What is Sprengel's Shoulder?

Sprengel’s Shoulder, also known as Sprengel’s Deformity, is a rare congenital condition where one shoulder blade (scapula) is positioned higher than the other. It can affect both the appearance and movement of the shoulder, typically becoming noticeable in early childhood. While it may seem like a minor cosmetic issue, it can lead to long-term mobility challenges if not addressed appropriately.

This blog covers the symptoms, causes, diagnosis, and treatment options for Sprengel’s Shoulder, with a special focus on physiotherapy-based management.

What is Sprengel’s Shoulder?

Sprengel’s Shoulder is a developmental defect that occurs when the shoulder blade fails to descend to its normal position during fetal development. As a result, the affected scapula remains elevated and underdeveloped, often with a rotated or misshapen structure. It most commonly affects one side of the body (unilateral), though bilateral cases also exist.

Symptoms of Sprengel’s Shoulder

Symptoms can range from mild to severe, and include:

• Visible elevation of one shoulder compared to the other
• Limited range of motion, especially during overhead activities
• Abnormally shaped or small scapula
• Muscle weakness in the shoulder and upper back
• Neck stiffness or head tilt due to compensation
• Associated spinal or rib abnormalities in some cases

Causes of Sprengel’s Shoulder

Sprengel’s Shoulder is congenital, meaning it develops before birth. The exact cause is not fully understood, but the condition is believed to result from:

• Failure of the scapula to descend between the 9th and 12th weeks of gestation
• Possible genetic influences
• Association with other congenital syndromes, including:
  • Klippel-Feil syndrome
  • Congenital scoliosis
  • Spina bifida
  • Rib or cervical spine anomalies

Diagnosis

Diagnosis is typically made during early childhood through a combination of clinical and imaging assessments:

• Physical examination to evaluate shoulder height, range of motion, and muscle strength
• X-rays or CT scans to visualize the scapula and spine alignment
• MRI to assess muscle development and detect associated abnormalities
• Cavendish classification system is often used to determine the severity (Grade I to IV)

Associated Conditions

Sprengel’s Shoulder can be accompanied by other skeletal and muscular abnormalities, such as:

• Congenital scoliosis
• Fused cervical vertebrae (as seen in Klippel-Feil syndrome)
• Omovertebral bone (a bony or fibrous connection between the scapula and cervical spine)
• Rib malformations

Treatment Options

Non-Surgical Management

Mild to moderate cases without severe functional limitations can often be managed with:

• Physiotherapy to improve shoulder mobility and strengthen surrounding muscles
• Stretching exercises to increase flexibility
• Postural correction and education to reduce compensation habits
• Occupational therapy to assist in performing daily tasks effectively

Non-surgical approaches aim to enhance quality of life and prevent long-term complications.

Surgical Treatment

Surgery may be recommended for more severe cases, particularly when:

• There is a significant restriction in shoulder movement
• Cosmetic appearance is a concern
• The child is between 3 and 8 years old (ideal window for surgical correction)

Common surgical procedures include:

• Woodward procedure – involves repositioning the scapula to a lower position
• Green’s procedure – partial removal of the scapula to allow better positioning and movement
• Removal of the omovertebral bone, if present

Post-surgical physiotherapy is essential to restore function and maintain range of motion.

Role of Physiotherapy

Physiotherapy plays a key role in both conservative and post-surgical management. Its goals include:

• Improving shoulder range of motion
• Strengthening shoulder girdle and postural muscles
• Preventing compensatory movements and muscle imbalances
• Enhancing functional ability in daily activities

Common Physiotherapy Interventions:

• Scapular stabilization exercises
• Assisted shoulder mobilization
• Resistance band training
• Postural alignment therapy
• Home exercise programs tailored to the child's needs

Early intervention through physiotherapy can lead to significant improvements in both movement and quality of life.

Think of physiotherapy as your child’s personal movement coach, which is gentle, goal-focused, and totally game-changing. Curious how it works in real life? Check out our Physiotherapy services to see the magic in motion.

When to Consult a Specialist

Parents should seek medical attention if a child shows:

• Noticeably higher shoulder on one side
• Difficulty lifting the arm or performing overhead movements
• Asymmetry in posture or delayed physical development
• Discomfort or limited activity participation due to shoulder dysfunction

An orthopedic consultation, supported by imaging, is often the first step toward proper diagnosis and treatment planning.

Conclusion

Sprengel’s Shoulder is a rare yet impactful congenital condition that affects shoulder alignment and function. While some cases are mild and manageable through physiotherapy, others may require surgical intervention to restore normal function and appearance. Early diagnosis and a multidisciplinary treatment approach can greatly enhance long-term outcomes.

For children diagnosed with Sprengel’s Shoulder, a structured rehabilitation plan combining expert physiotherapy and, if needed, timely surgical correction offers the best path forward.

The safest and most effective way to manage this condition is through Physiotherapy, and which will be the best if not TruePal, so register or enrol yourself to our physiotherfapy sessions to experience the most comfortable experience to minimize pain. 

FAQs

1. What is the difference between Klippel-Feil and Sprengel?

• Sprengel’s Deformity is a congenital condition where one shoulder blade (scapula) is positioned higher than normal. It often leads to restricted shoulder movement.
• Klippel-Feil Syndrome (KFS) is a condition characterized by the fusion of two or more cervical vertebrae. It often includes a short neck, limited neck mobility, and a low posterior hairline.

Key difference:
Sprengel affects the shoulder blade, while Klippel-Feil affects the neck vertebrae. However, they can co-occur.

2. How do you treat Sprengel's deformity?

Treatment depends on severity:

• Mild cases: Managed with physiotherapy to improve range of motion and strength.
• Moderate to severe cases: May require surgical correction, especially if function is significantly limited or for cosmetic reasons. The Woodward procedure is the most commonly performed surgery.

3. What is the Sprengel deformity triad?

The classical triad includes:

• Elevated scapula
• Medial scapular rotation
• Hypoplastic (underdeveloped) scapula

Sometimes, an omovertebral bone (a fibrous or bony connection between scapula and spine) is also present.

4. What is the Woodward procedure?

The Woodward procedure is a surgical correction for Sprengel's deformity. It involves:

• Detaching and repositioning muscles attached to the scapula (like the trapezius and rhomboids),
• Moving the scapula down to a more normal position,
• Sometimes removing the omovertebral bone.

It improves both function and appearance, especially when done before age 8.

5. What are the anomalies associated with Sprengel's deformity?

Sprengel’s deformity may be associated with:

• Klippel-Feil syndrome
• Scoliosis
• Rib abnormalities
• Spina bifida
• Omovertebral bone (in 20-50% of cases)
• Muscle hypoplasia or atrophy (e.g., trapezius)

6. What is the unhappy triad of shoulder dislocation?

The unhappy triad of the shoulder typically refers to:

• Anterior shoulder dislocation
• Rotator cuff tear
• Axillary nerve injury

7. What exercises are good for Sprengel shoulder?

For mild cases or post-surgery rehab:

• Scapular retraction exercises
• Wall angels
• Shoulder blade squeezes
• Assisted arm lifts (pulleys or therapist-guided)
• Resistance band shoulder abduction
• Postural correction exercises

Always consult a pediatric physiotherapist before beginning a regimen. You can also reach out to us.