| Sprengel Deformity
Definition 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."
Research 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.
Organisations
Contact A Family
209-211
City Road
London
EC1V 1JN
Contact A Family gives names of support groups
National Organization for Rare Disorders, Inc. (NORD)
P.O box 8923
New Fairfield, CT
06812-8923
Tel: (001) 203 746 6518
http://www.rarediseases.org/cgi-bin/nord
International Center for Skeletal Dysplasia
Saint Joseph's Hospital
7601 Osler Drive
Towson, MD
21204
Tel: (001) 410 337 1250
http://www.csmc.edu/genetics/skeldys/default.html
National Institute of Arthritis and Musculoskeletal and Skin Diseases
National Institutes of Health
Information Clearinghouse
1 AMS Circle Bethesda Maryland 20892-3675
Tel: (001) 301 495 4484
http://www.nih.gov/niams
Websites - The
Orthoseek website
- Wheelers Textbook
of Orthopaedics
<<
Back |
|
|
