Ct and mr imaging of the neck and upper thorax demonstrated a cervical osseous segmentation anomaly, a left common carotid artery originating from the innominate artery, and a left subclavian artery coursing through the. The degree of rotation and superior displacement were measured on the. Surgical treatment of sprengels deformity of the scapula in children. Congenital high scapula, commonly known as sprengels deformity sd, is seen as a characteristic dysmorphic high scapula. The mobility of the scapula is lessened and the movements of the arm are restricted. The patient was a boy, aged 11 years, who was seen at the stanford clinic. Normally this disorder is asymmetric with the left scapula most commonly affected, so it will sit higher on the back than the. Sprengel deformity is the most common congenital malformation of the shoulder girdle. If you continue browsing the site, you agree to the use of cookies on this website. Sprengel s deformity is a congenital structural abnormality of the shoulder girdle, and most patients with sprengel s deformity receive surgical treatment as a child or adolescent. Sprengel deformity symptoms, diagnosis, treatments and causes. Two decades later, willet and walsham reported two cases with anatomic descriptions of.
Sprengels shoulder is rare but is the most common shoulder girdle deformity. Sprengels deformity pediatric orthopaedic society of. 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. We present a case of kfs with commonly associated abnormalities as well as deformities that have not yet been reported in the. Sprengels deformity associated with musculoskeletal. This is a rhomboid or trapezoidshaped structure of cartilage or bone. Sprengel deformity is a rare congenital anomaly that involves the shoulder joint and scapula. Ct and mr imaging of the neck and upper thorax demonstrated a cervical osseous segmentation anomaly, a left common carotid artery originating from the innominate artery, and a left subclavian artery coursing. Coexistence of klippelfeil syndrome with sprengel deformity and omovertebral bone is a rare complex bone abnormality with unknown incidence and etiology. The cavendish classification of sprengels deformity cavendish, 1972 may be helpful in assessing the need for operative intervention based on appearance. 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.
Several studies have attempted to analyze the threedimensional aspects of this deformity. Between 2002 and 2009, 23 scapulae in 22 patients were treated. It is caused by failure of scapular descent during the 9th12th weeks of gestation. Modified woodward procedure for sprengels deformity. Klippel feil syndrome kfs is a condition affecting the development of the bones in the spine. We evaluated scapular dysplasia and malposition in 15 patients with the sprengel deformity using threedimensional ct 3dct. Feil syndrome kfs is a congenital anomaly resulting from fusion of cervical vertebral bodies secondary to the dysregulation of signaling pathways during somite development. Sprengel deformity synonyms, sprengel deformity antonyms.
I have a mild case of sprengels deformity of the right scapula which in and of itself is not curable except through radical surgery. An initial diagnosis can often be made on radiography, but ct or mri is often necessary to evaluate the details of the abnormality. Sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula a large triangular flattened bone lying over the ribs, posteriorly on either side, articulating laterally with the clavicle at the acromioclavicular joint and the humerus at the glenohumeral joint. Abstract sprengel deformity is a rare congenital anomaly that involves the shoulder joint and. Jun 07, 2016 the cavendish classification of sprengels deformity cavendish, 1972 may be helpful in assessing the need for operative intervention based on appearance. Pdf sprengel deformity ie, congenital elevation of the scapula is a rare clinical. Pdf madelung deformity is a common finding in various conditions affecting the wrist, with characteristic changes in the radius, carpus and ulna, the most characteristic being the triangular madelung deformity is due to premature closure or defective development of the ulnar third of the distal. It is characterized by malformation of the shoulder bones leading to restricted shoulder movement and other related signs and symptoms. Sprengel deformity article about sprengel deformity by the. Sprengel deformity sd is a rare congenital malformation of the scapula, named after otta gerhard karl sprengel in 1891 1, who suggested that increased amniotic fluid pressure interfered with the descent of the scapula, hence the appellation deformity 1,2.
As sprengel s deformity is usually unilateral, the patient has an asymmetrical appearance, the neck being thicker and shorter on the affected side. 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. More detailed information about the symptoms, causes, and treatments of sprengel deformity is available below symptoms of sprengel deformity. Oct 01, 2018 sprengel deformity is the most common congenital malformation of the shoulder girdle. Jan 05, 2016 sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade scapula. Sprengels deformity definition of sprengels deformity. 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. The full text of this article is available in pdf format. Another anomaly seen in approximately one third of patients with a sprengel deformity is the omovertebral bone. This rare congenital condition of unknown aetiology is the consequence of arrested caudal migration of the scapula during embryogenesis. Sprengel deformity medigoo health medical tests and. Sprengel s deformity a condition characterized by a faulty descent of the scapula from its fetal position next to c4, accompanied by a simultaneous scapular rotation, resulting in a horizontal axillary margin. Sprengel deformity congenital hand and arm differences. Sprengels deformity a condition characterized by a faulty descent of the scapula from its fetal position next to c4, accompanied by a simultaneous scapular rotation, resulting in a horizontal axillary margin.
Congenital elevation of the scapulasprengels deformity. Sprengel deformity presenting as a posttraumatic injury. May 06, 2016 sprengel deformity presented by dr joydeep mallik ganga hospital, coimbatore,tamilnadu slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Sprengel deformity, also known as congenital high scapula, undescended scapula, or scapula elevata, is a rare congenital deformity of one or both scapulae that appears at birth, although pellegrin et al. Pdf sprengel deformity ie, congenital elevation of the scapula is a rare clinical entity. Sprengel deformity, or congenital elevation of the scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality. 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. Sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula. Aim congenital undescended scapula sprengel deformity is a rare. Aug 14, 2019 sprengel deformity is a rare, congenital disorder seen in children. Sprengel deformity genetic and rare diseases information.
Two decades later, willet and walsham reported two cases with anatomic descriptions of this clinical entity. Lit sir richard calmady, walsh modern, 84 carey, philip embittered young man with club foot seeks fulfillment. Undescended scapula results from failed migration of the. Klippelfeil syndrome kfs is a congenital anomaly resulting from fusion of cervical vertebral bodies secondary to the dysregulation of signaling pathways during somite development.
Sprengel s deformity is a relatively rare condition consisting of an elevated, dysplastic scapula that causes cosmetic deformity and restriction of shoulder range of motion. Definition congenital elevation of the scapula orsprengels shoulder is an anomaly of the shoulder girdle that is associated with abnormal descent, and altered position and anatomy of the scapula. As sprengels deformity is usually unilateral, the patient has an asymmetrical appearance, the neck being thicker and shorter on the affected side. Common features may include a short neck, low hairline at the back of the head, and restricted movement of the upper spine.
More detailed information about the symptoms, causes, and treatments of sprengel deformity is available below. Sprengel deformity is an uncommon condition in which the scapula shoulder blade on one side is higher in the neck than the other side. A rare birth disorder where the shoulder blade is elevated which can affect arm movement. An anteroposterior chest xray, or chest radiograph, can accurately be used to diagnose sprengel deformity. Herein, we report a case of a 6yearold girl with coexistence of these congenital.
Currently, the etiology is unknown but has been postulated to involve neural crest. Normally, during prenatal development, both scapula begin high in the neck and slowly descend toward their normal resting position in the upper back. It is commonly associated with scoliosis and sprengel deformity. Sprengel deformity symptoms, diagnosis, treatments and. Diagnostic confusion with limited scoliosis can be dangerous to the patient because it may delay proper treatment of other abnormalities that may be present with even mild cases. We present a case of kfs with commonly associated abnormalities as well as deformities that have not yet been reported.
This condition also involves regional muscle hypoplasia or atrophy, which causes disfigurement and limitation of shoulder movement. Sprengel deformity usually happens in girls and may have a genetic base in some instances. Sprengels 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. Sprengel deformity is associated with cosmetic deformity and decreased shoulder function. 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. Pdf sprengel deformity sd, undescended scapula or high. Surgical management of sprengels deformity in adults. Sprengels deformity is a relatively rare condition consisting of an elevated, dysplastic scapula that causes cosmetic deformity and restriction of shoulder range of motion. Sprengel deformity sd, undescended scapula or high scapula is the most common congenital abnormality of the shoulder girdle. Sprengles deformity definition of sprengles deformity by.
Sprengels deformity radiology rsna publications online. Sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade scapula. Sprengels deformity pediatric orthopaedic society of north. Oct 01, 2018 sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula. It is the result of the shoulder blade on one or both sides being underdeveloped hypoplastic and abnormally high, due to failure of descent. People with kfs are born with abnormal fusion of at least two spinal bones vertebrae in the neck. Sprengel deformity article about sprengel deformity by. We present a case of klippelfeil syndrome and sprengel deformity with a bovine aortic arch and an aberrant course of the left subclavian artery in a 14yearold boy. A sprengel deformity, or congenital elevation of scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality sprengel deformity usually coexists with other congenital abnormalities, particularly those involving the vertebrae and ribs.
Sprengel deformity is a sporadic condition, which indicates that the exact cause of the condition is not known. Sprengel deformity presenting as a posttraumatic injury in an. In 1863, eulenberg first described what later became known as sprengel deformity. Sprengels deformity, a striking abnormality involving one or both scapul. Sprengels deformity, congenital elevation of the scapula, surgery, grading. 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. Severity can range considerably from being almost invisible when covered with clothes, to the shoulder being elevated over 5 centimeters, with neck webbing. There are weakness and poor development of the muscles which normally hold the scapula, especially the trapezius 5. The deformity is due to failure of descent of the scapula in intrauterine life. However, it is the most common congenital anomaly of the shoulder. 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. 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. Oct 27, 2014 sprengel deformity, also known as congenital high scapula, undescended scapula, or scapula elevata, is a rare congenital deformity of one or both scapulae that appears at birth, although pellegrin et al. A sprengel deformity, or congenital elevation of scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality.
Sprengels deformity definition of sprengels deformity by. The parents said that he had been born with the left shoulder higher than the right, and that the deformity had steadily grown worse. Medical definition of sprengels deformity merriamwebster. Nonsurgical management can be considered for mild cases. Klippelfeil syndrome and sprengel deformity combined with. Sprengels shoulder dnbid authorstream presentation. Signs and symptoms may include a lump in the back of the base of the neck and limited movement in the.
Sprengel deformity is caused by abnormal descent of the scapula during embryonic development. The shape, heighttowidth ratio, the areas of both scapulae, the anterior curvature of the supraspinous portion and glenoid version were assessed on scapular posterior, medial and inferior views. Sprengles deformity definition of sprengles deformity. We report a case of unilateral sprengels deformity associated with several other musculoskeletal and renal disorders consisting of absence of pectoralis major, weakness of trapezius and serratus anterior. Klippelfeil syndrome with sprengel deformity and extensive. Sprengel deformity medigoo health medical tests and free. Otto gerhard karl sprengel, german surgeon, 18521915 congenital elevation of the scapula, resulting from failure of descent of the scapula to its normal thoracic position during fetal life.
Sprengels deformity is a rare congenital anomaly of the shoulder girdle. Congenital elevation of the scapula or sprengels shoulder is an anomaly of the shoulder girdle that is associated with abnormal descent, and altered position. A diagnosis of sprengel deformity is based upon the identification of characteristic symptoms, a thorough clinical evaluation, and a variety of specialized tests, particularly advanced imaging tests. 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. A limited intervention such as excision of superomedial scapular prominence may be utilized for grades 1 and 2 whereas a more significant procedure to relocate the scapula, such as the. Klippelfeil syndrome and sprengel deformity combined with an.
Sprengel deformity nord national organization for rare. Sprengels deformity synonyms, sprengels deformity pronunciation, sprengels deformity translation, english dictionary definition of sprengels deformity. Sprengel deformity is commonly linked to a variety of conditions, including klippelfeil syndrome, scoliosis, and rib anomalies. Sprengel deformity is a rare, congenital disorder seen in children.
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