Sprengel Deformity with Bilateral Omovertebral Bars in Klippel–Feil Syndrome

A 4-year-old girl child presented with complaints of a short neck with restricted neck and shoulder movements. Her family history was unremarkable. Plain radiography revealed a clear diagnosis of a Sprengel abnormality. Noncontrast computed tomography (CT) with 3D reconstruction revealed fusion of the C6 and C7 vertebral bodies and posterior elements of D2-D3 and D4-D5. The coronal image showed dextroscoliosis, elevated right scapula with a small dysplastic omovertebral bar connecting the C7 posterior element to the superomedial aspect of the right scapula. The volume-rendered image showed a thick triangular hyperdense bony projection connecting the left scapula’s superomedial aspect with the fused C6 posterior element, suggesting an omovertebral bar; however, the left scapula was normal in position. Unfused posterior elements between C4 and C6 vertebral levels were noted. Type 2 split cord malformation (SCM) was noted at the C7 vertebral level [Figure 1a-h].Figure 1: Noncontrast computed tomography (NCCT) and Magnetic resonance imaging (MRI) images of the patient with Klippel feil syndrome. Sagittal NCCT image (a and b) showed block vertebra with fused posterior elements (arrows in a) and omovertebral bar (arrow in b). Coronal NCCT image showed block vertebra with scoliosis to right side (arrow). Volume rendered image (d) showed left side omovertebral bar (thin white arrow) with right side Sprengel shoulder and articulation of right scapula with transverse process of C7 vertebra (white thick arrow). Axial NCCT image (e, f) showed left side omovertebra (white arrow in e) with right side scapular articulation with C7 transverse process (white arrow in f). Sagittal (g) and axial (h) MRI image showed block vertebra (white arrow in g) with type 2 split cord malformation (thin white arrow in h) and articulation of right scapula with transverse process of C7 vertebra (white thick arrow in h)Klippel–Feil syndrome (KFS) is a congenital disorder that occurs due to signaling pathway dysregulation during the development of somites and manifests as the fusion of cervical vertebral bodies.[1] There can be several osseous and nonosseous abnormalities in KFS, including scoliosis, spina bifida occulta, Sprengel deformity, CVJ anomalies, rib, renal, and CNS anomalies.[2] The omovertebral bar which can be seen in one-third of individuals with Sprengel’s deformity is a fibrous or a bony connection between the scapula’s superomedial aspect with the transverse process, lamina, or spinous process of C5 to C7 levels.[3] We report a case of KFS with Sprengel deformity, bilateral omovertebral bars, and type 2 SCM which is an unusual presentation. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.