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Which of the following conditions is characterized by failure of the scapula to migrate caudally during fetal development?
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Sprengel's deformity is caused by failure of the shoulder to descend caudally during fetal development. This leads to elevation and medial rotation of the inferior border of the scapula. It is associated with Klippel-Feil syndrome, congenital scoliosis, fused ribs, the presence of an omovertebral bone and myelomeningocele. Treatment is aimed at improved cosmesis and range of motion of the shoulder.
Bellemans et al and Leibovic et al successfully treated Sprengel's deformity patients with a modified Green's procedure which entails transfer of the peri-scapular muscles (via detachment at the scapula).
Borges et al improved function with a different procedure called the modified Woodward, which lowers the scapula via detaching muscles off the spinous processes before reattaching more distally and adding a resection of the supero-medial scapula.
Bellemans M, Lamoureux J.
J Pediatr Orthop B. 1999 Jul;8(3):194-6. PMID: 10399122 (Link to Abstract)
Bellemans, JPOBR 1999
Leibovic SJ, Ehrlich MG, Zaleske DJ.
J Bone Joint Surg Am. 1990 Feb;72(2):192-7. PMID: 2303505 (Link to Abstract)
Leibovic, JBJS 1990
Borges JL, Shah A, Torres BC, Bowen JR.
J Pediatr Orthop. 1996 Jul-Aug;16(4):508-13. PMID: 8784708 (Link to Abstract)
Borges, JPO 1996
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A 4-year-old boy with Klippel-Feil syndrome has elevation of the left scapula since birth. Spine radiographs shows no evidence of scoliosis. What shoulder motion is likely to be most limited?
This patient has Sprengel’s deformity or congenital elevation of the scapula, the most common congenital shoulder abnormality. Associated conditions with Sprengel's deformity include Klippel-Feil syndrome, congenital scoliosis, and torticollis. Function of the shoulder mild cases is typically good, with clinically significant limitations in abduction and forward flexion limited to moderate and severe cases.
Harvey et al report that shoulder abduction is the most common reduction in glenohumeral motion and is poorly compensated by a concomitant lack of scapulothoracic motion. They suggest surgery is indicated due for functional or cosmetic reasons in Cavendish Class III and IV, which are highlighted by shoulder elevation >2 cm and abduction typically less than 90 °.
Farsetti in a retrospective analysis of 8 patients with Sprengel's reported an average improvement in shoulder abduction of 38 degrees and 7 of 8 reported improved cosmesis.
Farsetti P, Weinstein SL, Caterini R, De Maio F, Ippolito E
J Pediatr Orthop B. 2003 May;12(3):202-10. PMID: 12703036 (Link to Abstract)
Farsetti, JPOBR 2003
Harvey EJ, Bernstein M, Desy NM, Saran N, Ouellet JA.
J Am Acad Orthop Surg. 2012 Mar;20(3):177-86. PMID: 22382290 (Link to Abstract)
Harvey, JAAOS 2012
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HPI - A 9 year old boy is brought in by his parents with asymmetry and tilting of his head since birth.
When evaluating a patient with Sprengel deformity, what imaging is required?