In Sprengel deformity, the shoulder blade is positioned higher than normal and may be rotated or abnormally shaped. This can cause shoulder asymmetry, scapular prominence and limited shoulder movement.

In mild cases with good shoulder motion, observation may be sufficient. However, this does not mean that children with significant deformity should wait until adulthood. If the deformity is noticed, specialist evaluation should be done as early as possible.

An omovertebral connection is an abnormal bone, cartilage or fibrous connection between the shoulder blade and the spine. It may restrict shoulder blade movement and should be considered during surgical planning.

Sprengel deformity may be associated with neck-spine anomalies, rib anomalies, scoliosis, Klippel-Feil syndrome, cardiovascular or kidney-urinary system problems. Therefore, evaluation should be comprehensive.

Sprengel deformity is a congenital shoulder-girdle deformity in which the shoulder blade, also called the scapula, fails to descend to its normal position and remains higher than usual in the shoulder region. For this reason, it is also known as congenital high scapula.

Normally, the shoulder blade lies on the back of the chest wall and moves in coordination with shoulder motion. In Sprengel deformity, the shoulder blade may be higher, smaller, rotated, abnormally shaped or more prominent than normal. This may cause shoulder height difference, a visible scapular prominence on the back, short-neck appearance and limitation in raising the arm.

Sprengel deformity is usually unilateral. On the affected side, the shoulder may appear higher than the other side. Less commonly, it may be bilateral. In bilateral cases, the deformity may be harder to notice because both shoulders may look similar. These patients may present with difficulty raising both arms overhead or a more noticeable short-neck appearance.

Sprengel deformity is not only a cosmetic difference. In some children, shoulder motion may be significantly limited, an omovertebral connection may exist between the shoulder blade and the spine, or other congenital problems involving the neck-spine region, ribs, cardiovascular system or renal/urinary system may be associated.

For this reason, evaluation should not be limited to the question “Is one shoulder higher?” Shoulder motion, scapular position, the neck and spine, the presence of an omovertebral connection and associated systemic anomalies should be assessed together.

No. In mild cases, the main concern may be cosmetic appearance. However, in more significant cases, limited shoulder motion, an omovertebral connection, neck-spine anomalies and other congenital problems may be associated. Therefore, Sprengel deformity should be evaluated from cosmetic, functional and associated-anomaly perspectives.

Sprengel deformity occurs when the shoulder blade does not descend to its normal position during early fetal development. In the early weeks of development, the shoulder blade and surrounding muscle structures are located higher, closer to the neck region. Over time, the shoulder blade is expected to migrate downward to its normal position on the back of the chest wall.

If this migration and development process is disrupted, the shoulder blade may remain higher than normal. The scapula may be smaller, rotated, abnormally shaped or connected abnormally to the spine. This forms the basis of Sprengel deformity.

The exact cause of Sprengel deformity is not clearly known in most patients. Genetic predisposition, structural differences during early embryonic development and environmental factors may all play a role. Most cases occur sporadically, but in some patients Sprengel deformity may be seen together with other congenital anomalies.

In Sprengel deformity, not only the shoulder blade but also the surrounding muscles may be affected. Some muscles may be weaker, shorter, abnormally positioned or partially underdeveloped. Therefore, limitation of shoulder motion may result not only from the high position of the scapula, but also from developmental differences in the surrounding muscles and soft tissues.

No. Sprengel deformity is usually a congenital structural difference that develops during the early fetal period. It is not caused by how the parents carried, held, slept or exercised the child after birth.

Sprengel deformity is usually noticed in childhood because of shoulder asymmetry, a visible scapular prominence or limitation in raising the arm. In some children, it is apparent from birth. In others, it becomes more noticeable as the child grows, especially when the shoulders and back are examined carefully.

Common signs and symptoms include:

• One shoulder appearing higher than the other

• A visible prominence of the shoulder blade on the back

• The affected shoulder appearing higher or more posterior

• Limitation in raising the arm

• Leaning the trunk sideways to compensate while lifting the arm

• Short-neck appearance

• Asymmetry of the neck-shoulder line

• Postural difference on the back

• Stiffness or limitation of shoulder movement

• Rarely, pain around the shoulder or neck

• Difficulty raising both arms overhead in bilateral cases

Limitation of movement is especially noticed when the child tries to raise the arm forward or sideways overhead. This may occur because the shoulder blade cannot rotate normally, because an omovertebral connection restricts scapular motion or because surrounding muscles are developmentally different.

In mild cases, the child may not have a significant limitation in daily life. These patients may be noticed mainly because of cosmetic appearance. In more pronounced cases, activities such as combing hair, reaching a high shelf, sports or overhead arm movements may be difficult.

Yes. When Sprengel deformity is bilateral, both shoulders may appear similar and asymmetry may be less obvious. In these patients, difficulty raising both arms overhead, short-neck appearance or prominent shoulder blades on the back may be more helpful diagnostic clues.

An omovertebral connection is an abnormal congenital connection between the shoulder blade and the spine. It may be made of bone, cartilage or fibrous tissue. It is not present in every patient, but when it exists, it may affect both shoulder movement and surgical planning.

During normal shoulder motion, the shoulder blade glides and rotates on the chest wall. When the arm is raised, the scapula must also rotate and move in coordination with the shoulder joint. If an omovertebral connection is present, the shoulder blade may be tethered to the spine and this normal rotation may be restricted.

An omovertebral connection is important because it may:

• Restrict scapular motion

• Limit shoulder abduction, meaning raising the arm sideways and upward

• Contribute to a more visible deformity

• Need to be released or removed during surgery

• Have important relationships with nearby nerve and vessel structures

• Be evaluated more clearly with CT or three-dimensional CT

An omovertebral connection may sometimes be visible on plain X-rays, but it is not always clearly seen. In patients considered for surgery, CT, three-dimensional CT reconstruction or, in selected cases, MRI may help define the structure of the connection and its relationship with surrounding tissues.

No. The presence of an omovertebral connection alone does not automatically mean that surgery is required. The decision should be made by evaluating shoulder motion, deformity severity, the child’s age, cosmetic concern, the structure of the omovertebral connection and the expected benefit-risk balance together. However, in patients planned for surgery, the omovertebral connection is very important for surgical strategy.

Sprengel deformity may occur alone in some patients, while in others it may be associated with additional congenital anomalies. For this reason, evaluation should not be limited to the shoulder blade and shoulder region. The neck-spine region, rib development, scoliosis, cardiovascular system and renal/urinary system may need attention.

Conditions that may be associated with Sprengel deformity include:

• Klippel-Feil syndrome

• Congenital fusion or deformity of cervical vertebrae

• Congenital scoliosis

• Rib anomalies

• Omovertebral connection

• Developmental abnormalities of muscles around the shoulder blade

• Short-neck appearance

• Torticollis or limited neck motion

• Cardiovascular anomalies

• Kidney and urinary tract anomalies

• Less commonly, other congenital skeletal anomalies

Not all of these conditions are present in every patient. A child with mild Sprengel deformity may have no additional problem. However, because Sprengel deformity develops during the congenital developmental period, associated anomalies should be considered especially in children with more noticeable deformity.

When needed, consultation with pediatric cardiology, pediatric urology, pediatric nephrology, pediatric neurology or other relevant specialties may be requested. The purpose is not to create unnecessary anxiety, but to avoid missing important associated problems.

The same investigation list is not applied to every patient. However, because Sprengel deformity may be associated with congenital systemic anomalies, cardiovascular and renal/urinary system evaluation may be needed especially when deformity is significant, additional examination findings are present or a syndrome is suspected. This decision should be made according to the child’s examination findings.

Sprengel deformity is usually diagnosed through careful physical examination and appropriate imaging. The goal of diagnosis is not only to show that the shoulder blade is high. The severity of the deformity, shoulder motion, presence of an omovertebral connection, neck-spine structure and associated anomalies should also be evaluated.

During diagnosis, the following questions are considered:

• How high is the shoulder blade?

• How noticeable is the shoulder height difference?

• How prominent is the shoulder blade on the back?

• How high can the arm be raised?

• Is shoulder motion limited on one side or both sides?

• Is there an omovertebral connection?

• Is neck motion limited?

• Is scoliosis or another spinal anomaly present?

• Is additional cardiovascular or renal/urinary evaluation needed?

• If surgery is considered, what anatomical planning is required?

What Is Assessed During the Examination?

During the examination, the child is assessed while standing, sitting and moving. Shoulder levels, scapular position, neck-shoulder line, back appearance, scoliosis signs and arm movements are examined.

The examination particularly evaluates:

• Difference in shoulder height

• Position of the shoulder blade on the back

• Scapular prominence

• Short-neck appearance

• Neck range of motion

• Shoulder abduction and flexion

• Trunk compensation while raising the arm

• Comparison of both shoulder movements

• Scoliosis or trunk asymmetry

• Neurological examination

• Muscle strength and arm use

• Previous surgeries or treatments

• Cosmetic and functional expectations of the child and family

Which Imaging Tests May Be Used?

The first imaging method in Sprengel deformity is usually X-ray. Shoulder, scapula, chest wall and cervical spine X-rays may provide information about scapular level, scapular shape, spinal anomalies and some omovertebral structures.

Computed tomography may be useful especially for evaluating an omovertebral connection, scapular position, bony connections and surgical planning. Three-dimensional CT images may make the relationship between the shoulder blade, spine and chest wall easier to understand.

Magnetic resonance imaging may be used when additional evaluation of non-bony soft tissue connections, fibrous bands, the spinal canal or neurological structures is needed. MRI is not required in every patient; the need is determined according to examination and X-ray/CT findings.

If cardiovascular or renal/urinary associated anomalies are suspected, additional tests may be requested. This evaluation is planned according to the patient’s age, severity of deformity, examination findings and suspicion of an associated syndrome.

X-rays are very helpful in diagnosis, but decisions in Sprengel deformity should not be made based on X-rays alone. Shoulder motion, cosmetic appearance, omovertebral connection, neck-spine structure, associated anomalies and the child’s age should be evaluated together.

The severity of Sprengel deformity is not evaluated only by how high the shoulder looks. Clinical appearance, shoulder motion and imaging findings should be considered together. Two children with similar X-ray findings may have different shoulder function, cosmetic concerns and treatment needs.

Important factors in evaluation include:

• Difference in shoulder height

• Prominence of the shoulder blade on the back

• Degree of arm elevation

• Effect of limited shoulder motion on daily life

• Presence of an omovertebral connection

• Neck-spine anomalies

• Scoliosis or rib anomalies

• The child’s age

• Cosmetic expectations of the child and family

• Expected benefit-risk balance of surgery

Clinical Evaluation: Cavendish Classification

The Cavendish classification is a practical classification used to describe the clinical appearance of Sprengel deformity. It mainly describes how visible the shoulder and scapular asymmetry is from the outside.

The general concept is as follows:

• In mild cases, the deformity may not be noticeable when the child is dressed.

• In moderate cases, prominence of the upper-inner part of the shoulder blade may be seen.

• In more significant cases, shoulder elevation is clearly visible.

• In severe cases, shoulder height difference and disruption of the neck-shoulder line are very noticeable.

This classification alone is not sufficient for deciding on surgery. Cosmetic appearance is important, but shoulder motion, omovertebral connection, associated anomalies and family expectations also affect treatment planning.

Radiological Evaluation: Rigault Classification

The Rigault classification is a radiological classification used to evaluate the level of the shoulder blade in relation to the spine. It considers the position of the superomedial angle of the scapula relative to the cervical and upper thoracic vertebrae.

Radiological evaluation may be useful for surgical planning. However, a decision such as “surgery is needed” or “surgery is not needed” should not be based only on radiological classification. Some patients may have marked imaging findings but good function; others may have a less severe appearance but meaningful limitation in motion.

No. Classifications such as Cavendish and Rigault help describe the deformity, but they are not enough by themselves to decide on surgery. Treatment planning should evaluate shoulder motion, cosmetic concern, omovertebral connection, associated anomalies, age and expected benefit-risk balance together.

Yes. Not every child with Sprengel deformity needs surgery. In children with mild deformity, good shoulder range of motion, no significant functional loss in daily life and no major cosmetic concern, follow-up may be sufficient.

The goal of non-surgical follow-up is not to completely correct the deformity. The goals are to preserve shoulder motion, monitor whether functional limitation develops, evaluate associated anomalies and provide the family with accurate information.

Important points during non-surgical follow-up include:

• Regular evaluation of shoulder range of motion

• Monitoring the child’s ability to raise the arm

• Neck and spine examination

• Follow-up for scoliosis or postural asymmetry

• Checking for pain or functional loss

• Evaluation for associated anomalies when needed

• Discussing cosmetic and functional expectations with the family

Physical therapy and exercises may help some patients maintain shoulder range of motion, support muscle control and improve postural awareness. However, exercise cannot completely correct the congenital high position of the shoulder blade. For this reason, the purpose of exercise should be explained realistically to families.

A decision for non-surgical follow-up does not mean that the child will never be checked again. The appearance of the deformity, shoulder motion, pain, functional capacity and associated neck-spine findings should be reassessed over time.

Exercise cannot completely correct the congenital high position of the shoulder blade. However, it may help maintain shoulder range of motion, support muscle control and improve daily function. In mild cases, observation and exercises may be sufficient. In patients with significant deformity and limited motion, surgical evaluation may be needed.

The decision for surgery in Sprengel deformity should be individualized. Not every high scapula requires surgery. Surgery is usually considered when shoulder motion is significantly limited, cosmetic deformity is a major concern or structural connections such as an omovertebral connection restrict movement.

Important factors in surgical evaluation include:

• Clearly noticeable shoulder elevation

• Scapular prominence causing cosmetic concern

• Significant limitation in raising the arm

• Shoulder motion limitation affecting daily life

• Presence of an omovertebral connection

• Marked rotation or deformity of the shoulder blade

• The child’s age and growth status

• Associated neck-spine anomalies

• Evaluation of nerve-vessel safety

• Expectations of the child and family

The goal of surgery is not to make the shoulder blade completely “normal” anatomically. The aims are to reduce shoulder height difference, improve the appearance of the shoulder blade, increase shoulder motion and make daily life easier for the child.

How Is the Age for Surgery Decided?

There is no single surgical age that applies to every child with Sprengel deformity. In the literature, the most advantageous period for suitable patients who need surgery is generally considered to be early childhood. Many sources report the 3–8 year age range, while some authors emphasize earlier ages or surgery before 6–8 years.

The reason for this age emphasis is that soft tissues are usually more flexible in younger children and the shoulder blade may be repositioned more safely. As the child grows, bones, muscles and soft tissues may become more rigid; surgical correction may become technically more difficult and the gain in shoulder motion may be more limited.

However, it is not correct to say that surgery can never be performed after the age of 8. Larger children, adolescents and selected adult patients may also be evaluated for surgery. In this age group, expectations, technical difficulty and risks should be discussed more carefully.

The key point is not to wait until the child is 18 years old, but to have the child evaluated by a specialist as early as possible once Sprengel deformity is noticed. After this evaluation, some children may be followed without surgery, while surgical timing can be planned for children with significant deformity, limited shoulder motion, omovertebral connection or major cosmetic concern.

The goal is not to operate on every child at an early age. The important point is timely evaluation. This allows mild cases to be safely observed and helps avoid missing the appropriate window in children who are expected to benefit from surgery.

No. There is no rule that Sprengel deformity should wait until the age of 18. In mild cases, observation may be sufficient. However, if there is clear shoulder elevation, limited motion, an omovertebral connection or significant cosmetic concern, the child should be evaluated by a specialist as early as possible. Larger children and selected adult patients may also be evaluated, but expectations, technical difficulty and risks are different in this age group.

The main goals of Sprengel deformity surgery are to bring the high shoulder blade to a lower and more balanced position, release tight soft tissues around the shoulder, evaluate the omovertebral connection if present and improve shoulder motion. The surgical technique may vary according to the patient’s age, severity of deformity, presence of an omovertebral connection, muscle structure and the surgeon’s plan.

Different surgical techniques have been described. These include the Woodward and Modified Woodward procedures, the Green procedure, and in some cases additional bony correction or scapuloplasty approaches. The appropriate method is chosen according to the patient’s anatomy and surgical goals.

During surgery, tight soft tissues around the shoulder blade are usually released, omovertebral or abnormal connections are carefully addressed if present, and arrangements are made to help the shoulder blade settle in a lower position. In some patients, additional procedures such as clavicle osteotomy may be needed to reduce nerve-vessel tension.

Sprengel deformity surgery requires experience. Important nerve and vessel structures are present around the shoulder blade. Forcing the shoulder blade too far downward may create nerve tension. Therefore, the goal is not excessive correction, but safe and functional improvement.

What Is the Modified Woodward Procedure?

The Modified Woodward procedure aims to release and reorganize the muscles and soft tissues that hold the shoulder blade in a high position, allowing the scapula to move to a lower position. In this method, the spinal attachments of muscles such as the trapezius and rhomboids may be repositioned to help the shoulder blade descend to a more balanced level.

This procedure is an important surgical option in selected children with significant Sprengel deformity. Its success is closely related to correct patient selection, evaluation of the omovertebral connection, nerve-vessel safety, soft tissue balance and postoperative rehabilitation.

How Is the Omovertebral Connection Evaluated During Surgery?

If an omovertebral connection is present, the surgical plan should take it into account. The connection may be bony, cartilaginous or fibrous, and it may restrict movement between the shoulder blade and the spine. This can limit the ability to bring the scapula downward and restrict normal scapular rotation.

Before surgery, CT or MRI may be used to evaluate the structure of the omovertebral connection and its relationship with surrounding tissues. During surgery, the connection may be carefully released or removed. Protection of nearby nerve and vessel structures is very important during this step.

No. The goal of Sprengel deformity surgery is not to convert the shoulder blade into completely normal anatomy. The goals are to reduce shoulder elevation, improve cosmetic appearance, increase shoulder motion and make daily life easier for the child. Complete symmetry and completely normal shoulder motion may not be possible in every patient.

Recovery after Sprengel deformity surgery varies according to the patient’s age, surgical technique, severity of the deformity, presence of an omovertebral connection, whether additional procedures were performed and the postoperative rehabilitation plan. Recovery is not completed in a single day. Tissue healing, regaining shoulder motion and returning to daily activities take time.

In the early postoperative period, wound care, pain control, protection of the arm, nerve-vessel examination and healing of the soft tissues around the shoulder are important. Depending on the surgeon’s preference, an arm sling, movement restriction or a period of controlled rest may be recommended.

Early Follow-Up Period

In the early period, wound healing, pain level, circulation to the shoulder and arm, hand and arm movement, numbness or weakness are monitored. Fever, wound drainage, progressively increasing pain or significant worsening of arm movement requires early evaluation.

During follow-up visits, the position of the shoulder blade, shoulder motion, the child’s pain and daily use of the arm are assessed. The need for imaging depends on the type of surgery and the surgeon’s plan.

Rehabilitation and Regaining Motion

The aim of rehabilitation is to safely improve shoulder range of motion, support muscle control, improve postural awareness and help the child use the arm more comfortably in daily life. Exercises should be planned according to the child’s age, surgical technique and healing status.

Excessive and uncontrolled early force may harm soft tissues, while completely stopping exercises may negatively affect motion recovery. Therefore, rehabilitation should be performed within the timing and limits recommended by the surgeon.

After Sprengel deformity surgery, many patients may experience meaningful improvement in cosmetic appearance and shoulder motion. However, outcomes vary from patient to patient because of the congenital nature of the deformity, muscle development, omovertebral connection and associated anomalies.

No. Outcomes in Sprengel deformity vary according to the severity of the deformity, the child’s age, muscle development, presence of an omovertebral connection, associated anomalies and the surgical technique used. Some patients may have marked cosmetic improvement, while motion gain may be more limited in others.

Sprengel deformity surgery can be an effective option for improving shoulder appearance and motion in many patients. However, like every surgical procedure, it has risks. These risks may vary according to the child’s age, severity of deformity, presence of an omovertebral connection, associated anomalies, surgical technique and additional procedures.

Possible risks of Sprengel deformity surgery include:

• Infection

• Bleeding

• Wound healing problems

• Noticeable surgical scar

• Less improvement in shoulder motion than expected

• Less cosmetic correction than expected

• Recurrent or persistent scapular prominence

• Nerve stretching or nerve involvement

• Numbness, weakness or temporary nerve findings in the arm

• Effects on surrounding tissues during omovertebral connection release

• Problems with muscle balance around the shoulder

• Scapular prominence or winging appearance

• Pain not completely resolving

• Need for additional surgery or revision

The presence of these risks does not mean that every patient will develop a problem. However, the family should clearly understand the expected benefits, limitations, possible risks and postoperative process before surgery.

Early Risks

In the early period, wound healing, infection, pain control, bleeding, unexpected reduction in arm movement, numbness or weakness are monitored. The soft tissues around the shoulder and neck may be sensitive after surgery.

Weakness in the arm, problems with hand or finger movement, marked numbness, progressively increasing pain, fever or wound drainage should be evaluated promptly.

Long-Term Risks and Expectation Management

In the long term, the most important issue is expectation management. Because Sprengel deformity is a congenital structural difference, a completely normal and symmetrical shoulder appearance may not always be possible after surgery. Even if shoulder motion improves, full range of motion may not be achieved in every patient.

In some patients, scapular prominence may partially remain, shoulder height may not become completely equal or asymmetry may persist because of muscle development differences. Nevertheless, in properly selected patients, surgery may provide meaningful improvement in cosmetic appearance and shoulder function.

It is not possible to eliminate risks completely. However, careful patient selection, appropriate surgical timing, detailed evaluation of the omovertebral connection, assessment of associated anomalies, protection of nerve-vessel structures, realistic expectation setting and regular postoperative follow-up may help reduce risks.

Is Sprengel deformity only a cosmetic problem?

False: Sprengel deformity is only a high shoulder appearance.

Correct: In mild cases, the main issue may be cosmetic appearance. However, in more significant cases, limitation of arm elevation, an omovertebral connection, neck-spine anomalies, scoliosis, rib anomalies or other congenital problems may be associated. Evaluation should not be based on appearance alone.

Does every child with Sprengel deformity need surgery?

False: Every child diagnosed with Sprengel deformity must have surgery.

Correct: Not every child needs surgery. In mild cases with good shoulder motion and no significant cosmetic or functional limitation, observation may be sufficient. Surgery is considered when there is meaningful limitation of motion, significant cosmetic concern, an omovertebral connection affecting function or other factors that make surgical correction beneficial.

Should Sprengel deformity wait until the age of 18?

False: Even if surgery is needed, children with Sprengel deformity should wait until they are 18 years old.

Correct: There is no rule that Sprengel deformity should wait until the age of 18. If the deformity is noticed, the child should be evaluated by a specialist as early as possible. Mild cases may be observed, but if there is clear shoulder elevation, limited motion, an omovertebral connection or significant cosmetic concern, delaying evaluation until late adolescence may not be appropriate.

Can exercise completely correct Sprengel deformity?

False: With regular exercise, the shoulder blade will descend to its normal position and the deformity will completely disappear.

Correct: Exercise may help support shoulder range of motion, muscle control and posture awareness. However, it cannot completely correct the congenital high position of the shoulder blade. The goal of exercise is to support function, not to eliminate the bony deformity.

Is an omovertebral connection a malignant tumor?

False: An omovertebral connection is a dangerous or malignant tumor.

Correct: An omovertebral connection is a congenital connection between the shoulder blade and the spine. It may be made of bone, cartilage or fibrous tissue. It is not a tumor. However, it may restrict scapular movement and affect surgical planning, so it should be evaluated carefully.

Will the shoulder become completely normal and symmetrical after surgery?

False: After Sprengel deformity surgery, both shoulders become completely equal and normal.

Correct: The goal of surgery is to reduce shoulder elevation, improve the appearance of the shoulder blade and increase shoulder motion. Complete symmetry or completely normal shoulder movement may not be possible in every patient. Realistic expectation setting is very important before surgery.

Does Sprengel deformity disappear by itself as the child grows?

False: As the child grows, the high shoulder blade will descend by itself and become normal.

Correct: Sprengel deformity is a congenital structural difference. Body proportions may change as the child grows, and the deformity may appear differently, but the high position of the shoulder blade usually does not completely correct itself. Mild cases may be followed, while more significant cases require specialist evaluation.

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