Sprengel deformity usually happens in girls and may have a genetic base in some instances. It is the result of the shoulder blade on one or both sides being underdeveloped (hypoplastic) and abnormally high, due to failure of descent of the scapular during embryonic development. Sprengel deformity can impair functioning by restricting the movement of the shoulder blade and thus of the affected arm. Other related problems can also be present including the absence of or hypoplastic muscles, spine and neck problems like torticollis, scoliosis and Klippel-Feil syndrome and discrepency of limb length.
X-rays show the severity of Sprengel's deformity. In milder cases no treatment is suggested, but in more severe cases the treatment is surgery.
Surgery is best performed when the child is between 3-8 years, the younger the child the better motion and post-operative correction obtained. The Woodward procedure is given an 80% satisfactory functional and cosmetic result.
The Woodward procedure is described thus:
- "involves resection of omovertebral bone and division of vertebral attachments of trapezius rhomboids, and levator scapula
- Scapular is subsequently rotated and translated caudally
- detached muscle origins are then sutured to more inferior vertebral spinous processes
- 3 weeks of post-operative immobilisation are required
- osteotomy of clavicle may be required to prevent compression of N/V structure against first rib; postoperative improvement in shoulder abduction is maintained, although some loss of scapular translation can occur in first four months postoperatively
- one third of patients will have widening of their surgical scars, which can be cosmetically disturbing."
1. "Treatment of congenital elevation of the scapula. 10 (2-18) year follow-up of 37 cases of Sprengel's deformity". Greitemann B, Rondhuis JJ, Karbowski A. Klinik for Techn. Orthopadie u. Rehabilitation, Westfalische Wilhelms Universitat, Munster, Germany. This article concludes that for cases with impaired function the Woodward procedure is preferred, whilst cosmetic problems were best treated by resection of part of the superior angle.
2. "Surgery of the clavicle in Sprengel's deformity", Chung SM, Farahvar H, Clinic Orthop 1976 May;(116):138-41. This article talks of softening of a clavicle by the morcellization technique of Robinson et al, to improve the degree of correction and to avoid neurovascular complications, combined with the Woodward correction.
3. "Surgical treatment of Sprengel's disease", Skipichenko DN, Klin Khir 1999;(7):28-9. This article talks of the success of scapulolysis according to the Ternovsky-Andrianov method in modification of P. Ya. Fishchenko.
4. "Modified Woodward procedure for Sprengle deformity of the shoulder: long-term results." Borges Jl, Shah A, Torres BC, Bowen JR. Alfred I. DuPont Institute, Wilmington, DE 19899, USA. This article talks of a "modified by excision of the prominent superomedial border of the scapula". The results of the study are believed to support the premise that a modified Woodward procedure is not altered by growth and is maintained beyond skeletal maturity.
5. "Sprengel's deformity", Matejicek M, Dungl P, Slavik M, Karpisek M Acta Chir Orthop Traumatol Cech 1990 Feb;57(1):3-14. Ortopedicka Klinika ILF, Fakultni nemocnice na Bulovce, Praha. This article recommends the Green procedure with morcellation of the ipsilateral clavicle. A group of 7 patients operated on at the Orthopaedic Clinic of the Institute for Further Education of Physicians, Teaching Hospital at Bulovka in Prague in the period of 1980 - 1987. Results were satisfactory.
6. "Sprengel Deformity", Leibovic SJ, Ehrlich MG, Zaleske DJ Pediatric Orthopaedic Unit, Massachusetts General Hospital, Boston 02114. This article talks of a modification of Green's procedure "all muscular attachments to the scapula are freed, the omovertebral band is cut, and the scapula is sutured into a pocket in the latissimus dorsi after the scapula has been rotated and moved caudad to a more normal position. No spring or wire traction is employed". Results are deemed to be satisfactory. In 11 of 15 patients there was moderate or dramatic improvement in appearance postoperatively. Movement of the affected shoulder averaged from a preoperative 91 degrees to a postoperative 148 degrees. After 2 years the original malrotation of the scapular recurred, but was not reported to have compromised the large gain in movement postoperatively.
7. "Results of surgical treatment of Sprengel deformity by a modified Green's procedure". Bellemans M, Lamoureux J Department of Orthopaedics, Children University Hospital, Queen Fabiola, Brussels, Belgium. This article cites 7 cases where a modified Green procedure was used, "without dissection of the serratus anterio muscle and immediate postoperative mobilization." The postoperative gain in movement of 77 degrees is seen as favourable in regard to current literature.
Contact A Family
Contact A Family gives names of support groups
National Organization for Rare Disorders, Inc. (NORD)
P.O box 8923
New Fairfield, CT
Tel: (001) 203 746 6518
International Center for Skeletal Dysplasia
Saint Joseph's Hospital
7601 Osler Drive
Tel: (001) 410 337 1250
National Institute of Arthritis and Musculoskeletal and Skin Diseases
National Institutes of Health
1 AMS Circle Bethesda Maryland 20892-3675
Tel: (001) 301 495 4484
- The Orthoseek website
- Wheelers Textbook of Orthopaedics