Sprengel’s shoulder, also known as Sprengel deformity, is a rare congenital condition where the shoulder blade (scapula) sits higher than normal on the back. This abnormal position can limit shoulder movement, cause visible asymmetry, and in some cases, lead to discomfort or functional challenges. Although it is typically diagnosed in childhood, early identification and treatment are essential for improving shoulder mobility and overall quality of life. This guide explains everything you need to know about Sprengel’s shoulder, including its symptoms, causes, diagnosis, severity grading, treatment options, and the critical role of physiotherapy in managing the condition effectively.

Understanding Sprengel’s Shoulder (Sprengel Deformity)

Sprengel’s shoulder is a congenital condition characterised by the abnormal elevation of the scapula (shoulder blade). It occurs when the scapula fails to descend to its normal position during early fetal development, resulting in a high-riding shoulder that may appear smaller or misshapen. This deformity affects the surrounding periscapular muscles, often leading to limited shoulder movement and aesthetic concerns.

The condition can vary in severity, from mild cases with minimal functional impact to more noticeable deformities that restrict movement and cause discomfort. In many cases, an additional bony connection called the omovertebral bone forms between the scapula and the cervical spine, further limiting mobility.

Understanding the anatomy and mechanics of Sprengel’s shoulder is essential for determining the best approach to treatment, whether through physiotherapy, surgery, or a combination of both.

Common Symptoms of Sprengel’s Shoulder

The symptoms of Sprengel’s shoulder can range from mild cosmetic concerns to significant functional limitations, depending on the severity of the deformity. While some individuals may experience few noticeable problems, others may face daily challenges with shoulder movement and posture.

Typical symptoms include:

• A visibly elevated and smaller shoulder blade on one side
• Restricted shoulder movement, particularly difficulty lifting the arm above shoulder level
• Shoulder asymmetry or unevenness of the upper back
• Neck or upper back discomfort in moderate to severe cases
• The presence of a bony bridge (omovertebral bone) between the scapula and cervical spine, which may further limit mobility

Causes and Risk Factors

Sprengel’s shoulder is a congenital condition, meaning it develops before birth. The exact cause is not fully understood, but it results from the scapula failing to descend to its normal position during fetal development. Key causes and risk factors for this condition include:

• Congenital origin: The condition typically arises sporadically without a clear cause, though it is sometimes linked to other congenital abnormalities.
• Genetic factors: While rare, some cases may have a familial pattern, suggesting a possible genetic influence.
• Associated syndromes: Sprengel’s deformity may occur alongside other musculoskeletal conditions, such as Klippel-Feil syndrome (fusion of cervical vertebrae), scoliosis, or rib anomalies.

How Sprengel Deformity is Diagnosed

Diagnosing Sprengel’s shoulder involves a combination of clinical evaluation and imaging studies to assess the severity of the deformity and guide treatment planning. Early diagnosis is important to ensure timely intervention and improve functional outcomes. Key steps in diagnosis include:

Clinical Examination

• Observation of a visibly elevated scapula and assessment of shoulder asymmetry
• Evaluation of shoulder range of motion, particularly arm elevation
• Palpation to detect any abnormal bony structures, such as an omovertebral bone

Radiology and Imaging

• X-rays: Used to confirm the position of the scapula and identify associated skeletal abnormalities
• CT or MRI scans: Provide detailed images of bone and soft tissue, helping to assess the presence of the omovertebral bone and plan surgical or physiotherapy approaches (sprengel deformity radiology)

Classification and Severity Grading

To guide treatment decisions, Sprengel’s shoulder is typically classified based on its visible and functional impact. The most widely used system is the Cavendish classification, which grades the severity of the deformity from mild to severe.

• Grade 1 (Very Mild): Shoulder asymmetry is barely noticeable, often only seen when undressed.
• Grade 2 (Mild): A slight elevation of the shoulder is visible even when clothed, but movement is only mildly restricted.
• Grade 3 (Moderate): The elevated scapula is easily noticeable, with a prominent lump and moderate movement restriction.
• Grade 4 (Severe): The scapula is positioned close to the neck, with significant functional limitations and obvious deformity.

The severity of the deformity, as graded by this system, helps determine whether physiotherapy alone will suffice or if surgical intervention should be considered.

Sprengel Shoulder Treatment Options

The treatment approach for Sprengel’s shoulder depends on the severity of the deformity and the level of functional impairment. While mild cases may require only observation or physiotherapy, more pronounced deformities could benefit from surgical correction. Main treatment options include:

Conservative (non-surgical) management

• Physiotherapy is the first line of treatment for many cases, aiming to improve shoulder mobility and strengthen periscapular muscles.
• Regular monitoring to track shoulder function and appearance over time.

Surgical intervention

• Surgery is typically considered for moderate to severe cases (Cavendish Grade 3 or 4) where there is significant restriction of movement or cosmetic concern.
• Procedures such as the Woodward procedure aim to reposition the scapula to a more normal position and may involve removal of the omovertebral bone if present.

Physiotherapy for Sprengel’s Shoulder: Goals, Exercises, and Early Intervention

Physiotherapy plays a crucial role in managing Sprengel’s shoulder, particularly in mild to moderate cases where surgery is not immediately indicated. Even after surgical correction, physiotherapy remains essential for restoring mobility and strengthening shoulder function.

Goals of physiotherapy

• Enhancing shoulder range of motion
• Strengthening periscapular muscles to support better posture and movement
• Preventing muscle tightness and joint stiffness
• Improving overall shoulder function and comfort

Key physiotherapy exercises

• Scapular stabilisation: Exercises such as scapular retractions and shoulder blade squeezes to promote better alignment.
• Shoulder mobility drills: Arm raises, wall slides, and gentle stretching to increase flexibility.
• Strengthening routines: Resistance band exercises targeting the shoulder girdle and surrounding muscles.
• Stretching: Gentle neck and shoulder stretches to maintain flexibility and reduce tension.

Importance of early intervention

Starting physiotherapy early helps maximise shoulder function, even in cases where surgical treatment might be planned later. A well-structured home exercise programme, guided by a physiotherapist, supports long-term improvements and helps prevent the condition from worsening.

Surgical Treatment: When is it Needed?

Surgical treatment for Sprengel’s shoulder is typically reserved for moderate to severe cases where the deformity significantly restricts shoulder movement or causes cosmetic concerns. Surgery aims to reposition the elevated scapula to improve both function and appearance.

Key points about surgical treatment:

• Common procedures: The most widely used surgery is the Woodward procedure, which involves detaching and lowering the scapula to a more normal position. In many cases, the omovertebral bone (if present) is also removed to enhance mobility.
• Ideal age for surgery: Surgery is generally recommended between the ages of 3 and 8 years, as younger patients tend to achieve better outcomes in terms of both function and cosmetic appearance.
• Risks and benefits: While surgery can lead to significant improvements, it carries typical surgical risks such as infection, scarring, and nerve injury. However, most patients experience noticeable gains in shoulder mobility and reduced deformity.

Note: Decisions about surgery are made based on the severity of the condition, the child’s age, and the expected benefits of intervention, often in consultation with both orthopaedic surgeons and physiotherapists.

Recovery and Post-Treatment Care

Recovery following treatment for Sprengel’s shoulder, especially after surgery, requires a structured approach to ensure optimal results. Post-treatment care focuses on healing, restoring shoulder movement, and preventing complications. Key aspects of recovery and care include:

• Post-operative physiotherapy: Physiotherapy begins soon after surgery to maintain and gradually improve shoulder mobility. Exercises are introduced progressively to strengthen the shoulder and surrounding muscles.
• Monitoring healing: Regular follow-up appointments are essential to check wound healing, monitor for complications, and assess improvements in range of motion and appearance.
• Expected recovery timeline: Initial healing from surgery typically takes a few weeks, but full rehabilitation, including regaining strength and flexibility, may take several months.
• Home care guidance: Patients and caregivers are advised on proper wound care, activity restrictions, and gradual reintroduction of daily activities.

Potential Complications if Left Untreated

If Sprengel’s shoulder is left untreated, especially in moderate to severe cases, it can lead to several long-term complications that may affect both function and quality of life.

Possible complications include:

• Persistent functional limitations: Reduced shoulder mobility can hinder daily activities, such as reaching overhead or lifting objects.
• Postural imbalances: A high-riding scapula may contribute to uneven posture, which can strain the neck, shoulders, and back over time.
• Muscle weakness and tightness: Without intervention, the periscapular muscles may become weak or stiff, further restricting movement.
• Psychosocial concerns: Visible shoulder asymmetry, especially in children and adolescents, may lead to self-esteem issues or social discomfort.
• Rare chronic discomfort: Although not always painful, some individuals may develop ongoing neck or shoulder discomfort due to altered mechanics.

Why Choose MaxAtHome for Physiotherapy

Managing Sprengel’s shoulder requires a dedicated physiotherapy plan tailored to each patient’s needs, and MaxAtHome offers expert care directly in the comfort of your home. With personalised attention and skilled physiotherapists, MaxAtHome helps patients improve shoulder mobility, strength, and function effectively and safely.

Benefits of choosing MaxAtHome include:

• At-home physiotherapy services delivered by experienced professionals
• Personalised exercise plans focused on improving range of motion and strengthening periscapular muscles
• Regular progress monitoring to ensure safe and steady recovery
• Expert guidance on home exercises and long-term care strategies
• Convenient scheduling with minimal disruption to daily routines

If you are looking for professional physiotherapy to manage Sprengel’s shoulder at home, book your sessions easily through our online platform, or call 01244781023 to get started today.

Frequently Asked Questions

What is Sprengel shoulder and how is it identified?

Sprengel’s shoulder is a congenital condition where the shoulder blade is positioned higher than normal. It is identified through clinical examination and imaging, with symptoms including visible shoulder asymmetry and limited shoulder movement.

How do you exercise Sprengel's shoulder?

Exercises focus on improving mobility and strengthening periscapular muscles. These may include shoulder blade squeezes, arm raises, and scapular stabilisation exercises, guided by a physiotherapist.

What is physiotherapy treatment for Sprengel shoulder?

Physiotherapy aims to enhance range of motion, strengthen surrounding muscles, and improve posture. It includes personalised exercises and manual therapy, often combined with home exercise guidance.

What are the common symptoms of Sprengel shoulder?

Symptoms typically include a high-riding scapula, shoulder asymmetry, limited arm elevation, and in some cases, neck or back discomfort.

How does radiology help in diagnosing Sprengel deformity?

Radiology, such as X-rays, CT scans, or MRI, confirms the elevated scapula’s position and can detect associated abnormalities like the omovertebral bone, helping guide treatment decisions.

Can Sprengel shoulder affect both shoulders?

It is usually unilateral (affecting one shoulder), but in very rare cases, it may involve both shoulders.

At what age is Sprengel deformity usually treated?

Treatment often begins in early childhood, with surgery ideally performed between ages 3 and 8 if needed. Physiotherapy can start as soon as the condition is diagnosed.

Is surgery necessary for all cases of Sprengel shoulder?

No, mild cases can often be managed effectively with physiotherapy alone. Surgery is typically considered for more severe cases with significant functional or cosmetic concerns.

What is the success rate of Sprengel shoulder surgery?

Surgery generally has a high success rate in improving both the function and appearance of the shoulder, especially when performed at a young age.

How long is the recovery period after surgery?

Initial healing takes a few weeks, but full rehabilitation, including physiotherapy, may continue for several months to achieve optimal results.

Can physiotherapy help improve shoulder function?

Yes, physiotherapy plays a key role in improving mobility, strength, and posture, whether used as a standalone treatment or after surgery.

Are there any long-term effects of untreated Sprengel deformity?

If left untreated, moderate to severe cases may result in lasting mobility restrictions, postural issues, and in rare cases, chronic discomfort.

What is the omovertebral bone and why is it important?

The omovertebral bone is an abnormal bony connection between the scapula and cervical spine, often seen in Sprengel’s shoulder. It can further limit mobility and is usually removed during surgery if present.

Can children live normally with Sprengel deformity?

Many children with mild cases live normal lives with little functional impact, though monitoring and physiotherapy are recommended to maintain shoulder health.

Is Sprengel deformity painful?

It is not typically painful, but some individuals may experience discomfort, especially if movement is significantly restricted or other musculoskeletal issues are present.

How do doctors classify the severity of Sprengel shoulder?

Doctors use the Cavendish classification system, which grades the deformity from 1 (very mild) to 4 (severe), based on cosmetic appearance and functional impact.