Sprengels deformity definition of sprengels deformity. Hypoplastic and elevated left scapula a and limitation of elevation b. Sprengel deformity genetic and rare diseases information. Surgical management of sprengels deformity in adults. 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. Klippelfeil syndrome with sprengel deformity and extensive upper extremity deformity. The surgical importance of the clavicular component of sprengels deformity.
Sprengel shoulder is a rare congenital deformity of one or both scapulae that is usually detected at birth. Sprengels deformity is a rare congenital anomaly which arises from the failure of normal descent of the scapula, during embryological development, from its position in the neck, to its normal position in the posterior thorax 2. It is the result of the shoulder blade on one or both sides being underdeveloped hypoplastic and abnormally high, due to failure of descent. Haglunds deformity is a tendency to walk on the outside of the heel. However, the preoperative imaging studies and the intraoperative findings were more supportive of a congenital deformity with a delayed manifestation. Its surgical results in the presence of rib cage anomalies largely remain unreported. To research the causes of sprengel deformity, consider researching the causes of these these diseases that may be similar, or associated with sprengel deformity. Sprengels deformity medical definition merriamwebster. Furthermore, posttraumatic sprengel deformity is an even rarer entity than congenital sprengel deformity, as. 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. Discuss indications for correction of sprengel s deformity 4.
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 congenital condition characterized by abnormal development and elevation of the shoulder blade scapula. Sprengels deformity sd is a congenital structural abnormality of the shoulder girdle. Sprengel deformity nord national organization for rare. An awardwinning, radiologic teaching site for medical students and those starting out in radiology focusing on chest, gi, cardiac and musculoskeletal diseases containing hundreds of lectures, quizzes, handout notes, interactive material, most commons lists and pictorial differential diagnoses. Sprengels deformityfinal authorstream presentation. The patient was a boy, aged 11 years, who was seen at the stanford clinic. 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. A known complication of correcting this deformity is brachial plexus palsy. The affected abnormal shoulder blade is also abnormally connected to the spine, often restricting movement of the shoulder. Sprengel deformity is a rare, congenital disorder seen in children.
Thus, it is uncommon to identify an adult with untreated sprengels deformity. Sprengel s deformity sd is a congenital structural abnormality of the shoulder girdle. 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 sd, undescended scapula or high scapula is the most common congenital abnormality of the shoulder girdle. Klippelfeil syndrome with sprengel deformity radiology. 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. If you continue browsing the site, you agree to the use of cookies on this website.
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. The parents said that he had been born with the left shoulder higher than the right, and that the deformity had steadily grown worse. Sprengels deformity the scapulae normally complete their descent from the neck by. Sprengel deformity is commonly linked to a variety of conditions, including klippelfeil syndrome, scoliosis, and rib anomalies. Discuss indications for correction of sprengels deformity 4. It is commonly associated with scoliosis and sprengel deformity.
Glotz is characterized by the early onset of multiple bcc most commonly seen on the face, back and chestusually between the 1st and 2nd decade of life, benign mandibular odontogenic keratocysts 2nd3rd decade of lifepalmar or plantar pits asymmetrical, mm in diameter, sindrome gorlin goltz in depth and developing in the 2nd decade and skeletal anomalies fusion of vertebrae, bifid or. Normally this disorder is asymmetric with the left scapula most commonly affected, so it will sit higher on the back than the. Many people with sprengel deformity do not need surgery and may have physical therapy to maintain range of motion and strengthen weak muscles. The influence of simple and complex rib anomalies on shoulder abduction, cavendish grade, and radiological rigaults score following vso was studied retrospectively in eight paediatric patients. Sprengels deformity is a rare congenital anomaly of the shoulder girdle. For language access assistance, contact the ncats public information officer. Klippelfeil syndrome is associated with sprengel deformity congenital high scapula, scoliosis, urinary tract anomalies, and congenital heart disease. Media in category sprengels deformity the following 2 files are in this category, out of 2 total. Sprengels shoulder is rare but is the most common shoulder girdle deformity. Undescended scapula results from failed migration of the. 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 kfs is a congenital anomaly resulting from fusion of cervical vertebral bodies secondary to the dysregulation of signaling pathways during somite development. 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. 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. This tendency, which produces wear on the outer edge of the sole of the shoe, causes the heel to rotate inward, resulting in a grinding of the heel bone against the tendon. In sprengel deformity, the scapula is bound to the cervical spine by fibrous tissue, cartilage or an omovertebral bone, which restrict abduction of the shoulder. Sprengel deformity synonyms, sprengel deformity antonyms. If you have problems viewing pdf files, download the latest version of adobe reader. Therefore, it is rare to identify an adult with untreated sd. Sprengel deformity article about sprengel deformity by. Sprengel deformity is a rare congenital disorder in which the shoulder blade scapula is too high on one side of the body. We present a case of kfs with commonly associated abnormalities as well as deformities that have not yet been reported in the.
Sprengels deformity is a congenital structural abnormality of the shoulder girdle, and most patients with sprengels deformity receive surgical treatment as a child or adolescent. The deformity is due to failure of descent of the scapula in intrauterine life. Congenital scoliosis fused ribs congenital skeletal abnormality spina bifida. It is characterised by elevation and medial rotation of inferior part of the affected scapula, which is also smaller and more cephalad than the normal. Sprengel deformity medigoo health medical tests and.
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. Sprengles deformity definition of sprengles deformity. We report a series of four cases of congenital high scapula or sprengels deformity. Since a highergrade sprengel deformity limits the patients function by impeding necessary rotation of the scapula and shoulder girdle, surgical correction of the sprengel deformity was indicated in. Sprengel deformity, or congenital elevation of the scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality. Sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula. Sprengel deformity usually happens in girls and may have a genetic base in some instances. Nonsurgical management can be considered for mild cases.
Discuss surgical approaches to sprengels deformity 5. A unique specimen of unilateral sprengels deformity, and contralateral normal pectoral girdle, were studied morphologically and roentgenographically. Discuss surgical approaches to sprengel s deformity 5. A small spina bifida involving c5 and c6 was present. A unique specimen of unilateral sprengels deformity, and contralateral normal pectoral girdle, were studied. Discuss the reason for the high incidence of other anomalies accompanying sprengel s deformity, and list 4 of the more common accompanying conditions 3. More detailed information about the symptoms, causes, and treatments of sprengel deformity is available below symptoms of sprengel deformity. Vertical scapular osteotomy vso is a known procedure for sprengels shoulder deformity.
Sprengels deformity correction by vertical scapular. Klippelfeil syndrome kfs is a rare condition characterized by the congenital fusion of any two of the seventh cervical vertebrae. This condition also involves regional muscle hypoplasia or atrophy, which causes disfigurement and limitation of shoulder movement. Learningradiology sprengels, deformity, klippel, feil. A rare birth disorder where the shoulder blade is elevated which can affect arm movement.
An initial diagnosis can often be made on radiography, but ct or mri is often necessary to evaluate the details of the abnormality. Klippelfeil syndrome and sprengel deformity combined with. The affected shoulder blade may also be underdeveloped and smaller than normal and rotated toward. Congenital high scapula, commonly known as sprengels deformity sd, is seen as a. Treatment of sprengel deformity depends on the degree of functional impairment andor cosmetic disfigurement. Sprengels deformity is the most common congenital deformity of the shoulder. Sprengel shoulder undescended scapula shoulderdoc by. Discuss the reason for the high incidence of other anomalies accompanying sprengels deformity, and list 4 of the more common accompanying conditions 3. Sprengels deformity associated with musculoskeletal. Sprengels deformity synonyms, sprengels deformity pronunciation, sprengels deformity translation, english dictionary definition of sprengels deformity.
It is characterized by malformation of the shoulder bones leading to restricted shoulder movement and. The cavendish classification of sprengels deformity cavendish, 1972 may be helpful in assessing the need for operative intervention based on appearance. 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 and klippelfeil syndrome leading to. Pdf sprengel deformity sd, undescended scapula or high scapula is the most common congenital abnormality of the shoulder girdle. The main feature of this very rare congenital malformation of the pectoral girdle is an abnormally high, more or less dysmorphic scapula. 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 symptoms, diagnosis, treatments and. Sprengel deformity is the most common congenital abnormality of the shoulder girdle, resulting from an elevated. Sprengel deformity presenting as a posttraumatic injury. For those who do require surgery, the goals are to release the binding of the scapula and relocate the scapula. Sprengels deformity radiology rsna publications online.