"Understanding Sprengel Deformity: Diagnosis, Treatment, and Outcomes"
Learn about Sprengel's Deformity, a congenital condition characterized by a small and undescended scapula. Understand its diagnosis, treatment options, associated conditions, and surgical procedures. Explore the epidemiology, etiology, anatomy, presentation, and outcomes of this condition.
Sprengel's Deformity: A Congenital Condition
Sprengel's Deformity is a congenital condition characterized by a small and undescended scapula often associated with scapular winging and scapular hypoplasia.
Diagnosis is made clinically with a high-riding, medially rotated, triangular-shaped scapula, with associated limitations in shoulder abduction and flexion.
Treatment is observation in the absence of shoulder dysfunction. Operative management is indicated in the presence of severe cosmetic concerns or functional deformities (abduction < 110-120 degrees).
Epidemiology
Incidence: Sprengel's Deformity is the most common congenital shoulder anomaly in children.
Demographics: It has a male to female ratio of 1:3.
Anatomic location: It is bilateral in 10-30% of cases.
Etiology
Associated conditions: Sprengel's Deformity is often associated with scapular winging, hypoplasia, and an omovertebral connection between the superior medial angle of the scapula and the cervical spine (30-50%).
Pathophysiology: It is caused by the interruption of embryonic subclavian blood supply at the level of the subclavian, internal thoracic, or suprascapular artery. It is important to note that Poland syndrome is characterized by subclavian artery interruption proximal to the internal thoracic and distal to the vertebral artery.
Associated diseases: Sprengel's Deformity may be associated with Klippel-Feil (approximately 1/3 have Sprengel deformity), congenital scoliosis, upper extremity anomalies, diastematomyelia, and kidney disease.
Anatomy
Osteology
Sprengel's Deformity affects the scapula, which consists of the body, spine, acromion, coracoid process, and glenoid.
Articulations
Sprengel's Deformity affects the AC joint and the glenohumeral diarthrodial articulations of the scapula.
Muscles
The muscles that insert on the medial border of the scapula include levator scapulae, rhomboids major and minor, teres major, latissimus dorsi, and a small portion just proximal to the inferior angle.
Presentation
Symptoms
Sprengel's Deformity is often referred for evaluation of scoliosis.
Physical exam
Physical examination reveals a high-riding, medially rotated scapula with a loss of the long medial border. It has an equilateral triangle-like shape. Shoulder abduction is most limited due to the loss of normal scapulothoracic motion and glenoid malpositioning. Forward flexion is also limited.
Treatment
Nonoperative
Observation: Observation is recommended in the absence of severe cosmetic concerns or loss of shoulder function.
Understanding Sprengel's Deformity: Diagnosis, Treatment, and More
Learn about Sprengel's Deformity, a congenital condition characterized by a small and undescended scapula. Understand its diagnosis, treatment options, associated conditions, and surgical procedures. Explore the epidemiology, etiology, anatomy, presentation, and outcomes of this condition.
Operative
Surgical correction: Surgical correction is indicated in the presence of severe cosmetic concerns or functional deformities (abduction < 110-120 degrees).
Pre-operative planning: Pre-operative planning may involve MRI or CT to identify omovertebral bar.
- Woodward procedure: This involves the detachment and reattachment of the medial parascapular muscles at the spinous process origin to allow the scapula to move inferiorly and rotate into more shoulder abduction. A modified Woodward procedure includes resection of the superiormedial border of the scapula in conjunction with surgical descent.
- Schrock, Green procedure: This procedure involves the extraperiosteal detachment of paraspinal muscles at the scapular insertion and reinsertion after inferior movement of the scapula with traction cables.
- Clavicle osteotomy: In severe deformities, a clavicle osteotomy may be performed in conjunction with other procedures to avoid brachial plexus injury.
- Bony resection: Bony resection, involving the extraperiosteal resection of the proximal scapular prominence, may be done for cosmetic concerns and can be performed alone or with other procedures.
Outcomes
Woodward and Green procedures: These procedures can improve abduction by 40-50 degrees.
Sprengel Deformity (Congenital Elevation of Scapula)
Classification
There are two main classifications used to describe Sprengel's deformity:
| Cavendish's Clinical Classification | Rigault and Pouliquen's Radiological Classification |
|---|---|
| Grade I: Shoulders at the same level; deformity invisible when patient is dressed | Grade I: Between the transverse processes of T2 and T4 |
| Grade II: Shoulders at the same level; deformity visible when patient is dressed (lump at the base of the neck) | Grade II: Between the transverse processes of C5 and T2 |
| Grade III: Shoulders asymmetric: the affected shoulder is raised by 2 to 5 cm | Grade III: Above the transverse process of C5 |
| Grade IV: The affected shoulder is raised by more than 5 cm (superior angle close to the occiput) |
Clinical Cases
We'll take a look at four clinical cases of Sprengel's deformity and how imaging techniques played a crucial role in confirming and managing the condition.
Sprengel's Deformity: A Rare But Treatable Congenital Shoulder Anomaly
Sprengel's Deformity is a congenital condition characterized by a small and undescended scapula often associated with scapular winging and hypoplasia. Learn about its diagnosis, treatment options, and outcomes, as well as associated conditions and epidemiology.
Case 1
A 3-year-old girl was found to have a high right shoulder and scoliosis. An ultrasound examination confirmed the presence of a cartilaginous omovertebral structure. Physiotherapy was used to treat her scoliosis.
Case 2
A 2.5-year-old boy had an unsightly shoulder asymmetry and limited mobility of his left arm. X-rays showed a raised left scapula and an omovertebral bone, which was confirmed by a CT scan. Physiotherapy was used to improve the mobility of his left shoulder, and surgery was scheduled later for aesthetic reasons.
Case 3
An 8-month-old boy with left renal agenesis and anomalies in thoraco-abdominal systemic venous return was found to have a high left scapula and an omovertebral bone. The CT scan showed incomplete ossification of the structure and a defect in the closure of the posterior vertebral arches.
Case 4
A 1-year-old girl with congenital torticollis had a raised left scapula. X-rays and a CT scan showed an omovertebral bone and multilevel closure defects of the posterior arches of the cervical vertebrae.
Treatment
Surgical management of Sprengel's deformity requires prior removal of the omovertebral bone or its fibrous/cartilaginous equivalent. There are various surgical techniques available, but results may vary.
Conclusion
Sprengel's deformity is a rare congenital condition that causes a high scapula. Imaging techniques such as ultrasound and CT scans play a crucial role in confirming the diagnosis and planning the surgical procedure. Early detection and management can help improve the functioning of the upper limb and prevent complications later in life.
Exploring Sprengel's Deformity: Anatomy, Pathophysiology, and Associated Diseases
Sprengel's Deformity is a rare shoulder anomaly that affects the scapula and is often associated with other conditions like Klippel-Feil and congenital scoliosis. Dive deep into the anatomy, etiology, and pathophysiology of this congenital condition, as well as its associated diseases.