Sprengel deformity is congenital elevation of the scapula due to failure of normal scapular descent during embryological development. The affected scapula remains high in the neck, is often hypoplastic and malrotated, and may be connected to the cervical spine by an omovertebral bone, cartilage or fibrous band.

It is rare, but it is the most common congenital anomaly of the shoulder girdle. The child usually presents because one shoulder looks high, the neck appears webbed or asymmetric, or shoulder abduction is restricted. Pain is not usually the main complaint in childhood; appearance and motion are the key issues.

The condition matters because it is often not isolated. It may coexist with Klippel-Feil syndrome, scoliosis, rib anomalies, congenital renal anomalies, cardiac anomalies and other syndromic findings. The assessment therefore starts at the scapula but must not stop there.

The scapula forms in the cervical region and normally descends caudally during early development. Sprengel deformity reflects incomplete descent. The scapula is not simply sitting high; it is often structurally abnormal.

Key anatomical features:

• High scapula: the superior angle sits abnormally high in the neck.
• Hypoplastic scapula: the affected scapula is often smaller than the opposite side.
• Malrotation: the glenoid and body are rotated, impairing scapulothoracic movement.
• Omovertebral connection: a bone, cartilage or fibrous band may connect the superomedial scapula to cervical vertebrae.
• Muscle abnormalities: trapezius, rhomboids, levator scapulae and periscapular muscles may be hypoplastic, contracted or abnormally inserted.
• Rib cage limitation: rib and thoracic deformity may restrict how far the scapula can be lowered safely.

The limitation of abduction is mechanical. The glenohumeral joint may be structurally normal, but the scapula cannot rotate upward normally on the thorax. This is why shoulder abduction improves only if the scapula is mobilised and positioned more favourably.

View larger

Classification is used to describe severity and guide discussion. It does not replace the clinical decision: treatment still depends on function, appearance, age, associated anomalies and family priorities.

View larger

Associated anomalies decide how safe treatment is. A high scapula should trigger a structured screen rather than a narrow shoulder assessment.

The usual presentation is a child with a visibly high shoulder or asymmetric shoulder contour. The deformity may be noticed at birth, during early childhood, or when the child begins activities requiring overhead motion.

History should define:

• age at recognition and whether the deformity is progressive in appearance;
• family concern: cosmesis, shoulder function, sport, clothing, teasing or pain;
• shoulder function: overhead reach, dressing, hair care, sport and fatigue;
• neck symptoms: stiffness, pain, limited rotation or neurological symptoms;
• birth history and other congenital anomalies;
• previous physiotherapy, bracing or investigations;
• family goals and tolerance for scar, recurrence risk and prolonged follow-up.

Examination should be deliberate:

1. Inspect from behind with the trunk exposed. Compare shoulder height, scapular height, neck webbing, trapezial contour and scoliosis.
2. Palpate the superior angle and medial border of the scapula. Look for a prominent superomedial corner.
3. Measure active and passive shoulder forward elevation and abduction on both sides.
4. Observe scapulothoracic rhythm. The scapula may fail to upwardly rotate as the arm elevates.
5. Assess glenohumeral motion separately from scapulothoracic motion.
6. Examine cervical range of motion and signs of Klippel-Feil syndrome.
7. Screen neurological status in the upper limb, especially if there are cervical abnormalities.
8. Examine the spine and chest wall for scoliosis and rib anomalies.
9. Look for renal, cardiac or syndromic clues from the history and general examination.

Management is based on severity, function, age and safety. The aim is not to make every scapula look normal; the aim is to improve meaningful cosmesis and shoulder motion without creating neurological injury.

View larger

The operative plan must be explicit before the incision: expected descent, omovertebral excision, soft-tissue release, need for clavicle osteotomy, nerve monitoring plan, and postoperative immobilisation.

Woodward ProcedureModified Green ProcedureSafety Details

Purpose: lower the scapula by releasing trapezius and rhomboid origins from the spinous processes, excising tethers, translating the scapula inferiorly and reattaching the muscle origins lower.

Stepwise technique:

1. Position: prone or lateral/prone variant with the entire shoulder girdle, neck and thoracic spine accessible.
2. Preparation: mark spinous processes, scapular borders, scapular spine, superior angle and planned midline/parascapular exposure.
3. Exposure: posterior incision along the upper thoracic/cervical midline or planned approach; raise skin flaps carefully.
4. Identify muscle origins: trapezius and rhomboids are elevated from the spinous processes as a sleeve.
5. Release tether: identify and excise omovertebral bone or fibrous band if present.
6. Scapular mobilisation: release tight levator scapulae, periscapular tethering and superomedial restriction as needed.
7. Inferior translation: lower the scapula gradually. Avoid aggressive forced descent.
8. Nerve protection: monitor brachial plexus clinically or with neuromonitoring; consider clavicle osteotomy if tension is concerning.
9. Fixation and reattachment: reattach the muscle sleeve at a lower level to maintain correction.
10. Closure: meticulous haemostasis, drain as needed, layered closure and immobilisation.

View larger

Purpose: mobilise the scapula by releasing periscapular muscles from the scapula, excising tethers and reattaching the scapula/muscles in a lower position.

Practical points:

• expose the superomedial scapula and periscapular muscles;
• release trapezius, levator scapulae and rhomboid attachments as needed;
• excise the omovertebral bone and prominent superomedial scapular region if it blocks descent;
• translate the scapula inferiorly without excessive traction;
• secure the corrected position and repair muscle attachments;
• consider clavicle osteotomy if descent places the plexus under tension.

View larger

Safety decisions:

• Neuromonitoring: useful in severe cases or when major descent is planned; signal change should stop further descent.
• Clavicle osteotomy: consider when the scapula will be lowered substantially or plexus tension is observed.
• Omovertebral bone: define it before surgery and excise it under direct vision.
• Scapular resection: avoid excessive resection that weakens muscle attachments or creates contour problems.
• Correction limit: a safe partial correction is better than a cosmetically ambitious neurological injury.

Postoperative care protects the correction and then restores motion.

Typical care includes:

• observation of upper-limb neurological status after surgery;
• wound and drain monitoring;
• shoulder immobilisation according to surgeon preference and fixation stability;
• analgesia and scar care;
• early elbow, wrist and hand motion;
• gradual shoulder range after soft tissues have healed;
• physiotherapy for scapular control, posture and overhead function;
• serial review for correction loss, recurrence, winging and shoulder motion.

The family should know that improvement is expected, but the shoulder may not become perfectly level or perfectly mobile. Persistent asymmetry, scar and some abduction limitation may remain.

A high or asymmetric shoulder is not always Sprengel deformity. The distinction matters because management differs and some mimics carry their own systemic risk.

Much of the Sprengel evidence base is observational, so several practical questions remain genuinely unsettled. Examiners reward a candidate who can name the uncertainty rather than overstate the data.

• Optimal age to operate. Most series favour correction in younger children (often under 8 years) for better descent and remodelling, but the evidence is observational and confounded by deformity severity. There is no randomised threshold age.
• Woodward versus modified Green. Both relocate the scapula and report broadly similar improvements; comparative data are weak, and procedure choice largely reflects surgeon training rather than proven superiority.
• Routine clavicle osteotomy. Some surgeons add it selectively only when descent threatens the plexus, while others use it routinely with neuromonitoring to allow larger, safer correction. The trade-off of added clavicle morbidity against safer descent is not resolved.
• Role and necessity of neuromonitoring. Reported intraoperative alerts are uncommon, so whether neuromonitoring should be universal or reserved for major planned descent remains debated.
• Vertical scapular osteotomy and bony resections. These address rotational and superomedial prominence but add muscle and contour morbidity; their place outside specialised practice is uncertain.
• How far to correct. Aggressive cosmetic descent risks brachial plexus injury and contour problems. The consensus principle is that a safe partial correction is preferable to a neurologically unsafe complete one.

There is no high-level society guideline or implant registry specific to Sprengel deformity; it is a rare, non-arthroplasty congenital condition, so practice is guided by paediatric orthopaedic principles, narrative reviews and surgical series rather than registry data. The points below frame a globally consistent approach.

Global epidemiology

• Sprengel deformity is rare but is the most common congenital anomaly of the shoulder girdle; it is more often unilateral and is reported slightly more frequently in girls in several series.
• Klippel-Feil syndrome, congenital scoliosis and rib anomalies are the most consistently reported associations across populations; congenital renal and cardiac anomalies are described and justify screening when syndromic features are present.

Side-by-side principles across practice settings

Registry note

• Arthroplasty registries (NJR, AJRR, AOANJRR, SHAR, NZJR) do not capture Sprengel deformity because no implant is used. Outcome evidence comes from single-centre and multicentre surgical cohorts, which is why reporting is heterogeneous and major-complication rates, although low, are imprecise.

High- versus limited-resource practice variation

• In well-resourced settings, 3D CT for omovertebral and scapular morphology and intraoperative neuromonitoring or clavicle osteotomy are increasingly used to allow safer, larger correction.
• In limited-resource settings, plain radiographs and clinical grading drive decisions, neuromonitoring may be unavailable, and surgeons may favour conservative correction targets and selective clavicle osteotomy to protect the plexus without advanced monitoring. The core safety principle — screen widely and do not over-correct — is universal.

Complications can be early, structural or expectation-related.

When shown a child with a high scapula, structure the answer in this order:

1. Diagnosis: congenital elevated scapula consistent with Sprengel deformity.
2. Severity: describe clinical appearance, Cavendish grade, shoulder abduction and functional limitation.
3. Associated anomalies: specifically mention cervical spine, Klippel-Feil, scoliosis, ribs, renal and cardiac screening.
4. Imaging: AP shoulder/chest, cervical spine films, spine imaging if needed, CT or 3D CT for omovertebral bone.
5. Treatment choice: mild functional deformity can be observed; severe cosmetic or motion loss may need surgery.
6. Operation: Woodward or modified Green procedure, omovertebral excision, safe inferior translation, possible clavicle osteotomy and nerve monitoring.
7. Counselling: improvement rather than normality, scar, correction loss, nerve injury and persistent limitation.