Sprengel deformity is a rare congenital disorder in which the shoulder blade scapula is too high on one side of the body. Sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade 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. The parents said that he had been born with the left shoulder higher than the right, and that the deformity had steadily grown worse. A small spina bifida involving c5 and c6 was present. Sprengel deformity is caused by abnormal descent of the scapula during embryonic development. Sprengel deformity ie, congenital elevation of the scapula is a rare clinical entity. Sprengel deformity is associated with cosmetic deformity and decreased shoulder function.
Other terms for this entity are sprengel s anomaly, sprengel s shoulder, congenital high scapula, and undescended scapula. The association between these abnormalities is discussed. Sprengels deformity is an unusual skeletal abnormality. Passive glenohumeral abduction and rotation are typically normal. Seven children between the age group of two and six years were treated for sprengel s deformity, with omovertebral bar, and other congenital anomalies. Congenital elevation of the scapula or sprengels shoulder is an anomaly of the shoulder girdle that is associated with abnormal descent, and altered position. Surgical treatment of sprengels deformity of the scapula in children.
Sprengels shoulder free download as powerpoint presentation. Feb 01, 2006 access to this database is free of charge. 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 deformity presenting as a posttraumatic injury in an. The cervical spine exhibited multiple abnormalities involving both the vertebral centra as well as the posterior elements klippelfeil abnormality. Click on the link to view a sample search on this topic. This condition also involves regional muscle hypoplasia or atrophy, which causes disfigurement and limitation of shoulder movement. We report a series of four cases of congenital high scapula or sprengel s deformity diagnosed and followed in our establishment. Post the definition of sprengel s deformity to facebook share the definition of sprengel s deformity on twitter. In two grade 4 patients, neither cosmetic nor functional effect was achieved and one developed a keloid scar. Congenital elevation of the scapula is a rare congenital deformity first described by eulenburg1 in 1863. Sprengel deformity or congenital elevation of the scapula is a disorder of development that involves a high scapula and limited scapulothoracic motion. Discuss surgical approaches to sprengel s deformity 5. Discuss indications for correction of sprengel s deformity 4.
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. Sprengel s shoulder dnbid authorstream presentation. Sprengels deformity medical definition merriamwebster. The patient was a boy, aged 11 years, who was seen at the stanford clinic. We report a case of unilateral sprengel s deformity associated with several other musculoskeletal and renal disorders consisting of absence of pectoralis major, weakness of. Oct 01, 2018 sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula. This first edition guides you to the present indications for intervention and care in upper limb. Sprengel s shoulder treated by the woodward procedure. Sprengel deformity sd, undescended scapula or high scapula is the most common congenital abnormality of the shoulder girdle. Between september 1994 and march 1999, we used 3dct in 15 patients with the sprengel deformity.
What is it, and what should i use for the icd9 code. The surgical importance of the clavicular component of sprengel s deformity. 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 sdeformity is acongenital elevation of the scapula characterised by medial rotation of. 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 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. Signs and symptoms may include a lump in the back of the base of the neck and limited movement in the. Scapular osteotomy for sprengels shoulder congenital. It received its present name after sprengel s description in 1891 2.
Sprengel s deformity is the most common congenital deformity of the shoulder. 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 deformity with situs inversus in a neonate. Article pdf available october 2018 with 1,085 reads. A known complication of correcting this deformity is brachial plexus palsy. 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. Department of surgery, free university, amsterdam, the netherlands bilateral sprengels syndrome with situs inversus totalis g. 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. Outcome of mears procedure for sprengels deformity bhasker. Sprengel deformity, or congenital elevation of the scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality. Get a printable copy pdf file of the complete article 2. Normally this disorder is asymmetric with the left scapula most commonly affected, so it will sit higher on the back than the.
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. Shoulder abduction and cosmesis did not change appreciably over the years for the patients that did. Sprengel deformity nord national organization for rare. We present a family showing apparently autosomal dominant transmission of sprengel s shoulder with cleft palate. Learn indepth information on sprengels shoulder, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis.
The affected abnormal shoulder blade is also abnormally connected to the spine, often restricting movement of the shoulder. We report the results of the mears procedure in the treatment of sprengel s shoulder. A unique specimen of unilateral sprengel s deformity, and contralateral normal pectoral girdle, were studied morphologically and roentgenographically. Hydrocephalus, costovertebral dysplasia, and sprengel anomaly. 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. My provider dictated that an 8yearold patient has sprengel s deformity. An initial diagnosis can often be made on radiography, but ct or mri is often necessary to evaluate the details of the abnormality. Dominant transmission of sprengels shoulder and cleft palate.
Full text full text is available as a scanned copy of the original print version. Sprengels shoulder dnbid authorstream presentation. Sprengels deformity of the shoulder is a dysplasia of the pectoral girdle, resulting in cosmetic and functional disability. It was sprengel2 who recognized that the deformity was caused by failure of the scapula to descend. Scapula is elevated on the affected side of the shoulder 1. It received its present name after sprengels description in 1891 2.
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 usually happens in girls and may have a genetic base in some instances. Some, but not all, of the kindred showed features of the klippelfeil syndrome. Sprengel deformity is a complex anomaly that is associated with malposition and dysplasia of the scapula. Scribd is the worlds largest social reading and publishing site. 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.
Sprengels deformity associated with musculoskeletal. Sprengel deformity congenital hand and arm differences. A sprengel deformity, or congenital elevation of scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality. Cavendish fourgrade system od evaluation of sprengels deformity grade i very mild shoulder joints are level when dressed,deformity invisible grade ii mild shoulder joints are almost level when dressed,deformity is visible grade iii moderate shoulder joint is elevated 25cm deformity is easily visible. Jun 07, 2016 shoulder range of motion is measured, with particular attention to active forward flexion and abduction. Registered users can save articles, searches, and manage email alerts. Sprengel shoulder undescended scapula shoulderdoc by prof. Sprengel deformity is a rare congenital anomaly that involves the shoulder joint and scapula. Figure 2 shows posterior clinical views of a 4yearold patient with deficient rightsided abduction secondary to sprengels deformity preoperatively and postoperatively.
Pediatric hand and upper limb surgery ebook pdf free download a practical guide edited by peter m. Free full text similar articles sprengel s shoulder. Modified woodward procedure for sprengels deformity. 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 full text of this article is available in pdf format. Pdf sprengel deformity ie, congenital elevation of the scapula is a rare clinical entity. According to the boys mother, he hardly ever complained about it, and was a good swimmer. The main feature of this very rare congenital malformation of the pectoral girdle is an abnormally high, more or less dysmorphic scapula. Sprengels deformity, a striking abnormality involving one or both scapul. Jan 05, 2016 sprengel deformity is a congenital condition characterized by abnormal development and elevation of the shoulder blade scapula. Sprengel deformity genetic and rare diseases information. Please remove adblock adverts are the main source of revenue for dovemed. 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.
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. Abstract sprengel deformity is a rare congenital anomaly that involves the shoulder joint and. Sprengels deformity with situs inversus in a neonate ghulam nabi, md and omar nabi siraj, mbbs introduction. Outcome of mears procedure for sprengels deformity. They all showed better active shoulder joint movement. Severity can range considerably from being almost invisible when covered with clothes, to the shoulder being elevated over 5 centimeters, with neck webbing. Pdf sprengel deformity sd, undescended scapula or high. 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. Sprengel s shoulder free download as powerpoint presentation. Sprengel deformity presenting as a posttraumatic injury. 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 of the shoulder girdle. Pediatric hand and upper limb surgery ebook pdf free download.
Your icd9 book has a notation under this code designating it as the correct choice for. Shoulder range of motion is measured, with particular attention to active forward flexion and abduction. 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. Register for a free account existing user log in existing user log in. Sprengel shoulder undescended scapula shoulderdoc by. Sprengels deformity correction by vertical scapular.
Sprengel s deformity is a rare congenital anomaly of the shoulder girdle. An otherwise healthy 5yearold boy was referred for shoulder asymmetry and limited range of motion on the left. A unique specimen of unilateral sprengels deformity, and contralateral normal pectoral girdle, were studied morphologically and roentgenographically. Sprengel deformity usually coexists with other congenital abnormalities, particularly those. However, it is the most common congenital anomaly of the shoulder. Pubmed is a searchable database of medical literature and lists journal articles that discuss hydrocephalus, costovertebral dysplasia, and sprengel anomaly.
Sprengel s deformity is the congenital failure of descent of the scapula. Sprengels deformity, congenital elevation of the scapula, surgery, grading. 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. 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. The deformity is due to failure of descent of the scapula in intrauterine life. Vertical scapular osteotomy vso is a known procedure for sprengel s shoulder deformity. Its surgical results in the presence of rib cage anomalies largely remain unreported. Pdf sprengel deformity ie, congenital elevation of the scapula is a rare clinical. Bae published by lippincott williams and wilkins about the book. Sprengels shoulder treated by the woodward procedure. The deformity is associated with other congenital anomalies, which often dictate the management and outcome of treatment. Normally, during prenatal development, both scapula begin high in the neck and slowly descend toward their normal resting position in the upper back.
1408 549 256 451 1447 230 1056 1065 1047 848 1256 1432 594 582 890 636 1305 1191 1397 898 646 1209 1362 546 1334 57 1510 1563 672 1251 400 43 989 1401 418 1178 812 1410 522 354 607 1315 1121 902 1385