Shprengel's disease

Description:

Shprengel's disease or high standing of a shovel - an inborn disease.

Symptoms of the Disease of Shprengel:

It is often combined with Klippel-Feyl's syndrome, scoliosis, cervical edges, a sailing neck. Rather often Shprengel's disease is combined with other malformations of internals: heart diseases, intestines, urinary system. High standing of a shovel meets both one - and bilateral anomaly of a musculoskeletal system. In the course of pre-natal formation of a backbone and an upper shoulder girdle the shovel falls from area of cervical department of a backbone by thorax level. The shovel delay at higher level at patients is caused by existence of an abnormal omovertebralny stone or dense connective tissue (fibrous) tyazh between the upper edge of a shovel and backbone. Therefore distinguish bone and fibrous forms of a disease of Shprengel. In that and other case at this disease the shovel is reduced in sizes, deformed, its parts are unevenly developed, the muscles providing motive function of an upper extremity are shortened, fibro changed. The shovel fixed to a thorax is located under the wrong corner. All described elements of anomalies limit function of an upper shoulder girdle. And, along with cosmetic defects, is indications to operational treatment.

Shprengel etiologies:

Cause of illness, as well as other inborn and dysplastic diseases, action harmful teratogenic ekzo-or endogenous factors the moment of a zaklaka of a backbone and an upper shoulder girdle is.

Treatment of the Disease of Shprengel:

For effective bringing down of a shovel in normal situation it is necessary to mobilize a shovel by excision of an abnormal omovertebralny stone or a fibrous tyazh, podnadkostnichgy cutting off of infrascapular muscles and without violence to move it to normal situation. The essential moment is removal of a so-called supraspinal part of a shovel which has a hook appearance. At bringing down of a shovel this hook can cause a prelum of nervous roots of a brachial plexus and their paralysis. The reduced shovel for a bottom corner on a long thick ligature is fixed to 8-9 edge in the correct situation. On 2-3 week the upper extremity is fixed a soft bandage of Dezo. In 3 weeks begin rehabilitation actions for improvement of function of an upper extremity by carrying out physiotherapy exercises and massage.

At a bilateral disease of Shprengel operation on bringing down on other party is carried out in 1-1,5 months. At a combination with Klippel-Feyl's syndromes operation of bringing down of a shovel can be combined with  the operation "tservikalization" of verkhnegrudny department of a backbone.

At a part of patients at a combination of a disease Shprengel and Klippel-Feyl the sailing neck in the form of a skin fold from sides from a neck which is shown or amplifies after bringing down of a shovel and "tservikalization" of chest department of a backbone can be observed. The final stage of elimination of all these defects is operation on separation of a skin fold.