PinnacleOrthoCentre Introduction
Sprengel shoulder is a condition in which the bone of the shoulder 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 its 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 shapes as compared to the opposite scapula.
Omo-vertebral bar:
The omo-vertebral bar is an abnormal bridge of bone between the superior edge of the 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 an abnormal fusion of vertebrae of the cervical spine. This is called Klippel Feil syndrome and results in restriction of neck movements. Also, failure of the formation and segmentation of vertebrae may result in spinal deformities (scoliosis and kyphosis). Also, there may be associated anomalies of the spinal cord and an 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 the diagnosis of these abnormalities.
Problems:
Cosmetic deformity:
Shoulders at uneven levels result 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 shoulder depends on the severity of the deformity. In children with mild deformity in whom the limitation of shoulder abduction is not too severe, no treatment may be offered.
However, for children in whom deformity is cosmetically displeasing and restriction of shoulder abduction is functionally disabling, surgery should be offered. Surgery in cases of Sprengel 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 an 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 shoulder usually has good outcomes with the improvement of cosmetic deformity as well as a range of shoulder overhead abduction.
–This article is contributed by Dr Sandeep Vaidya, Paediatric Orthopaedic Surgeon, Pinnacle Orthocentre Hospital, Thane. Dr Vaidya is also available for consultations at BJ Wadia Children’s Hospital, Mumbai; Ajit Scan Centre, Kalyan; and Ace Children’s Hospital, Dombivli. For more information, call 7028859555/ 8879970811/ (022)25419000/ 25429000 OR email drsvvaidya@gmail.com.