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Joseph Junewick, MD FACR
over 7 years ago
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Sprengel Deformity

Case Detail

Anatomy: Musculoskeletal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: CR
Activities:
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History

Child with scoliosis.


Case Images


Diagnosis

Sprengel Deformity

Findings

Elevation and rotation of the left scapula with fibrous attachment to the spinous processes of C5 and C6.

Discussion

Sprengel deformity is a congenital upward and forward displacement of one or both scapulae, which may be up to 12 cm higher than normal. A fibrous band or an “omovertebral bone” connects the scapula to either the C5 or C6 vertebrae. The shoulder girdle is usually abnormal with an absent or weak trapezius muscle, as well as hypoplastic or fibrosed rhomboid and levator scapulae muscles. Clinically, the patient’s neck is thicker and shorter than normal. Klippel- Feil syndrome, congenital spine anomalies (scoliosis, segmentation defects, dysraphism, tethered cord, syrinx and diastematomyelia), situs inversus, genitourinary anomalies and mandibulofacial dystosis may be asscoiated with Sprengel deformity. According to Dave et al., 20-30 % of patients have associated genitourinary tract anomalies, 10% have congenital heart disease and 5 -10 % have spinal dysraphism including diastematomyelia, tethered chord and fibrous bands.

Reference

Dave S, Naik LD. Sprengels deformity: anaesthesia management. J Postgrad Med. 2000 Apr-Jun;46(2):96-7.

Greenspan A. Orthopedic Imaging: a Practical Approach, 4th ed.

Contributor

Tod Mattis, MD



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