Sprengel Deformity in Biological Sisters

Pargas, Carlos; Santana, Adolfredo; Czoch, Wojciech L.; Rogers, Kenneth; Mackenzie, William G.

doi:10.5435/jaaosglobal-d-19-00120

Cited by 5 publications

(9 citation statements)

References 10 publications

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“…Surgery consists of relocating the scapula to its anatomical position and removing the omovertebral bone, which is responsible for the restriction seen in scapular motion. [6][7][8] Few cases of adult patients with SD who underwent surgery are described in the literature. Doita et al presented 2 adult patients with unilateral SD and an omovertebral bone that differed in size and position in both cases.…”

Section: Discussionmentioning

confidence: 99%

“…5 The treatment of SD is based on patient's age, degree of functional impairment, and cosmetic concerns. Awareness regarding children's psychological development should also be considered 6 . Surgery is recommended between 3 and 7 years of age when moderate to severe shoulder impairment and/or severe cosmetic concerns are present 1,5 .…”

mentioning

confidence: 99%

“…Several techniques have been described, but the most popular are the Green and Woodward procedures aiming for scapula repositioning with resection of the omovertebral bone or fibrous structure when detected. 6…”

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confidence: 99%

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Bilateral Sprengel Deformity With Bilateral Omovertebral Bone: An Unusual Case in an Adult Patient

Pinheiro

Fernandes

Sarmento

2023

JBJS Case Connector

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Case:We describe an adult patient with bilateral Sprengel deformity (BSD) with bilateral omovertebral bone and thoracic spinal failure of segmentation (T2-T4) successfully surgically treated only at 19-year-old. After resection of the bilateral omovertebral bone and simultaneous relocation of the scapula, disabilities of the arm, shoulder, and hand score improved from 26.7 preoperatively to 2.5 at the 3-year follow-up.Conclusion:The surgical treatment of Sprengel deformity is typically recommended between 3 and 7 years of age when significant shoulder impairment and/or severe cosmetic concerns are present. The authors were able to achieve a successful surgical outcome in a patient with BSD despite an unusual late presentation.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Bilateral Sprengel Deformity With Bilateral Omovertebral Bone: An Unusual Case in an Adult Patient

Pinheiro

Fernandes

Sarmento

2023

JBJS Case Connector

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“…Surgery was not necessary if a patient had good shoulder abduction, regardess of how bad their deformity was. 5 Patients who have good shoulder abduction recently applied to clinics asking for cosmesis improvement. Some parents or patients might still ask for surgery to improve their appearance.…”

Section: Discussionmentioning

confidence: 99%

Surgical Correction of Sprengel Deformity of the Shoulder by Wood Ward’s Technique – A Case Report

Arun¹,

Kumar²,

Jagadish³

et al. 2022

JCBS

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The most typical congenital defect of the shoulder girdle is called Sprengel deformity (SD), also known as congenital high scapula or congenital undescended scapula. It is distinguished by a protrusion at the scapula's upper medial border and a restriction of shoulder abduction. 7-year-old female child was brought to the hospital with complaints of difficulty to perform overhead abduction and their parents complained of cosmetic defect noted over the right upper back i.e., asymmetry of upper back. On examination, the child was able to per- form overhead abduction from 0-100deg at gleno humeral joint after which scapula-thoracic motion takes place. X ray revealed a high or undescended scapula. CT scan was performed as a pre-operative planning proto- col to rule out the presence of omovertebra. No omovertebrae noted and deformity was graded as Cavendish grade III. Patient was taken up for proposed surgery. Postoperatively patient was put in shoulder arm pouch for 4 weeks and gradually started on shoulder range of motion exercises. At final follow up, wound healed completely and patient was able to abduct the shoulder for 0-14’0deg. We conclude that treating sprengel deformity with the Woodward method correctly combined with post-operative therapy yields out- standing functional and aesthetic benefits. Keywords: Sprengel defor

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“…28 Defined as a hypoplastic and high-riding scapula, Sprengel deformity has been reported in 6.4% to 16.7% of KFS cases. 30 It is often associated with an omovertebral bar, or an osseous or fibrous connection between the superomedial scapula and cervical spine, in approximately half of patients (Figure 2). This anomaly has added significance in cases of KFS due to already limited mobility in the cervical spine, especially with the omovertebral bar being most commonly connected between C4 and C7.…”

Section: Associated Conditions Sprengel Deformitymentioning

confidence: 99%

Klippel-Feil Syndrome: Pathogenesis, Diagnosis, and Management

Litrenta

Dryer

2021

J Am Acad Orthop Surg

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Klippel-Feil syndrome (KFS), or congenital fusion of the cervical vertebrae, has been thought to be an extremely rare diagnosis. However, recent literature suggests an increased prevalence, with a high proportion of asymptomatic individuals. Occurring as a sporadic mutation or associated with several genes, the pathogenesis involves failure of cervical somite segmentation and differentiation during embryogenesis. Most commonly, the C2-C3 and C5-C6 levels are involved. KFS is associated with other orthopaedic conditions including Sprengel deformity, congenital scoliosis, and cervical spine abnormalities, as well as several visceral pathologies. There are several classification systems, some based on the anatomic levels of fusion and others on its genetic inheritance. Management of patients with KFS primarily involves observation for asymptomatic individuals. Surgical treatment may be for neurologic complaints, correction of deformity, concomitant spinal anomalies, or for associated conditions and varies significantly. Participation in sports is an important consideration. Recommendations for contact sports or activities depend on both the level and the number of vertebrae involved in the fusion. A multidisciplinary team should be involved in the treatment plan and recommendations for complex presentations.

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Sprengel Deformity in Biological Sisters

Cited by 5 publications

References 10 publications

Bilateral Sprengel Deformity With Bilateral Omovertebral Bone: An Unusual Case in an Adult Patient

Bilateral Sprengel Deformity With Bilateral Omovertebral Bone: An Unusual Case in an Adult Patient

Surgical Correction of Sprengel Deformity of the Shoulder by Wood Ward’s Technique – A Case Report

Klippel-Feil Syndrome: Pathogenesis, Diagnosis, and Management

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