08/10/2025
🦴 Sprengel Deformity: Understanding Congenital Elevation of the Scapula
📌Sprengel deformity, also known as congenital elevation of the scapula, is a complex congenital anomaly characterized by the persistent upward displacement of the scapula (shoulder blade) relative to the thoracic cage. This condition was first described by Eulenberg in 1863.
👉
🔍 Key Characteristics and Associated Features
The severity of the functional impairment generally correlates with the severity of the deformity.
■ Scapular Abnormalities:
The affected scapula is typically hypoplastic (underdeveloped) and often misshapen. In severe cases, the scapula can be so elevated that it almost touches the occiput (the back of the head).
■ Omovertebral Bone:
In about half of patients, an extra ossicle is present, known as the omovertebral bone. This structure is a rhomboidal plaque of bone and cartilage contained within a strong fascial sheath. It extends from the superior angle of the scapula to the spinous process, lamina, or transverse process of one or more lower cervical vertebrae. Recognizing this abnormality is crucial for surgical planning.
■ Functional Impairment:
The primary limitation of shoulder movement is abduction, caused by diminished scapulothoracic motion. Patients may also present with their head deviated toward the affected side.
■ Associated Congenital Anomalies:
The presence of Sprengel deformity can indicate abnormalities in other organ systems. Other anomalies often present include cervical ribs, malformations of ribs, and anomalies of the cervical vertebrae, such as Klippel-Feil syndrome.
👇
📊 Classifying Severity
The severity of Sprengel deformity is often assessed using the Cavendish Classification, which grades the deformity from mild to severe:
■ Grade 1 (Very Mild): Shoulders are level. Deformity is not visible when the patient is dressed.
■ Grade 2 (Mild): Shoulders are almost level. Deformity is visible as a lump in the web of the neck when the patient is dressed.
■ Grade 3 (Moderate): Shoulders are elevated 2–5 cm. Deformity is easily seen.
■ Grade 4 (Severe): Shoulder is grossly elevated. The superior angle of the scapula lies near the occiput.
👇
🧠 Diagnosis and Surgical Planning
■ Radiographic workup is essential, including plain radiographs to assess the level of the scapula relative to the vertebrae and the contralateral side, and to recognize associated abnormalities like the omovertebral bone.
■ Morphometric analysis using three-dimensional CT (3D-CT) has shown that affected scapulae typically have a characteristic shape with a decreased height-to-width ratio.
■ 3D-CT can be helpful in planning scapuloplasty and delineating the deformity, as the point of tethering of the omovertebral connection, when present, may influence the shape, rotation, and superior displacement of the scapula.
👇
💡 Treatment Considerations
■ If the deformity and impairment are mild (e.g., Cavendish Grade 1 or mild Grade 2), no treatment may be indicated.
■ For more severe cases, surgery may be necessary, provided the long-term functional and cosmetic outcomes are carefully measured against the risks.
Surgical Timing and Outcomes:
■ Surgery to bring the scapula inferiorly to a near-normal position is ideally attempted soon after 3 years of age. The operation becomes significantly more challenging as the child grows.
■ In one 26-year review, surgically treated patients showed almost 40 degrees more abduction than those treated non-surgically, along with subjective cosmetic improvement.
■ One critical consideration during surgical correction is that the affected scapula is hypoplastic; therefore, surgeons should aim to align the spine of the scapula with the opposite side, rather than aligning the inferior angles.
👇
🩺 Surgical Procedures
Numerous operations have been developed to correct Sprengel deformity, including:
■ Green Procedure (and Modified):
This involves surgical release of muscles from the scapula, excision of the supraspinatus portion of the scapula, removal of any omovertebral bone, inferior displacement of the scapula, and muscle reattachment. One study reported that the modified Green procedure resulted in a 63-degree increase in range of motion at 4-year follow-up.
■ Woodward Operation:
This procedure involves transferring the origin of the trapezius muscle to a more inferior position on the spinous processes. It is often preferred for functional impairment. Advantages cited for the Woodward procedure include a lower risk of scar-keloid formation (which might fix the scapula in a poor position), greater mobilization capability, and a thinner postoperative scar compared to Green's procedure. Long-term follow-up of the Woodward procedure demonstrated improvement in Cavendish grade and significant improvement in shoulder abduction and improved contrast.
■ Mears Technique:
This technique includes partial resection of the scapula, removal of any omovertebral communication, and release of the long head of the triceps. Mears suggested that the contracture of the long head of the triceps significantly inhibits full abduction.
👇
⚠️ Surgical Risks and Complications
■ The most severe complication of surgery for Sprengel deformity is brachial plexus palsy.
■ In older children, attempting to move the scapula too far inferiorly toward its normal level can cause injury to the brachial plexus.
■ To avoid this, some surgeons recommend morcellation of the clavicle (cutting it into small pieces and replacing them in the periosteal tube) on the ipsilateral side, especially in cases of severe deformity or in children older than 8 years, where a 2-cm midclavicular osteotomy may be recommended to decompress the brachial plexus and first rib.
