The affected abnormal shoulder blade is also abnormally connected to the spine, often restricting movement of the shoulder. In case of severe deformity surgery is done under 6 years of age. Sprengel s shoulder free download as powerpoint presentation. Full text is available as a scanned copy of the original print version.
The condition occurs equally in males and females and. Congenital elevation of the scapula or sprengels shoulder is an anomaly of the shoulder girdle that is associated with abnormal descent, and altered position. An update on the surgical management yasser ali kamal yasser ak. Thank you for using the download pdf file feature, to download a correct pdf file, please follow the steps. May 25, 2012 sprengel deformity is an uncommon condition in which the scapula shoulder blade on one side is higher in the neck than the other side. The affected shoulder blade may also be underdeveloped and smaller than normal and rotated toward. Surgical correction of sprengels deformity by a modified greens procedure with contralateral posterior iliac crest fixation instead of skeletal traction. Surgical treatment of sprengels deformity of the scapula in children. Sprengels deformity of the shoulder is a dysplasia of the pectoral girdle, resulting in cosmetic and functional disability.
Discuss surgical approaches to sprengel s deformity 5. Sprengel deformity is a congenital failure of descent of the scapula. Although the etiology of both spinal dysraphism and sprengels deformity remains unclear, all deformities of the spine, ribs, and shoulder might result from a common genetic defect affecting somitogenesis. Woodward procedure improves shoulder function in sprengel. After conversion, you can see that there are following files listed in output folder. Thank you for using the download pdf file feature, to. Pdf sprengel deformity sd, undescended scapula or high scapula is the most common congenital abnormality of the shoulder girdle. The deformity is associated with other congenital anomalies, which often dictate the management and outcome of treatment. I have a mild case of sprengel s deformity of the right scapula which in and of itself is not curable except through radical surgery. The sprengel deformity was corrected surgically with a woodward procedure. Rotator cuff sprain and strains university of california. Many people with sprengel deformity do not need surgery and may have physical therapy to maintain range of motion and strengthen weak muscles. Aug 04, 2017 klippel feil syndrome kfs is a congenital, musculoskeletal condition characterized by the fusion of at least two vertebrae of the neck. However, it is the most common congenital anomaly of the.
Jun 07, 2016 shoulder range of motion is measured, with particular attention to active forward flexion and abduction. The surgical importance of the clavicular component of sprengel s deformity. Scapular osteotomy for sprengels shoulder congenital. Anatomy of the shoulder joint three bones, the collarbone clavicle, the shoulder blade scapula, and the upper arm bone humerus come together to form the shoulder.
Sprengel s deformity also known as high scapula or congenital high scapula is a rare congenital skeletal abnormality where a person has one shoulder blade that sits higher on the back than the other. Discuss indications for correction of sprengel s deformity 4. Madelungs deformity clinodactyly oligodactyly polydactyly. Dominant transmission of sprengels shoulder and cleft palate. Signs and symptoms may include a lump in the back of the base of the neck and limited movement in the. A sprengel shoulder deformity is a congential elevation of the scapula that results from the failure of caudal migration of the scapula during fetal life. This is a passive technique 0 to use muscle energy, move shoulder to the barrier, have patient actively resist, then move into.
Get a printable copy pdf file of the complete article 669k, or click on a page image below to browse page by page. Recognize the strengths and limitations in cpgs to define best practices that meet the needs of patients under most circumstances but do not replace the need for sound clinical. The purpose of the journal of the belgian society of radiology is the publication of articles dealing with diagnostic and interventional radiology, related imaging techniques, allied sciences, and. Figure 2 shows posterior clinical views of a 4yearold patient with deficient rightsided abduction secondary to sprengels deformity preoperatively and postoperatively. Complications of surgery rarely include winging of scapula and brachial plexus injury. Congenital elevation of the scapula is a rare congenital deformity first described by eulenburg1 in 1863. Pdf klippelfeil syndrome with sprengel deformity and. Sprengel shoulder undescended scapula shoulderdoc by prof. It is the most common congenital shoulder deformity and is sporadic in nature 12. Dont be blind to the radiological eye signs multimodality.
An initial diagnosis can often be made on radiography, but ct or mri is often necessary to evaluate the details of the abnormality. Get a printable copy pdf file of the complete article 2. Impingement syndrome shoulder anatomy to understand impingement syndrome, it is important to know about the anatomy of the shoulder. Thefather ofthe proband wasa twin, but the other twin haddied at birthandwassaidto havebeendeformed. Sprengel s deformity is felt to be an arrest of the caudal descent of the scapula which normally occurs between the ninth and twelfth weeks gestation. Sprengel deformity is an uncommon condition in which the scapula shoulder blade on one side is higher in the neck than the other side.
Learn indepth information on sprengels shoulder, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. Treatment of sprengel deformity depends on the degree of functional impairment andor cosmetic disfigurement. Oct 01, 2018 sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula. Congenital and acquired atrophy of the shoulder girdle.
Shoulder examination introduction shoulder disorders are can be broadly classified into. I have sprengel s deformity scapula elevata, i wasnt diagnosed with it as a child and therefore didnt have surgery. Sprengel deformity nord national organization for rare. Pdf sprengel deformity ie, congenital elevation of the scapula is a rare clinical entity. We treated a young afghan boy who was presented to the german level ii provincial reconstructive team in kunduz, afghanistan, with the complaint of a shoulder deformity evolving after an accident that occurred several years before the presentation of the patient to clinic. No treatment is required in case of mild deformity. Modified woodward procedure for sprengels deformity. This condition also involves regional muscle hypoplasia or atrophy, which causes disfigurement and limitation of shoulder movement. Aetiology scapulae normally complete their decent from c5 in the neck to t5, by the third month of fetal life arrest of decent may occur due to either fibrous or bony tissue, which causes congenital undescended scapula classification grade 1. Sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula.
The deformity commonly occurs sporadically, though in combination with other congenital anomalies, such as congenital scoliosis, fusion of cervical vertebrae, and conditions like klippelfeil syndrome may coexist. Apr 18, 20 sprengel s deformity is a rare and complex congenital deformity of the shoulder girdle. Passive glenohumeral abduction and rotation are typically normal. Scapular osteotomy for sprengel s shoulder congenital elevation of the scapula indications. Although the woodward procedure reportedly affords satisfactory correction, the longterm functional outcome postoperatively has. Sprengel deformity and omovertebral bone international journal. Sprengel shoulder undescended scapula shoulderdoc by. The structure of my shoulder has always caused my muscles some discomfort during exercise but in my younger days i rarely had to quit due to pain. Severity can range considerably from being almost invisible when covered with clothes, to the shoulder being elevated over 5 centimeters, with neck webbing. Untreated sprengel s deformity can lead to shoulder muscle hypoplasia and atrophy, reduced shoulder function, and cosmetic disfigurement. The deformity is due to a failure in early fetal development where the shoulder fails to descend properly from the neck to its final position. Apr 10, 2009 i have a mild case of sprengel s deformity of the right scapula which in and of itself is not curable except through radical surgery. For those who do require surgery, the goals are to release the binding of the scapula and relocate the scapula. This report is about a rare case in a 4 yearold girl who presented with right shoulder deformity and pain associated with right proximal arm swelling.
Discuss the reason for the high incidence of other anomalies accompanying sprengel s deformity, and list 4 of the more common accompanying conditions 3. Sprengels shoulder also called as congenital high scapula characterized by an abnormally raised scapula, on one or both the sides the scapular muscles are poorly developed and may be represented by fibrous bands it may be accompanied by scoliosis with convexity on the involved side. Search and free download all ebooks, handbook, textbook, user guide pdf files on the internet quickly and easily. Sprengels shoulder free download as powerpoint presentation. Jan 05, 2016 sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade scapula. Blue arrows point to elevation and medial angulation of both.
It provides stability and strength for the shoulder joint, anchoring the humerus upper arm bone to the scapula shoulder blade so that movements of the arm can occur in a. Scribd is the worlds largest social reading and publishing site. Sprengels deformity is a relatively rare condition consisting of an elevated, dysplastic scapula that causes cosmetic deformity and restriction of shoulder range of. Thankfully my range of motion is minimally limited compared to severe cases, however i have been experiencing it along with pain, numbness and frustration about the cosmetic aspect of it. In addition to his own research he supervises phd and msc students. Three major features result from this vertebral fusion. Please remove adblock adverts are the main source of revenue for dovemed.
Klippelfeil syndrome is a bone disorder characterized by the abnormal joining fusion of two or more spinal bones in the neck cervical vertebrae. Sprengel shoulder is a rare congenital deformity of one or both scapulae that is usually detected at birth. Sprengels deformity pediatric orthopaedic society of north. The main feature of this very rare congenital malformation of the pectoral girdle is an abnormally high, more or less dysmorphic scapula. We report a series of four cases of congenital high scapula or sprengel s deformity diagnosed and followed in our establishment. Normally this disorder is asymmetric with the left scapula most commonly affected, so it will sit higher on the back than the. Look from the front, side and above asymmetry, scars, deltoid wasting, scj or acj deformity, swelling of the joint from behind look and feel for rotator cuff wasting, scapula shape and situation e. Sprengel deformity sd, undescended scapula or high scapula is the most common congenital abnormality of the shoulder girdle. The purpose of the journal of the belgian society of radiology is the publication of articles dealing with diagnostic and interventional radiology, related imaging techniques, allied sciences, and continuing education. Sprengel s shoulder symptoms, causes, diagnosis, and treatment information for sprengel s shoulder sprengel deformity with alternative diagnoses, fulltext book chapters, misdiagnosis, research treatments, prevention, and prognosis. The rotator cuff is made up of four muscles supraspinatus, infraspinatus, subscapularis, and teres minor and their tendons. Connecting the shoulder and the spine article pdf available in child s nervous system 297 february 20 with 576 reads how we measure reads. Limited shoulder abduction and cosmetic appearance are the major concerns. It maintains the shoulder in a functional position with the axial skeleton and allows varied arm positions in shoulder anatomy.
Limit shoulder abduction and cosmetic ap pearances. Normally this disorder is asymmetric with the left scapula most commonly affected, so it will sit higher on the back than the right. The cup is the head of the humerus arm bone and the saucer is the glenoid socket of the scapula or shoulder blade. Following a wellstructured conditioning program will also help you return to sports and other. Sprengel deformity is a rare congenital anomaly that involves the shoulder joint and scapula. Sprengel deformity usually happens in girls and may have a genetic base in some instances.
The combination of spinal dysraphism and sprengels deformity is rare, and is associated with segmentation defects of the spine and ribs. Congenital and acquired atrophy of the shoulder girdle muscles in a patient with sprengel s deformity james d. Frozen shoulder is a disorder in which the connective tissue surrounding the shoulder becomes inflamed and stiff, restricting motion and causing pain. This syndrome can cause chronic headaches as well as pain in both the neck and the back. Pathophysiology failure of descent of the scapula from its embryonic position in the neck normally completed in the 3rd month of intrauterine life. Sprengel deformity genetic and rare diseases information. Sprengel deformity is a rare congenital disorder in which the shoulder blade scapula is too high on one side of the body. Sprengel s deformity is a rare and complex congenital deformity of the shoulder girdle. Hehad a cleft palate andbilateral sprengel s shoulder, morepronounced.
Collins, md introductions prengels deformity is a congenital anomaly of the shoulder girdle which results in elevation of the scapula congenital high scapula and limitation of movement of the shoulder. Shoulder instability may factitiously increase shoulder motion. Sprengel deformity is a high shoulder blade scapula, due to failure in early foetal development where the shoulder fails to descent properly from the neck to its final position. Normally, during prenatal development, both scapula begin high in the neck and slowly descend toward their normal resting position in the upper back. Jeremy is an associate editor for the journal of orthopaedic and sports physical therapy jospt. Sprengel deformity, or congenital elevation of the scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality. There is resultant shoulder muscle hypoplasiaatrophy leading to reduced shoulder movement. Deformities of the left upper extremity include radioulnar synostosis, a fourrayed hand, and absent thenar musculature. Jun 15, 2019 sprengels deformity is a rare congenital skeletal abnormality where a person has one shoulder sprengels deformity. Shoulder range of motion is measured, with particular attention to active forward flexion and abduction. 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 s shoulder also called as congenital high scapula characterized by an abnormally raised scapula, on one or both the sides the scapular muscles are poorly developed and may be represented by fibrous bands it may be accompanied by scoliosis with. Sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade scapula.
Clinically, sprengels deformity can range from minimal, with no restriction of shoulder motion. It was sprengel2 who recognized that the deformity was caused by failure of the scapula to descend. Learningradiology sprengels, deformity, klippel, feil. Klippel feil syndrome genetic and rare diseases information. Sprengel deformity is the most common congenital abnormality of the shoulder girdle, resulting from an elevated, dysplastic, and variably fixed scapula. Sprengel s shoulder and myositis ossificans mo are rarely seen concomitantly. Utilization 0 the spencer technique may be utilized as an articulation or a muscle energy technique. Sprengel deformity is a congenital failure of the root of the scapula bone. The patients parents explain this deformity has been present since birth, and now the child is unable to reach overhead and participate in play. Thankfully my range of motion is minimally limited compared to severe cases, however i have been experiencing it along with pain, numbness and frustration about the. Sprengel deformity congenital hand and arm differences. Full text full text is available as a scanned copy of the original print version. It occurs due to failure of the scapula to descend during intrauterine development and.
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