Sprengel's disease is an inherited bone disease. A child with Sprengel's disease is born with the shoulder blade too high, and often with other birth defects. Then, one method of treatment is surgery. What are the causes and symptoms of Sprengel's disease? What is the operation?

Sprengel's disease(Latinscapula alta congenita ), or Sprengel deformity, iscongenital high position of the shoulder blade , which is one of the two bones of the shoulder belt that is connected to the humerus and collarbone. It is the most common congenital defect of the shoulder girdle. Depending on the position of the scapula crest, the disease is mild, moderate or severe.

Sprengel's disease usually affects only one shoulder blade and is more common in girls than in boys.

Sprengel disease - causes

The causes of the disease are unknown. However, due to the fact that deformities occur only in the area of ​​the scapula, it is assumed that in the early stages of fetal development, some harmful factor only affects this area.

Sprengel disease - symptoms

The shoulder blade is higher than it should be. Besides, it is smaller and distorted compared to the correct one. The shoulder blade may be so small and set so high that it can be felt around the back of the head. In addition, the upper part of the shoulder blade bends forward and the lower part protrudes from the chest.

The mobility of the shoulder joint is also limited, which is caused by the incorrect position and limited mobility of the scapula in relation to the chest. Also, the head tends to be tilted towards the distortion.

In 1/3 of patients, the upper angle of the scapula is connected to the arches of the cervical vertebrae (the so-calledos omovertebrale ).

In addition, you can observe scoliosis, hypoplasia (hypoplasia) of the ribs in the area of ​​the scapula or clefts of the vertebral arches.

Sometimes the disease can include Klipp-Feil syndrome, a congenital short neck (caused by multiple changes in the shape and number of cervical vertebrae), as well as other congenital syndromes such as Greig's syndrome, Poland's syndrome, Goldenhara and the VATER association.

Sprengel disease - diagnosis

The diagnosis is made on the basis of the above-mentioned symptoms and x-rays of the cervical and thoracic spine, aalso of the chest and shoulder joints.

Sprengel disease - treatment

Mild deformities do not require the intervention of a surgeon, as long as the range of motion in the shoulder joint is maintained.

In other cases, an operation is performed which consists in releasing the scapula from abnormal connections with the cervical spine ( os omovertebrale)and releasing and fixing the scapula in a lower position. During the operation, the most symmetrical positioning of the shoulder blades is aimed at, but in practice their uniform setting is not possible, because it is associated with the risk of neurological complications (the lower the shoulder blade moves, the greater the risk of brachial plexus paralysis). Therefore, after the treatment, the shoulder blade will remain slightly shorter than the he althy one. The operation is performed using the Green (scapular release) or Woodward (paraspinal release) method.

Correction of a too high shoulder blade is performed in children from 3 to 5 years of age. Later, the deformation may be more difficult to correct. In addition, the risk of damage to the brachial plexus increases (compression of the plexus between the collarbone and the upper ribs).

After the operation, it is necessary to exercise to increase the range of motion in the shoulder joint and strengthen the muscles of the shoulder girdle.

Bibliography:

Compendium of orthopedics , edited by Kusz D., Wydawnictwo Lekarskie PZWL, WarsawWiktor Dega orthopedics and rehabilitation , edited by Marciniak W., Szulc A., PZWL Medical Publishing, Warsaw

Category:

Spine, bones, joints