Sprengels deformity of the shoulder joint treated by woodward. Klippelfeil syndrome with sprengel deformity and extensive. Scribd is the worlds largest social reading and publishing site. A case of klippel feil anomaly with sprengel shoulder 1. A case of klippel feil anomaly with sprengel shoulder. Sprengel deformity and omovertebral bone, marco pato.
Sprengel deformity is a rare congenital anomaly of the shoulder girdle characterized by fixed elevation and lateral rotation of the scapula, with hypoplasia or atrophy of periscapular muscles causing disfigurement and limited shoulder movement. We report a case of a 14yearold girl with bilateral sprengel s deformity presenting with a. A known complication of correcting this deformity is brachial plexus palsy. It has cosmetic implications that make it readily identifiable, and may be also associated with functional impairment.
Modified woodward procedure for sprengels deformity. Severity can range considerably from being almost invisible when covered with clothes, to the shoulder being elevated over 5 centimeters, with neck webbing. Sprengel deformity is a rare congenital disorder in which the shoulder blade scapula is too high on one side of the body. Sprengels deformity associated with musculoskeletal. Discuss surgical approaches to sprengel s deformity 5. It was sprengel2 who recognized that the deformity was caused by failure of the scapula to descend.
The outcome of 56 children 61 shoulders treated surgically at the rizzoli institute between april 1975 and june 2010 for congenital elevation. Sprengel deformity nord national organization for rare. A unique specimen of unilateral sprengel s deformity, and contralateral normal pectoral girdle, were studied morphologically and roentgenographically. Oct 01, 2018 sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula. Hypoplastic and elevated left scapula a and limitation of elevation b. 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 convexity on the involved side. Signs and symptoms may include a lump in the back of the base of the neck and limited movement in the. 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. The cervical spine exhibited multiple abnormalities involving both the vertebral centra as well as the posterior elements klippelfeil abnormality.
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. Congenital elevation of the scapula is a rare congenital deformity first described by eulenburg1 in 1863. Sprengels shoulder is rare but is the most common shoulder girdle deformity. It is commonly associated with scoliosis and sprengel deformity.
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. Sprengels shoulder treated by the woodward procedure. The patient was a boy, aged 11 years, who was seen at the stanford clinic. 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. Sprengels deformity, qualitative evaluations and elaborate corrective exercise are required. No treatment is required in case of mild deformity. Post the definition of sprengels deformity to facebook share the definition of sprengels deformity on twitter.
Please remove adblock adverts are the main source of revenue for dovemed. Sprengel deformity congenital hand and arm differences. Sprengel deformity, or congenital elevation of the scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality. Click on the link to view a sample search on this topic. 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. Normally this disorder is asymmetric with the left scapula most commonly affected, so it will sit higher on the back than the. Feil syndrome kfs is a congenital anomaly resulting from fusion of cervical vertebral bodies secondary to the dysregulation of signaling pathways during somite development. Scapular osteotomy for sprengels shoulder congenital elevation of the scapula indications.
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. Sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade scapula. Discuss indications for correction of sprengel s deformity 4. Scapular osteotomy for sprengels shoulder congenital. Hydrocephalus, costovertebral dysplasia, and sprengel anomaly. Sprengel shoulder is a rare congenital deformity of one or both scapulae that is usually detected at birth. Abstract purpose to report the functional and cosmetic results of cases with sprengels shoulder who underwent the woodward procedure. 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. Sprengels shoulder free download as powerpoint presentation. The deformity is associated with other congenital anomalies, which often dictate the management and outcome of treatment. Multiple surgical options are available in the treatment of severe sprengels.
In case of severe deformity surgery is done under 6 years of age. Sprengels shoulder dnbid authorstream presentation. Sprengel deformity sd, undescended scapula or high scapula is the most common congenital abnormality of the shoulder girdle. Materials and methods twelve children were operated at a mean. Sprengel s shoulder dnbid authorstream presentation. 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. Discuss the reason for the high incidence of other anomalies accompanying sprengel s deformity, and list 4 of the more common accompanying conditions 3. Sprengel deformity genetic and rare diseases information. The deformity is usually associated with muscle hypoplasia or atrophy, and a combination of these factors results in disfigurement and functional limitation of the shoulder. Passive glenohumeral abduction and rotation are typically normal. An initial diagnosis can often be made on radiography, but ct or mri is often necessary to evaluate the details of the abnormality. This condition also involves regional muscle hypoplasia or atrophy, which causes disfigurement and limitation of shoulder movement.
Sprengels deformity is defined as a congenital failure of the descend of the scapula and was first described by eulenburg 1 in 1863. 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. Sprengels deformity of the shoulder is a dysplasia of the pectoral girdle, resulting in cosmetic and functional disability. Several studies have attempted to analyze the threedimensional aspects of this deformity. I have sprengel s deformity scapula elevata, i wasnt diagnosed with it as a child and therefore didnt have surgery. Jan 05, 2016 sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade scapula. Elaborate corrective exercise program after surgical. Learn indepth information on sprengels shoulder, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. The affected shoulder blade may also be underdeveloped and smaller than normal and rotated toward. 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. Sprengel s deformity is the most common congenital deformity of the shoulder. Sprengels deformity is the most common congenital deformity of the shoulder. Sprengel s deformity is a rare and complex congenital deformity of the shoulder girdle.
Figure 2 shows posterior clinical views of a 4yearold patient with deficient rightsided abduction secondary to sprengels deformity preoperatively and postoperatively. Sprengel shoulder undescended scapula shoulderdoc by prof. Pdf sprengel deformity sd, undescended scapula or high scapula is the most common congenital abnormality of. Carpal coalition and sprengels shoulder in polands. It received its present name after sprengels description in 1891 2. If you continue browsing the site, you agree to the use of cookies on this website. 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 affected abnormal shoulder blade is also abnormally connected to the spine, often restricting movement of the shoulder. The deformity is due to failure of descent of the scapula in intrauterine life. Sprengel deformity ie, congenital elevation of the scapula is a. Sprengels shoulder deformity is a rare condition that happens. Sprengel deformity is the most common congenital abnormality of the shoulder girdle, resulting from an elevated, dysplastic, and variably fixed scapula. Scapula sprengels deformity scapula greens procedure. Apr 18, 20 sprengel s deformity is a rare and complex congenital deformity of the shoulder girdle. 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. Feb 01, 2006 pubmed is a searchable database of medical literature and lists journal articles that discuss hydrocephalus, costovertebral dysplasia, and sprengel anomaly.
Sprengels deformity, a striking abnormality involving one or both scapul. Carpal coalition and sprengels shoulder in polands syndrome. The present study investigated the effectiveness of an elaborate corrective exercise program on the scapulohumeral rhythm and scapular height during shoulder abduction, in addition to shoulder abductor strength, with and. A unique specimen of unilateral sprengels deformity, and contralateral normal pectoral girdle, were studied. Congenital elevation of the scapula sprengel s deformity.
We present a case of kfs with commonly associated abnormalities as well as deformities that have not yet been reported. A small spina bifida involving c5 and c6 was present. The combination of spinal dysraphism and sprengels deformity is rare, and is associated with segmentation defects of the spine and ribs. Connecting the shoulder and the spine article pdf available in child s nervous system 297 february 20 with 576 reads how we measure reads.
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