Sprengel's deformity

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Sprengel's deformity
Synonyms	Congenital high scapula
Pronounce	N/A
Specialty	Orthopedic surgery
Symptoms	Elevated shoulder, restricted range of motion
Complications	Scoliosis, shoulder asymmetry
Onset	Congenital
Duration	Lifelong
Types	N/A
Causes	Congenital malformation
Risks
Diagnosis	Physical examination, X-ray, MRI
Differential diagnosis	Klippel-Feil syndrome, Poland syndrome
Prevention	None
Treatment	Surgical correction, physical therapy
Medication
Prognosis	Generally good with treatment
Frequency	Rare
Deaths

Klippel–Feil syndrome and Sprengel's deformity

Autosomal dominant inheritance pattern

Sprengel's Deformity is a rare, congenital condition characterized by the elevation of the scapula in the body. It is the most common congenital deformity of the shoulder girdle.

Etiology

The exact cause of Sprengel's Deformity is unknown. However, it is believed to occur due to a disruption in the normal embryological development of the shoulder girdle. This disruption is thought to occur between the fourth and seventh weeks of gestation.

Clinical Presentation

Patients with Sprengel's Deformity typically present with a visible deformity of the shoulder. This may include an elevated shoulder, a decreased range of motion, and a cosmetic deformity due to the prominence of the scapula. In some cases, patients may also present with neck stiffness and pain.

Diagnosis

The diagnosis of Sprengel's Deformity is typically made based on the clinical presentation and confirmed with imaging studies. These may include X-rays, CT scans, and MRI scans.

Treatment

The treatment of Sprengel's Deformity is primarily surgical. The goal of surgery is to improve the cosmetic appearance of the shoulder and to increase the range of motion. Physical therapy may also be beneficial in improving function and mobility.

Prognosis

The prognosis for patients with Sprengel's Deformity is generally good. With appropriate treatment, most patients can achieve a good cosmetic result and improved function. See also:

• Congenital disorders
• Orthopedic disorders
• Pediatric disorders

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