Sprengel Deformity
What is Sprengel Deformity?
Sprengel Deformity is a condition characterized by the scapula (shoulder blade) being higher than its normal position, causing limited shoulder movement and cosmetic concerns. It emerges during fetal development in the womb.
During weeks 5-8 of pregnancy, the scapula and surrounding muscles develop at the level of the neck, migrating towards the shoulder area over time. Issues causing Sprengel Deformity during pregnancy might affect the cardiovascular and urinary systems, requiring evaluation by a pediatric cardiologist and urologist.
What Causes Sprengel Deformity?
The exact cause of Sprengel Deformity is unknown, but it is believed to result from the interaction of genetic and environmental factors.
What Are the Symptoms of Sprengel Deformity?
Sprengel Deformity is usually noticeable from birth. Key symptoms include the elevated and posterior positioning of the shoulder, restricted shoulder movements, abnormal posture, and a prominent scapula. Diagnosis involves clinical examination, imaging tests, and specialist evaluation.
The affected scapula appears higher and sometimes behind the other, limiting shoulder movements. In severe cases, raising the arm fully might be challenging, with an attempt to lift the arm causing the body to lean sideways. In cases where both scapulae are affected, the shoulders might appear equal, making detection more challenging. However, difficulty raising both arms above the head and a short-neck appearance can still be indicative.
How is Sprengel Deformity Diagnosed?
Diagnosis is made through examination by a specialist and X-rays. Computed tomography (CT) and magnetic resonance imaging (MRI) can be used to investigate omovertebra and other associated congenital abnormalities.
What is Omovertebra?
It is an additional structure, sometimes partial, sometimes absent, or replaced by a rigid tissue, affecting the muscles around the scapula. This tissue can be cartilaginous or bony. Omovertebra, often present in cases of Sprengel Deformity, restricts the necessary rotation movement between the scapula and the spine, contributing to limited shoulder movement.
What Are the Treatment Options?
If there is significant movement limitation or pronounced shoulder imbalance, surgical treatment is often the only option. Surgery aims to correct the scapula and improve shoulder movements. The postoperative rehabilitation process, including specific exercises, is crucial for maximizing the benefits of surgery.
Outcomes and Prognosis
Surgery is not always necessary for Sprengel Deformity. In cases where surgery is needed, collaboration among an experienced surgical team can lead to both cosmetic improvement and enhanced shoulder movements for most patients. However, the extent of improvement and outcomes depend on the individual's condition and how much the scapula and surrounding muscles were affected during fetal development.
This article provides general information. For personalized details about your situation and to evaluate treatment options, consult with an experienced orthopedic surgeon.