Sprengel’s Deformity ICD-9
- 755.52 (congenital elevation of the scapula; Sprengel's deformity)
Sprengel’s Deformity Etiology / Epidemiology / Natural History
- Failure of scapular descent during embryologic development.
- Right = left
- @ 20% bilateral
Sprengel’s Deformity Anatomy
- scapula lies at level of C4-5 at 5th week of gestation and migrates caudally below T3 by 12 wks
- failure of descent results in high, small and wide scapula that is malrotated and limits scapulthoracic motion most notable in abduction(90-100 degrees)
- 50% of pts have a fibrous band, cartilaginous bar, or omovertebral bone extending from vertebral border of superior angle of scapula to a posterior element of 4th-7th cervical vertebrae
Sprengel’s Deformity Clinical Evaluation
- Hypoplastic, high riding scapula +/- decreased scapulothoracic motion.
- may cause neck pain or cosmetic concerns
Sprengel’s Deformity Xray / Diagnositc Tests
Sprengel’s Deformity Classification / Treatment
- Cavendish Classification (Cavendish ME, JBJS 1092;54Br:395).
- Grade 1: glenohumeral joints lefel, no deformity visible with patient is dressed.
Treatment: none generally needed.
- Grade 2: gelnohumeral joints leel, prominence in the neck visible when dressed.
Treatment: consider resection of scapular prominence and omovertebral bar mainly for cosmesis.
- Grade 3: shoulder elevation 2-5cm above contralateral side
Treatment: resection of supierior margin of the scapula and omovertebral bar +/- derotation and caudal repositioning of the scapular.
- Grade 4: scapula lies at level of occiput
Treatment: resection of supierior margin of the scapula and omovertebral bar with derotation and caudal repositioning of the scapular.
- Modified Green procedure=excision of supraspinous portion of scapula and omovertral bone vs relocation of scapula. Average 75 degree improvement in abduction (Bellemans M, JPO 8:194;1999).
- Woodward procedure: detachment and relocation of the parascdapular musculature.
- Vertical osteotomy (McMurtry I, JBJS 2005;87Br:986).
- Older children may require claivicular osteotomy to prevent iatrogenic nerve injury
Sprengel’s Deformity Associated Deformities
- Klippel-Feil syndrome
- Facial asymmentry
- Rib synostosis
- Clavicular abnormalities
- Renal anomalies
- Pulmonary disorders
- Shoulder musculature hypoplasia
Sprengel’s Deformity Complications
Sprengel’s Deformity Follow-up Care
Sprengel’s Deformity Review References