What causes Sprengel deformity?
Sprengel deformity typically occurs sporadically for no apparent reason but autosomal dominant inheritance has been reported. It is caused by an interruption of normal development and movement of the scapula during early fetal growth (probably between the 9th and 12th weeks of gestation).
What is Omovertebral bone?
[2] The omovertebral bone is an anomalous bone connecting the elevated scapula to the cervical spine, being called omovertebral bar should it be fused between them. It contributes to the immobility of the scapula and the functional impairment, and its resection should be performed if surgical treatment is pursued [1].
How common is Sprengel deformity?
Sprengel’s deformity is a relatively rare condition consisting of an elevated, dysplastic scapula that causes cosmetic deformity and restriction of shoulder range of motion. It is caused by failure of scapular descent during the 9th-12th weeks of gestation.
Who is Sprengel?
General Discussion. Sprengel deformity is a rare congenital disorder in which the shoulder blade (scapula) is too high on one side of the body. The affected abnormal shoulder blade is also abnormally connected to the spine, often restricting movement of the shoulder.
Is Sprengel’s deformity a disability?
Sprengel’s deformity of the shoulder is a dysplasia of the pectoral girdle, resulting in cosmetic and functional disability. The deformity is associated with other congenital anomalies, which often dictate the management and outcome of treatment.
What causes elevated scapula?
Scapular winging is almost always caused by damage to one of three nerves that control muscles in your arms, back, and neck: the long thoracic nerve, which controls the serratus anterior muscle. the dorsal scapular nerve, which controls the rhomboid muscles. the spinal accessory nerve, which controls the trapezius …
What is true winging?
True winging of the scapula is caused by serratus anterior muscle palsy. Any other cause of winging deformity such as tumor, glenohumeral instability, fracture, paralysis of the trapezius muscle, or others is considered pseudo-winging of the scapula [3].
Is Klippel Feil rare?
Klippel-Feil syndrome is estimated to occur in 1 out of every 40,000 births. It was first identified in 1912 by Frenchmen Maurice Klippel and André Feil. Klippel-Feil syndrome forms in utero when genes GDF6 or GDF3 mutate. The condition is present at birth, but it may go undetected for years if the symptoms are slight.
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.
What is Sprengel deformity of the scapula?
Sprengel deformity is usually noticed at birth and has both cosmetic and functional implications. The elevated scapula is visually noticeable and there is an associated restriction in the motion of the scapula and glenohumeral joint.
What is the clinical presentation of Sprengel deformity?
Clinical presentation. Sprengel deformity is usually noticed at birth and has both cosmetic and functional implications. The elevated scapula is visually noticeable and there is an associated restriction in the motion of the scapula and glenohumeral joint.
Is Sprengel’s deformity an omovertebral bone?
This connection is known as an omovertebral bone. There is a high correlation between Sprengel’s deformity and Klippel–Feil syndrome. Sprengel’s deformity is inherited in an autosomal dominant manner.