24 hours emergency & ambulance Service: +7028859555

Introduction

Sprengel’s shoulder is a condition in which the bone of the shouder blade (scapula) is at an upper level than normal. Due to this, the child has asymmetric shoulders with the affected shoulder being at a higher level than the normal side.

Normally the scapula bone lies at the level of third to seventh thoracic vertebrae. However, in Sprengel’s shoulder, during embrologic development, the scapula fails to descend to it’s normal level and remains at a higher level. Hence, this condition is also called “Congenital elevation of scapula”.

Pathology:

Higher scapula:

As mentioned, the affected scapula is at a higher level than the normal side.

Mis-shapen scapula:

The scapula is hypo-plastic (smaller) and of different shape as compared to the opposite scapula.

Omo-vertebral bar:

The omo-vertebral bar is an abnormal bridge of bone between the superior edge of scapula and spine. This abnormal bridge of bone causes restriction of shoulder overhead abduction.

Associated conditions:

Spine:

A child with Sprengel’s shoulder may have abnormal fusion of vertebrae of cervical spine. This is called Klippel Feil syndrome and results in restriction of neck movements. Also failure of formation and segmentation of vertebrae may result in spinal deformities (scoliosis and kyphosis). Also, there may be associated anomalies of the spinal cord and MRI spine may be needed for diagnosis of these abnormalities.

Heart/ kidneys:

A child with Sprengel’s shoulder may also have associated abnormalities of the heart and kidneys. 2D Echo and Ultrasonography of the abdomen are needed for diagnosis of these abnormalities.

Problems:

Cosmetic deformity:

Shoulders at uneven levels results in cosmetically displeasing deformity. The severity of this deformity may vary.

Restriction of shoulder abduction:

In addition to cosmetic deformity, there may be restriction of shoulder overhead abduction which results in functional limitations.

Treatment:

Treatment of Sprengel’s shoulder depends on severity of deformity. In children with mild deformity in whom the limitation of shoulder abduction is not too severe, no treatment may be offered.

However, children in whom deformity is cosmetically displeasing and restriction of shoulder abduction is functionally disabling, surgery should be offered. Surgery in cases of Sprengel’s shoulder should be performed after the age of three years.

Surgery

Modified Woodward’s surgery:

In this surgery, muscles connecting the scapula and spine are disinserted from their attachment to the spine, the scapula is pulled downwards, and the muscles are re-sutured to the spine at a more normal lower level. Excision of abnormal omo-vertebral bar between the scapula and spine is an important part of the operation which helps to improve overhead abduction movements of the shoulder. Additionally, clavicle bone may be morselized in older children so that there is no pressure on the nerves of the brachial plexus when the scapula is pulled downwards.

Green’s surgery:

In this surgery, the muscles connecting the scapula and spine are disinserted from their attachments to the scapula (unlike the spine in modified Woodward operation). Other steps of Green’s surgery are the same as in modified Woodward’s surgery.

Outcomes:

Surgery for Sprengel’s shoulder usually has good outcomes with improvement of cosmetic deformity as well as range of shoulder overhead abduction.

Article written by public information by Dr Sandeep Vaidya, Chief, Division of Children’s Orthopaedics. For more information, email drsandeepvaidya@pinnacleorthocentre.org. To schedule an appointment , log in to www.pinnacleorthocentre.com, or, call 7028859555.