Sprengel's deformity

A developmental abnormality in which the scapula doesn’t descend to its proper position or a high lying shoulder blade is called sprengel's deformity. In the intrauterine life it is lying in the cervical region then it descends. The associated muscles may be weak (trapezius, rhomboid muscles etc).

Sprengel's deformity

Presentation:

·         Shoulder asymmetry

·         Restricted shoulder abduction

·         Scapula is adducted and 2 – 10 cm elevated.

·         Inferior pole is deviated medially, the glenoid faces inferiorly, and the length of vertebral border is decreased.

Associated problems:

·         Poland syndrome

·         Klippel-Feil syndrome (short, webbed neck; decreased range of motion (ROM) in the cervical spine)

·         Scoliosis

Cavendish classification:

Based on the severity of the condition, a Sprengel deformity can be classified as follows (Cavendish grades).

Grade 1:

The deformity is very mild with clothes it cannot be seen. The shoulders are almost at level.

Grade 2:

This is mild deformity but the superomedial portion of the high scapula is visible as a lump.

Grade 3:

It is a moderate deformity. The affected shoulder is 2 – 5 cm higher than the opposite shoulder. It is visible.

Grade 4:

It is severe deformity. The scapula is very high with neck webbing and brevicollis.

Diagnosis:

Imaging Chest X – ray:

To confirm the diagnosis and to look for associated anomalies. 

 CT – Scan (thorax):

Omovertebral body can be seen, which is a bony bar that attaches the scapula to spine (cervical).

Management:

·         Physiotherapy to gain mobility

·         Definitive treatment is surgery

·         Repositioning of the scapula is done (modified green scapuloplasty and wood ward procedure)