SPRENGEL’S DEFORMITY-final

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SPRENGEL’S DEFORMITY :

SPRENGEL’S DEFORMITY Presented by DR. MD. NAZRUL ISLAM( D.Ortho -student)

SPRENGEL’S DEFORMITY:

SPRENGEL’S DEFORMITY The scapulae normally complete their descent from the neck by the third month of fetal life. In this condition, scapula fails to descend down from it’s initial high position(cervical spine) Here the scapula lies too high & is abnormally small.

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It is associated with other congenital anomalies like cervical spine(e.g. Klippel-feil syndrome),high thoracic kyphosis or scoliosis.

ETIOLOGY:

ETIOLOGY This may be due to failure of scapular descent from the cervical spine.The high scapula may be attached to the spine by a tough fibrous band or a cartilaginous bar(the omovertebral bar).

PATHOLOGY:

PATHOLOGY The pathological changes are seen in the bones and muscles. The scapula may be normal ,broad or high and there could be other features like hemivertebra , wedging of vertebra etc. Among the muscles trapezius may be absent.

CLINICAL FEATURE::

CLINICAL FEATURE: Deformity is the only symptom & it may be noticed at birth. The shoulder on the affected side is elevated, the scapula looks & feels abnormally high, smaller than usual & somewhat prominent. Occasionally both scapulae are affected.

Cont……:

Cont…… 4 . The neck appears shorter than usual & there may be kyphosis or scoliosis of the thoracic spine.

Cont…:

Cont… 5. Shoulder movements are painless but abduction & elevation may be limited by fixation of the scapula.

INVESTIGATIONS::

INVESTIGATIONS: Plain Xray of scapula (P/A view) : will show- the elevated scapula & any associated vertebral anomalies. Sometimes there is also a bony bridge between the scapula & the cervical spine(the omo -vertebral bar).

TREATMENT::

TREATMENT: No treatment is required in case of mild deformity. In case of severe deformity surgery is done under 6 years of age.This consists of release of muscle from the scapula,excising the supraspinous portion of the scapula & the omo -vertebral bar, pulling the scapula down, then reattaching the muscles to hold it firmly in it’s new position. In older children this carries a risk of brachial nerve compression or traction between the clavicle & first rib.

SOME PHOTOS OF SPRENGEL’S DEFORMITY-:

SOME PHOTOS OF SPRENGEL’S DEFORMITY- Figure-1

Figure-2:

Figure-2

Figure-3:

Figure-3

THANKS TO ALL :

THANKS TO ALL

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