosis and thoracic cage deformities.
Case 1. 6-year-old boy who presented with a short neck and bilateral lumps on his neck was diagnosed with bilateral Sprengel deformity. Axial CT images a) At the C7 level shows where the right scapula appears (double arrow), the upper third of the left scapula is already visible (arrow). b) At the T1 level where the right acromion lies (closed arrowhead), the scan reveals the left scapular body just inferior to the scapular spine (open arrowhead). c) At the T2, the scan shows the level of the upper part of the body of the right scapula (asterisk), only the lower third of the left scapular body (double asterisk), as is expected since the right scapula is at a lower level compared with the left one.
JCIS-4-55-g003.jpg
PMC4220418_01_JCIS-4-55-g003.jpg
F2
Surgery was decided and a CT scan was performed to delineate the anatomy in detail, assess the degree of asymmetry, and provide the details for morphometric analysis and surgery planning [Figure 2].
PMC4220418_02
Case 2. 7-year-old girl with Klippel-Feil syndrome and left Sprengel deformity referred for imaging studies before operative intervention. a) A synthesis AP X-ray of the right and left shoulder shows the undescended and medially rotated left scapula (arrows). There is also a right cervical rib (asterisk). b) Axial CT images at the level of lower cervical vertebrae show the left scapula (white arrowheads) and the left glenohumeral joint (double asterisk) lying at a higher level compared to the contralateral side. There are also cervical unfused vertebral arches (empty asterisks).
JCIS-4-55-g004.jpg
PMC4220418_02_JCIS-4-55-g004.jpg
F3
Anteroposterior X-ray scans of the shoulders were performed, followed by a CT and an MRI scan, in order to evaluate the omovertebral association and demonstrate the spectrum of abnormalities that was required for thorough planning of surgery [Figures 3 and 4].
Case 2. 7-year-old girl with Klippel-Feil syndrome and left Sprengel deformity. 3D-CT reconstruction images show the difference between the position of the scalpulae: (a and b) giving the superior displacement of the affected scapula relative to the normal one. The A/B ratio measures the rotational deformity of the affected scapula. Lines A and B are drawn between the inferior scapular angle (yellow arrowhead) and a line perpendicular to the axis along the spinous processes (asterisks). Lines A and B connect the center of the glenoid cavity (green dots) to the medial border of the scapular spine (blue dots).
JCIS-4-55-g005.jpg
PMC4220418_02_JCIS-4-55-g005.jpg
F4
Anteroposterior X-ray scans of the shoulders were performed, followed by a CT and an MRI scan, in order to evaluate the omovertebral association and demonstrate the spectrum of abnormalities that was required for thorough planning of surgery [Figures 3 and 4].
PMC4220418_03
Case 3. 14-year-old boy with a right-sided nuchal deformity, shown with CT and MRI to be a Sprengel scapula. (a and b) Axial CT-scan images demonstrate the elevated position of the right scapula (white arrows). The shoulder joints (asterisks) are asymmetrical. (c) Coronal MRI reveals mildly hypoplastic musculature of the right shoulder (double arrow). (d) 3D-CT reconstruction images show that the right scapula (black arrowhead) is higher than the left (yellow arrowhead). Note the associated deformity of the rib cage on the right side (yellow arrow) and the asymmetry of the shoulder joints (double asterisks).
JCIS-4-55-g006.jpg
PMC4220418_03_JCIS-4-55-g006.jpg
F5
These revealed a mild Sprengel deformity and provided the details of the bony associations and anatomy of extra-osseous structures as needed for preparation of the intervention [Figure 5].