You are in: eMedicine Specialties > Orthopedic Surgery > HAND AND UPPER EXTREMITY Sprengel DeformityArticle Last Updated: Jan 16, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Mihir M Thacker, MBBS, MS(Orth), DNB(Orth), FCPS(Orth), D'Ortho, Assistant Professor of Orthopedic Surgery and Pediatrics, Thomas Jefferson University; Consulting Staff, Department of Pediatric Orthopedic Surgery, Alfred I duPont Hospital for Children; Orthopedic Oncologist, Helen F Graham Cancer Center and Christiana Care Health Services Mihir M Thacker is a member of the following medical societies: Limb Lengthening and Reconstruction Society ASAMI-North America, Medical Council of India, and Musculoskeletal Tumor Society Coauthor(s): David Feldman, MD, Assistant Professor, Department of Orthopaedic Surgery, New York University Medical Center Editors: Michael S Clarke, MD, Clinical Associate Professor, Department of Orthopedic Surgery, University of Missouri-Columbia School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Robert J Nowinski, DO, Clinical Assistant Professor of Orthopaedic Surgery, Ohio University College of Osteopathic Medicine; Private Practice, Orthopedic Specialists and Sports Medicine, Newark, Ohio; Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital; Harris Gellman, MD, Consulting Surgeon, Broward Hand Center, Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine Author and Editor Disclosure Synonyms and related keywords: Sprengel's deformity, Sprengel anomaly, Sprengel's anomaly, congenital high scapula, congenital elevation of the scapula, failure of scapular descent, Sprengel's shoulder, Sprengel shoulder, hochgradige dislocation der scapula, high-grade dislocation of the scapula, hypoplastic scapula, elevated scapula, absent ribs, fused ribs, chest wall asymmetry, Klippel-Feil syndrome, cervical ribs, congenital scoliosis, cervical spina bifida, diastematomyelia, omovertebral bone, high shoulder, shoulder deformity, Greig syndrome, Greig cephalopolysyndactyly syndrome, GCPS, Poland syndrome, Poland anomaly, VATER association, velocardiofacial / velo-cardio-facial syndrome, VCFS, DiGeorge syndrome, floating harbor syndrome, floating-harbor syndrome, Goldenhar syndrome, oculo-auriculo-vertebral / oculoauriculovertebral syndrome, OAV syndrome, X-linked dominant hydrocephalus, skeletal anomalies, mental disturbance syndrome INTRODUCTIONSection 2 of 12
  Eulenberg first described what later became known as Sprengel deformity in 1863,1 and, 2 decades later, Willet and Walsham reported 2 cases with anatomic descriptions of this clinical entity.2 Sprengel then described 4 cases of upward displacement of the scapula in 1891.3 Multiple case reports and surgical techniques followed in the literature for Sprengel deformity, also known as congenital elevation of the scapula. History of the ProcedureIn 1863, Eulenberg described Sprengel deformity as "hochgradige dislocation der scapula" (ie, a high-grade dislocation of the scapula).1 In 1883, Willet and Walsham were the first to describe the omovertebral bone, as well as the first to describe the excision of the bone, with good results.2 Sprengel described 4 cases of the condition in 1891.3 Kolliker, who also described 4 cases in 1891, gave the condition its eponym, Sprengel deformity.4 ProblemSprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula.5, 6, 7, 8 This condition also involves regional muscle hypoplasia or atrophy, which causes disfigurement and limitation of shoulder movement. FrequencySprengel deformity is the most common congenital malformation of the shoulder girdle.9 The male-to-female ratio is 3:1. EtiologyGenetics The condition is sporadic. Rarely, it may run in families (autosomal dominant pattern of inheritance).10, 11 Embryology The scapula is a cervical appendage that normally differentiates opposite the fourth, fifth, and sixth cervical vertebrae at about 5 weeks' gestation.12 This structure normally descends to the thorax by the end of the third month of intrauterine life; any impediment to its descent results in a hypoplastic, elevated scapula, known as the Sprengel deformity. Congenital elevation of the scapula is caused by an interruption in the normal caudad migration of the scapula. This produces both cosmetic and functional impairment and probably occurs between the 9th and 12th week of gestation. An arrest in the development of bone, cartilage, and muscle also occurs. The trapezius, rhomboid, or levator scapulae muscle may be absent, hypoplastic, or contain multiple fibrous adhesions. The serratus anterior muscle may be weak, leading to winging of the scapula. Other muscles, such as the pectoralis major, latissimus dorsi, or the sternocleidomastoid, may be hypoplastic and similarly involved. Associated malformations are almost always present with a Sprengel deformity. These can include anomalies in the cervicothoracic vertebrae or the thoracic rib cage. The most common anomalies are absent or fused ribs, chest-wall asymmetry, Klippel-Feil syndrome, cervical ribs, congenital scoliosis, and cervical spina bifida. When scoliosis is present, the most common curves are in the cervicothoracic or upper thoracic region. A relationship between a Sprengel deformity and diastematomyelia has also been shown. Another anomaly that is seen in approximately one third of patients with a Sprengel deformity is the omovertebral bone. This is a rhomboid- or trapezoid-shaped structure of cartilage or bone that usually lies in a strong fascial sheath, which extends from the superomedial border of the scapula to the spinous processes, lamina, or transverse processes of the cervical spine, most commonly the fourth to seventh cervical vertebrae. A well-developed joint can form between the scapula and the omovertebral bone; this bone can also be a solid osseous bridge. The omovertebral bone is best visualized on a lateral or oblique radiograph of the cervical spine. PathophysiologyDespite the works of Engel (Bleb theory),13 Oxnard,14 and Ogden et al,7, 15 no satisfactory explanation exists regarding the pathogenesis of the Sprengel deformity. The gross pathology can be described as follows:
ClinicalThe hallmarks of the Sprengel deformity are shoulder asymmetry and restriction of shoulder abduction. Clinically, the affected scapula is usually elevated 2-10 cm and is adducted, and its inferior pole is medially rotated. Due to this rotation, the glenoid faces inferiorly. A prominence in the suprascapular region is characteristic due to the upwardly rotated superomedial angle of the scapula, which causes the ipsilateral side of the neck to appear fuller and its normal contour to be lost. The scapula is hypoplastic, and the length of the vertebral border is decreased. Occasionally, some anterior bending of the supraspinous portion is present. Passive movement of the glenohumeral joint, including abduction and external and internal rotation, may be normal. However, scapulothoracic movements may be severely limited. In 40% of patients with a Sprengel deformity, combined abduction is limited to less than 100º. The omovertebral bone may also limit abduction by affecting scapular mobility and can also limit neck movement if this bone is attached high in the cervical spine. Other causes of limited abduction include abnormal and weakened scapular muscles. The left side is more commonly affected than the right side. The condition may sometimes be bilateral, in which case, although it is cosmetically much more acceptable, functionally, it is more disabling. Problems that may be associated with this condition include syndromes such as the following:
These syndromes are extremely rare, with the possible exception of the Klippel-Feil syndrome. Based on the severity of the condition, a Sprengel deformity can be classified as follows (Cavendish grades)8:
This classification, however, is difficult to apply in bilateral cases (see Image 1). INDICATIONSSection 3 of 12
Indications for surgical intervention of a Sprengel deformity include significant cosmetic concerns and significant restriction of shoulder abduction in children younger than age 6 years. RELEVANT ANATOMYSection 4 of 12
Some vital structures are at risk during the extensive dissection that is required as part of the relocation procedure. These structures include the following29:
CONTRAINDICATIONSSection 5 of 12
The presence of a mild deformity with minimal restriction of movement is a contraindication to surgery. Although treatment of the Sprengel deformity is essentially surgical, some factors exist that could compromise the results of surgery and, thus, may be considered to be contraindications. These factors include the presence of associated syndromes that affect the final functional outcome. WORKUPSection 6 of 12
Imaging Studies
TREATMENTSection 7 of 12
Medical therapyNonoperative 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. Surgical therapyMany 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 surgery be performed when patients are younger than age 8 years in order to obtain the best surgical result. Surgical options include subperiosteal resection of part of the scapula, extraperiosteal release, transplantation of the muscular origins of the scapula, excision of the superomedial portion of the scapula, and vertical scapular osteotomy.31, 32, 33, 34 Clavicle resection and excision of the omovertebral bone have also been described. Many of these procedures leave unsightly scars; therefore, the cosmetic improvement needs to be carefully considered. The ability to increase shoulder abduction with surgery is also limited. Preoperative detailsIt is extremely important to explain the expected outcome of the surgery to the patient's parents and to ensure that they have realistic expectations of the surgery. Parents must be told that while 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, a CT scan or magnetic resonance image (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. Intraoperative detailsAlthough multiple surgical procedures are described in the literature, the Green and the Woodward procedures remain the criterion standards for correction of the Sprengel deformity. These 2 surgical procedures are described below. Modified Green scapuloplasty The modified Green scapuloplasty procedure is usually performed for a moderate or severe deformity. The patient is placed in the supine position on a radiolucent table. A supraclavicular incision is made 2 cm cephalad to the mid portion 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 carefully protected. 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 muscle. Dissection then proceeds laterally to expose the spine of the scapula. The insertion of the entire trapezius muscle onto the scapular spine is sectioned and tagged. The trapezius is reflected medially. Care must be taken to avoid injury to the spinal accessory nerve. The supraspinatus muscle 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 extraperiosteally dissected, divided, and tagged. Starting medially, the subscapularis muscle 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 muscle are divided extraperiosteally. Blunt dissection is used to create a large pocket in the superior part of the latissimus dorsi muscle. 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. In order 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 muscle to the base of the scapular spine, subscapularis muscle to the vertebral border of the scapula, serratus anterior muscle to the vertebral border, levator scapulae muscles to the superior border, rhomboid muscles to the medial border, trapezius muscle to the scapular spine, and the superior edge of the latissimus dorsi muscle to the inferolateral edge of the trapezius. The wound is then closed in layers. Woodward procedure The Woodward procedure is also performed for a moderate or severe Sprengel deformity. 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 muscle. 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 scapula muscle 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 muscle must be detached from its insertion in the vertebral border of the scapula. The supraspinatus and the subscapularis muscles 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 muscle 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. Postoperative detailsPostoperatively, 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. Follow-upThe 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. COMPLICATIONSSection 8 of 12
Postoperative complications include the following:
OUTCOME AND PROGNOSISSection 9 of 12
Prognostic factors include the following:
FUTURE AND CONTROVERSIESSection 10 of 12
Future 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.35 He reported a significant improvement in function following this procedure. Controversies Surgical correction in older patients (>8 y) 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 2 adults, and they recommended surgery even in older patients.16 MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
Article Last Updated: Jan 16, 2008 | |||||||||||||||||||||
