MICHAEL T. LEGEYT, M.D. Orthopaedic Resident

KIRK DABNEY, M.D. Attending Pediatric Orthopaedic Surgeon

July 17, 1996






HISTORY: This is a 5 + 3 year old white female the product of a full-term normal spontaneous vaginal delivery with a diagnosis of CHARGE syndrome (defects of the eyes, ears, heart, choanal atresia, mental retardation, and genital hypoplasia) who presented for evaluation of a webbed neck left side worse than right. Pt has developmental delay and began walking at 4 years of age. She had undergone previous surgery for strabismus and atrial septal defect.

PHYSICAL EXAM: General - Small for chronological age

XRAYS: Demonstrate elevated left scapula worse than right, No evidence of omovertebral bone on either side, Thoracic Kyphosis - no other spinal abnormalities

TREATMENT: Modified Woodward Procedure with clavicular osteotomy and morcellation on left Side.

FOLLOW-UP: Doing well with a healed incision at 3 weeks post op



  1. Cosmesis
  2. Functional
  3. Associated Anomalies
  4. Age of Patient
Literature Review
Etiology (Horowitz):

Sprengel's Deformity is a failure of descent of the scapula caused by:

  1. too great an intrauterine pressure
  2. Abnormal articulations of scapula with the spine (omovertebral bone)
  3. defective musculature of the scapulothoracic region
  4. arrest of development due to ineffective muscular tension

The scapula appears at the 5th week of gestation at C5 - T1, then migrates to the adult position by birth to T2 - T7. Some have bony articulations with the spine via omovertebral bones. Others have defective musculature: Trapezius, Rhomboids, Levator Scapula are most common; Pectoralis Major/Minor, Latissimus Dorsi, Sternocleidomastoid, Serratus Anterior less commonly. The affected muscles undergo degeneration, necrosis, fibrosis and secondary contracture. Also the scapula is hypoplasic on the affected side.

Associated Anomalies (70% of Cases): Absence/Fusion of Ribs, Cervical Ribs, Klippel - Feil Syndrome, Congenital Scoliosis with Hemivertebrae, Cervical Spina Bifida, Syringomyelia, Paraplegia, Platybasia, Situs inversus, Mandibulofacial dysostosis, Clavicular Abnormalities, Cardiac Anomalies ASD, VSD, Kidney Malformations, Etc.

Cavendish Classification
Outcome Expectations:

In a recent article from the Alfred I. duPont Institute reviewing the long term results (average follow-up 8 years) of the modified Woodward procedure in 15 patients, there was improved cosmesis with a decrease of at least one Cavendish Grade in all patients, average scapular lowering of 2.7cm, and an average improvement in shoulder abduction by 35 degrees. There were only minor complications noted including one transient brachial plexus palsy, one widened scar, and one scapular winging.

  1. Borges, JLP; et al. Modified Woodward Procedure for Sprengel's Deformity of the Shoulder: Long-Term Results. JPO 16:508-513,1996.
  2. Cavendish, ME. Congenital elevation of the scapula. JBJS 54-B:395,1972.
  3. Eulenberg, M. Casuist ische Mittheilungen aus dem. Begiete der Orthopadie. Arch Klin Chir 4:301,1863.
  4. Eulenberg, M. Beitrag sur Dislocation der scapula amtliche Berichte uber die Versammlungen deutscher Naturforscher und Aerzte fur die Jahre. 37;291,1863.
  5. Horowitz, AE. Congenital elevation of the scapula-Sprengel's deformity. Am J Orthop Surg 6:260,1908.
  6. Kolliker, T. Mittheilungen aus der chirurgischen Bemerkungen zum Aufsatze von Dr. Sprengel. " Die angeborene Verschieburg des Schulterblattes nach oben." Arch Klin Chir 42:925,1891.
  7. Robinson, RA; et al. The surgical importance of the clavicular component of Sprengel's deformity. JBJS 49:1481,1967.
  8. Sprengel, O. Die angeborene Verschieburg des Schulterblattes nach oben. Arch Klin Chir 42:545,1891.
  9. Willet, A and Walsham, WJ. An account of the dissection of the parts removed after death from the body of a woman the subject of congenital malformation of the spinal column, bony thorax, and left scapular arch; with remarks on the probable nature of the defects in development producing the deformities. Med Chir Trans, London 63:256,1880.
  10. Woodward, JW. Congenital elevation of the scapula. Correction by release and transplantation of muscle origins. A preliminary report. JBJS 43:219-228,1961.