Sprengel Deformity Treatment & Management
- Author: Mihir M Thacker, MBBS, MS(Orth), DNB(Orth), FCPS(Orth), D'Ortho; Chief Editor: Harris Gellman, MD more...
Nonoperative treatment for the Sprengel deformity consists of physical therapy. Exercises are used to maintain an individual's range of motion and to strengthen the weak periscapular muscles.
Many patients with the Sprengel deformity do not require operative intervention. For those who do require surgery, the aims in the correction of this condition are twofold. First, the cause of the scapular binding must be released. Second, the scapula must be relocated.
The main objectives in performing surgery are to improve the cosmetic appearance and contour of the neck and to improve the scapular function when it is severely impaired. The optimal age for operative intervention is controversial; however, most authors would recommend that surgery be performed when patients are younger than age 8 years in order to obtain the best surgical result.
Surgical options include the following[34, 35, 36, 37] :
Subperiosteal resection of part of the scapula
Transplantation of the muscular origins of the scapula
Excision of the superomedial portion of the scapula
Vertical scapular osteotomy
Clavicle resection and excision of the omovertebral bone have also been described. Many of these procedures leave unsightly scars; therefore, the cosmetic improvement must be considered carefully. The ability to increase shoulder abduction with surgery is also limited.
It is extremely important to explain the expected outcome of the surgery to the patient's parents and to ensure that they have realistic expectations for surgical treatment. Parents must be told that whereas cosmesis may be improved, the improvement in the patient's range of motion may be limited.
Before surgery, certain factors should be considered, including the cosmetic severity, the functional impairment, the age of the patient, other congenital anomalies, and the medical fitness of the patient to undergo the surgery. These factors are important because they ultimately determine the postoperative outcome.
Preoperatively, radiographs of both shoulders, including the cervical and thoracic spine, should be obtained to determine the presence of congenital scoliosis, Klippel-Feil syndrome, or an omovertebral bone. Furthermore, computed tomography (CT) or magnetic resonance imaging (MRI) may be useful to delineate the attachments of the omovertebral bone or to determine the presence of spina bifida occulta or an intraspinous lesion.
Although multiple surgical procedures are described in the literature, the Green scapuloplasty and the Woodward procedure remain the criterion standards for correction of the Sprengel deformity.
Modified Green scapuloplasty
The modified Green scapuloplasty procedure usually is performed for a moderate or severe deformity.[38, 39]
The patient is placed in the supine position on a radiolucent table. A supraclavicular incision is made 2 cm cephalad to the midportion of the clavicle, in line with the skin creases. The deep fascia is incised, and the periosteum of the clavicle is divided longitudinally. The underlying subclavian vessels and brachial plexus must be protected carefully. The anterior cortex of the middle third of the clavicle is sectioned with an oscillating saw. Gentle force is then used to produce a greenstick fracture. The periosteum is then closed.
Next, the patient is turned to the prone position. A midline incision is made from C4 to T10. A plane is developed between the subcutaneous tissues and the fascia underlying the trapezius. Dissection proceeds laterally to expose the spine of the scapula. The insertion of the entire trapezius onto the scapular spine is sectioned and tagged. The trapezius is reflected medially. Care must be taken not to injure the spinal accessory nerve. The supraspinatus is then detached extraperiosteally to the greater scapular notch. The transverse scapular artery and the suprascapular neurovascular bundle must be protected.
The omovertebral bar is then excised. The scapular attachment is sectioned first. The omovertebral bar is then gently detached from its insertion to the cervical spine. The insertions of the levator scapulae and rhomboid muscles are dissected extraperiosteally, divided, and tagged. Starting medially, the subscapularis is elevated extraperiosteally. The suprascapular neurovascular bundle is protected, and the supraspinous portion of the scapula, along with its periosteum, is excised.
The scapular attachments of the latissimus dorsi are divided extraperiosteally. Blunt dissection is used to create a large pocket in the superior part of the latissimus dorsi. Fibrous bands may connect the scapula to the chest wall; these should be divided in order to mobilize the scapula. The scapula is then displaced distally. To prevent migration, the inferior pole of the scapula is fixed to the adjacent ribs. If winging is present, the scapula can be fixed to the rib cage in a lower and more laterally rotated position.
The muscles are reattached in the following order:
Supraspinatus to the base of the scapular spine
Subscapularis to the vertebral border of the scapula
Serratus anterior to the vertebral border
Levator scapulae muscles to the superior border
Rhomboid muscles to the medial border
Trapezius to the scapular spine
Superior edge of the latissimus dorsi to the inferolateral edge of the trapezius
The wound is then closed in layers.
The Woodward procedure is also performed for a moderate or severe Sprengel deformity.[40, 41]
The patient is placed in the prone position. A midline incision is made from C1 to T9. The wound is dissected laterally to the medial border of the scapula. The lateral border of the trapezius is identified. The lower portion of the trapezius is dissected from the latissimus dorsi . The origin of the trapezius is detached from the scapular spine, and the edges are tagged. The origins of the rhomboid muscles are divided and tagged. The entire muscle sheet is retracted laterally, exposing the omovertebral bar, which is excised extraperiosteally.
The levator scapulae is sectioned at its attachment to the scapula. Fibrous bands may attach the scapula to the chest wall; these should be sectioned. The serratus anterior must be detached from its insertion in the vertebral border of the scapula. The supraspinatus and the subscapularis are elevated extraperiosteally.
The supraspinous portion of the scapula is resected with its periosteum. Care is taken to avoid injury to the suprascapular nerve and vessels. The scapula is then lowered to the desired position. The subscapularis is reattached to the vertebral border, and the supraspinatus is sutured to the scapular spine. The serratus anterior is reattached to the vertebral border, and the latissimus dorsi is reattached to the scapula. The trapezius and the rhomboid muscles are then resutured to the spinous processes at a more distal level.
The wound is then closed in layers.
Postoperatively, the arm is supported with a sling (for 3 weeks), and gentle range of motion (active and passive) and strengthening exercises are gradually started. Physical therapy is continued for up to 6 months.
The patient is seen monthly for the first 3 months, every 3 months subsequently for the first year, and yearly thereafter. Scapular appearance, function, and motion, and general patient satisfaction are assessed at each visit. The appearance of the surgical scar, scapular symmetry, presence and degree of winging, scapular range of motion, muscle bulk, and strength are measured. Radiographs and clinical pictures are obtained for comparison.
Postoperative complications include the following:
Outcome and Prognosis
Prognostic factors include the following:
Severity of the deformity
Age at surgery - Generally, results of surgery in children older than age 6 years are not as good
Type of procedure - Relocation surgeries have better functional outcomes
Associated anomalies - Anomalies such as Klippel-Feil syndrome compromise the eventual result
Future and Controversies
In a report by Mears, the author described a novel approach that included an oblique plane osteotomy of the scapular body, along with release of the long head of triceps from the scapula. He reported a significant improvement in function following this procedure.
Surgical correction in older patients (>8 years) is controversial, and the results of surgery are not as good. However, in a study by Doita et al, the authors had good results after surgical correction in two adults, and they recommended surgery even in older patients.
Hamner DL, Hall JE. Sprengel's deformity associated with multidirectional shoulder instability. J Pediatr Orthop. 1995 Sep-Oct. 15(5):641-3. [Medline].
Tachdjian MO. Pediatric Orthopedics. 2nd ed. Philadelphia, Pa: WB Saunders; 1990. Vol 1: 136-8.
Ogden JA, Conlogue GJ, Phillips MS, Bronson ML. Sprengel's deformity. Radiology of the pathologic deformation. Skeletal Radiol. 1979. 4(4):204-11. [Medline].
Mooney JF 3rd, White DR, Glazier S. Previously unreported structure associated with Sprengel deformity. J Pediatr Orthop. 2009 Jan-Feb. 29(1):26-8. [Medline].
Eulenberg M. Casuistische mittelheilungen aus dem gembeite der orthopadie. Arch Klin Chir. 1863. 4:301-11.
Willet A, Walsham WJ. A second case of malformation of the left shoulder-girdle; removal of the abnormal portion of bone; with remarks on the probable nature of the deformity. Med Chir Trans. 66. 1883:145-58. [Full Text].
Sprengel OK. Die angeborene verschiebung des schulterblattes nach oben. Archiv Fur Klinische Chirurgie, Berlin. 1891. 42:545-9.
Kolliker T. Mittheilungen aus der chirurgischen casuistik und kleinere mittheilungen. Bemerkungen zum aufsatze von Dr. Sprengel. Die angeborene verschiebung des schulterblattes nach oben. Arch Klin Chir. 1891. 42:925.
Chen CP. Syndromes and disorders associated with omphalocele (III): single gene disorders, neural tube defects, diaphragmatic defects and others. Taiwan J Obstet Gynecol. 2007 Jun. 46(2):111-20. [Medline]. [Full Text].
Ferlini A, Ragno M, Gobbi P, et al. Hydrocephalus, skeletal anomalies, and mental disturbances in a mother and three daughters: a new syndrome. Am J Med Genet. 1995 Dec 4. 59(4):506-11. [Medline].
Engel D. The etiology of the undescended scapula and related syndromes. J Bone Joint Surg. 1943. 25:613-25. [Full Text].
Oxnard CE. Evolution of the human shoulder: some possible pathways. Am J Phys Anthropol. 1969 May. 30(3):319-31. [Medline].
Ogden JA, Phillips SB. Radiology of postnatal skeletal development. VII. The scapula. Skeletal Radiol. 1983. 9(3):157-69. [Medline].
Doita M, Iio H, Mizuno K. Surgical management of Sprengel's deformity in adults. A report of two cases. Clin Orthop Relat Res. 2000 Feb. 371:119-24. [Medline].
Floemer F, Magerkurth O, Jauckus C, Lütschg J, Schneider JF. Klippel-Feil syndrome and Sprengel deformity combined with an intraspinal course of the left subclavian artery and a bovine aortic arch variant. AJNR Am J Neuroradiol. 2007 Nov 16. epub ahead of print. [Medline].
Samartzis D, Herman J, Lubicky JP, Shen FH. Sprengel's deformity in Klippel-Feil syndrome. Spine. 2007 Aug 15. 32(18):E512-6. [Medline].
Hensinger RN. Orthopedic problems of the shoulder and neck. Pediatr Clin North Am. 1977 Nov. 24(4):889-902. [Medline].
Keats TE. Ocular hypertelorism (Greig's syndrome) associated with Sprengel's deformity. Am J Roentgenol Radium Ther Nucl Med. 1970 Sep. 110(1):119-22. [Medline].
Hadley MD. Carpal coalition and Sprengel's shoulder in Poland's syndrome. J Hand Surg [Br]. 1985 Jun. 10(2):253-5. [Medline].
Fernbach SK, Glass RB. The expanded spectrum of limb anomalies in the VATER association. Pediatr Radiol. 1988. 18(3):215-20. [Medline].
Pollard ME, Cushing MV, Ogden JA. Musculoskeletal abnormalities in velocardiofacial syndrome. J Pediatr Orthop. 1999 Sep-Oct. 19(5):607-12. [Medline].
Hersh JH, Groom KR, Yen FF, Verdi GD. Changing phenotype in floating-harbor syndrome. Am J Med Genet. 1998 Feb 26. 76(1):58-61. [Medline].
Avon SW, Shively JL. Orthopaedic manifestations of Goldenhar syndrome. J Pediatr Orthop. 1988 Nov-Dec. 8(6):683-6. [Medline].
Boon JM, Potgieter D, Van Jaarsveld Z, Frantzen DJ. Congenital undescended scapula (Sprengel deformity): a case study. Clin Anat. 2002 Mar. 15(2):139-42. [Medline].
Yamada K, Suenaga N, Iwasaki N, Oizumi N, Minami A, Funakoshi T. Correction in malrotation of the scapula and muscle transfer for the management of severe Sprengel deformity: static and dynamic evaluation using 3-dimensional computed tomography. J Pediatr Orthop. 2013 Mar. 33(2):205-11. [Medline].
McMurtry I, Bennet GC, Bradish C. Osteotomy for congenital elevation of the scapula (Sprengel's deformity). J Bone Joint Surg Br. 2005 Jul. 87(7):986-9. [Medline].
Ross DM, Cruess RL. The surgical correction of congenital elevation of the scapula. A review of seventy-seven cases. Clin Orthop Relat Res. 1977 Jun. 125:17-23. [Medline].
Woodward JW. Congenital elevation of the scapula: correction by release and transplantation of muscle origins. J Bone Joint Surg Am. 1961. 43:219-28. [Full Text].
Green WT. The surgical correction of congenital elevation of the scapula (Sprengel's deformity). J Bone Joint Surg Am. 1957. 39-A:1439-48.
Wada A, Nakamura T, Fujii T, Takamura K, Yanagida H, Yamaguchi T, et al. Sprengel deformity: morphometric assessment and surgical treatment by the modified green procedure. J Pediatr Orthop. 2014 Jan. 34(1):55-62. [Medline].
Siu KK, Ko JY, Huang CC, Wang FS, Chen JM, Wong T. Woodward procedure improves shoulder function in Sprengel deformity. Chang Gung Med J. 2011 Jul-Aug. 34(4):403-9. [Medline].
Walstra FE, Alta TD, van der Eijken JW, Willems WJ, Ham SJ. Long-term follow-up of Sprengel's deformity treated with the Woodward procedure. J Shoulder Elbow Surg. 2013 Jun. 22(6):752-9. [Medline].
Mears DC. Partial resection of the scapula and a release of the long head of triceps for the management of Sprengel's deformity. J Pediatr Orthop. 2001 Mar-Apr. 21(2):242-5. [Medline].