Bilateral Sprengel deformity with Klippel-Feil syndrome

Case contributed by Dr Ammar Haouimi

Presentation

Deformity of both shoulders since birth.

Patient Data

Age: 4 years
Gender: Female

The photograph of this child shows a high position of both scapulae.

Elevated both scapulae with a superomedial angle at T6 level on the right, and at T5 on the left side (grade II according to the radiographic Rigault classification).

An omovertebral bone is seen bilaterally connecting the scapulae to the cervical spine.

The omovertebral bone connects the spinous process of C5 to the superomedial angle of the scapulae.

Fusion of the vertebral bodies of C4, C5, and C6 with reduced anteroposterior diameter and rudimentary intervertebral discs at same levels (findings in keeping with Klippel-Feil syndrome

The atlas shows an anterior arch cleft. 

​No significant hypoplasia or atrophy of the shoulder girdle muscles.

Case Discussion

Features of bilateral Sprengel deformity with Klippel-Feil syndrome

Sprengel deformity, or congenital elevation of the scapula, is the most common congenital deformity of the shoulder. The initial diagnosis can be made on simple radiography, however, CT or MRI is often necessary to evaluate this deformity as well as the associated anomalies.

 

Additional contributor: R. Bouguelaa MD

PlayAdd to Share

Case information

rID: 66395
Published: 19th Feb 2019
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: Aurès Medical Imaging Center - Batna Algeria

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.