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SPRENGEL'S SHOULDER

Questions

What is Sprengel's Shoulder?

Sprengel's shoulder is also called a high scapula. Sprengel deformity is the most common congenital abnormality of the shoulder girdle characterized by an abnormally raised scapula on one side or both sides. The affected shoulder blade is abnormally connected to the spine, which causes restriction of the shoulder movement. Scoliosis with convexity on the involved side may also be observed. The movement of the scapula is marked by limitation due to fibrous bands or a bony bar, as the muscles of the scapula are poorly developed.

Signs and Symptoms of Sprengel's shoulder.

Depending on the severity of the condition and the additional skeletal or muscular abnormalities present Sprengel's deformity can change.

 

·         Asymmetry in the shoulder alignment.

·         An elevated shoulder blade may cause a lump in the base of the neck.

·         Underdeveloped muscles in the surrounding area.

·         Restricted ROM (range of motion)of shoulder and arm on the affected side.

·         Limitation of shoulder abduction and elevation.

·         Limited or restricted movement of the cervical spine.

·         Neck deformities like mild tilting (torticollis) to severe spine deformity.

Causes for Sprengel's shoulder.

Sprengel's deformity is a developmental condition, caused due to:

 

·         Disorder during early fetal development.

·         Genetic defect.

·     Sprengel deformity may be associated with, Klippel-Feil syndrome, clavicular abnormalities, rib abnormalities, limb length discrepancy, scoliosis, spina bifida, hemivertebrae, underdevelopment (hypoplasia) of neck or shoulder muscles.

 

Pathology

Sprengel's deformity often affects surrounding structures, which require a normal scapula for development. The elevated scapula is the most common sign, but the weakness of the surrounding musculature is also seen. An arrest in the development of bone, cartilage, and muscles. The trapezius, rhomboids, levator scapulae, pectoralis major, latissimus dorsi, and sternocleidomastoid may be absent or poorly developed. Weakness of serratus anterior muscle may lead to winging of the scapula.

 

Cavendish Classification of Sprengel's Deformity

 

 

Grade

 

 

Description

 

 

Very mild

 

Shoulders are in level. The deformity cannot be seen when the patient is dressed.

 

 

Mild

 

Shoulders are almost in level. The deformity can be seen as a lump when the patient is dressed.

 

Moderate

 

The shoulder is elevated by 2–5 cm. The deformity is easily visible.

 

 

Severe

 

The shoulder is much elevated with the superior angle of the scapula lies near the occiput,  with or without neck webbing.

 

Diagnosis of Sprengel's shoulder.

X-ray:

The x-ray shows bony and cartilage deformity or abnormality.

 

Computed tomography (CT):

Computed tomography (CT) scans are used to identify associated abnormalities like scoliosis, cervical and scapular abnormalities.

 

Magnetic resonance imaging (MRI):

Magnetic resonance imaging (MRI) helps to identify any bony, cartilaginous, or muscular weakness or defects.

 

Conservative treatment for Sprengel's shoulder:

In non-surgically cases encourage the child to participate in sports such as swimming to maintain ROM. Moderate to severe cases may require surgical management.

 

Surgery:

There are several surgical procedures used to treat Sprengel's deformity. Woodward techniques and Green are the most commonly used procedures. These procedures involve removal of the protruding portion of the scapula and omovertebral bone as well as translation of the scapula inferiorly to a more caudad position. To prevent brachial plexus injury osteotomy of the clavicle can also be done.

Physiotherapy Treatment Used after Surgery.

After the surgery, the patient's shoulder immobilized to help the healing process and prevent possible discomfort. Physiotherapy aims at improving deformity, to facilitate effortless motion of the shoulder.

 

Transcutaneous electrical nerve stimulation (TENS):

Transcutaneous electrical nerve stimulation provides pain relief, which may be caused after performing stretching exercises.

 

Ultrasound:

Ultrasound is very effective in breaking adhesions.

 

Thermotherapy:

Thermotherapy is used for relaxation of the muscles before performing the exercise program.

 

Mobilization:

Physiotherapy, consisting of passive and active ROM exercises, beginning after 6 weeks. Gradual relaxed passive mobilization of the shoulder and scapula are done to improve the range of motion and flexibility of the shoulder. Early mobilization of the scapula and shoulder including movements of abduction and elevation.

 

Strengthening exercises

Strengthening exercises of all the groups of muscles strengthen the weakened muscles.. Strengthening of the shoulder girdle muscles by isometric and isotonic exercises like shoulder shrugs, shoulder rotation, push up, arm support, forearm support, bridging, etc.

 

Stretching exercise for Upper trapezius muscle:

The child sits in a chair. Place her right hand below her buttocks. The therapist holds the child's scapula by grasping the acromion and lateral border of the scapula. The child then performs neck flexion and neck rotation towards the left side. Then the child places her left hand below her buttocks. The therapist holds the child's scapula by grasping the acromion and lateral border of the scapula. The child then performs neck flexion and neck rotation towards the right side. The child maintains the neck in these positions for 10 seconds during stretching.

 

 

Stretching exercises for Levator scapulae:

The child lies in a prone position, with her head rotated to the opposite right side. The child then holds her head and pulls it in flexion with her left hand and abducted her right shoulder as much as possible, without elevating the scapula.  The therapist restricts the scapular elevation by grasping the scapula.

 

Cross-body stretching:

The child is in a supine position. The therapist grasps the child's acromion and lateral border of the scapula. The child then flexes her right shoulder with the elbow to 90°. The child uses her left hand to hold her right elbow and then pulls her elbow from the right side to the left side, as far as possible.

 

Protraction exercise scapula:

The child lies in a supine position. When her right shoulder in 90° flexion and her elbow fully extended. The child then extends her right elbow with maximal force in the forward direction. The therapist grasps the child's scapula by holding the acromion to prevent the scapular elevation and trunk rotation.

 

Posterior tilt exercise for scapula:

The child is lying in a prone position, with the shoulder abducted to 130– 145°, humerus overhead, and forearm in a neutral position. The child then places the left hand under her forehead and lies slightly on the forehead with the dorsum of her hand. While she lifts her right arm, with her elbow extended, the therapist restricts scapular elevation by grasping the superior angle of the scapula.

Family Education

The parents are advised to supervise all the exercises to be performed by the child. While performing the exercises at home, the parents should look for the proper movement of the scapula and prevent the elevation of the scapula. If the superior border of the scapula moves upwards, then the exercise should be stopped and the child should be asked to perform the exercise again.