Abstract
Sprengel deformity is a congenital anomaly arising mainly in the shoulder girdle, associated with elevation of dysplastic scapula. skeletal anomalies, mainly Klippel-Feil syndrome, hemivertebrae, and omovertebral bone may be present along Sprengel anomaly. The omovertebral bone is an abnormal bone that originates from the superomedial edge of the scapula with different insertion points along the posterior cervical spine, seen in about third of the patients with Sprengel anomaly. While cosmetic to functional impairment is a common presentation to the omovertebral bone, cervical myelopathy is a rare presentation. Here, we described our experience, management and follow up of 13-year-old boy presented with cervical myelopathy secondary to the omovertebral bone.
Sprengel deformity is a complex anomaly of the shoulder girdle, associated with elevation of dysplastic scapula.1-4 Clinically, it is associated with disfigurement and limitation of shoulder movement. Other skeletal anomalies – mainly, Klippel–Feil syndrome, hemivertebrae, and omovertebral bone may be present.4 The omovertebral bone is an abnormal bone that originates from the superomedial edge of the scapula with different insertion points along the posterior cervical spine, seen in about a third of patients with Sprengel anomaly.2,5 The clinical presentation ranges from cosmetic to functional impairment, while cervical myelopathy is a rare presentation related to omovertebral bone. The management and follow up is less described in the literature contributed to its rare occurance.6 Here, the author described a 13-year- old boy diagnosed with Sprengel deformity presented with myelopathic manifestation, where cervical magnetic resonance imaging (MRI) showed signal cord changes secondary to compression by intraspinal extension of the omovertebral bone. The patient underwent uneventful resection of the abnormal bone, and spinal cord decompression.
Case Report
Patient perspectives
This 13- year- old boy, presented with a history of imbalance and lower limb stiffness for 3 months.
Clinical findings
Physical examination showed hyper-reflexia in the left knee and ankle jerk without clonus, in addition to positive Babinski sign in the left lower limb. There was bilateral elevation of both scapula when the patient undressed, which represents grade 1 based on the Cavendish classification for Sprengel deformity3 (Table 1).
Cavendish classification.
Diagnostic assessment
The CT of the cervical spine showed abnormal bone extending from the C3/4 vertebrae to the level of C6 (Figure 1 A,B). MRI the cervical spine showed impingement of the omovertebral bone over the dorsal aspect of the spinal cord, with signal cord changes (Figure 2 A,B).
CT cervical spine A) sagittal view with extension of the omovertebral bone dorsally at level of C4 arrow indicate lamina of C4), arrow head indicate the downward extension of omovertebral bone), B) axial view showed the presence of omovertebral bone at the level of C4 vertebrae
MRI cervical spine A) showed impingement of the omovertebral bone over the dorsal aspect of the spinal cord, B) Arrow head showed loss of CSF signal with spinal cord impingement at C4 level.
Therapeutic intervention
Patient underwent C4 laminectomy and complete resection of the omovertebral bone (Figure 3 A,B).
Intraoperative view A) Intraoperative exposure of the omovertebral bone before completely resected, B) showed the bone after complete resection which measured 6cm in its length.
Timeline of patient’s clinical condition and management.
Follow up and outcome
Postoperatively, the patient has his baseline preoperative neurologic status with no deterioration.
Discussion
Sprengel deformity is a congenital anomaly arising mainly in the shoulder girdle, associated with elevation of dysplastic scapula. it is occurred secondary to defect in the signaling of the germ layer differentiation between third and fifth weks of embryonic peroid.5 One-third of Sprengel deformity patients develop an omovertebral bone anomaly, which is described as bone attachment originating from ridge of the scapula with different insertion points to the spinous process, lamina, or transverse process of the cervical vertebrae. The omovertebral bone is usually limited to one side and thought to play a major role in malpositioning of the scapula.2
Willet et al8 was pioneered in describing the omovertebral bone and its resection, also explaining the three hypotheses for its etiology: (1) it is developed from the vertebrae, either as an extra growth of the spinous process or as an excessive development of the lateral projecting process called “hyperapophyses”; (2) it originates from the scapula and becomes fused with the cervical vertebrae by an overgrowth of the epiphysis on the posterior border of the scapula; (3) it develops independently of the spine and scapula as an ossification of the connective tissue between the posterior deep cervical muscles.8,9 The natural history of the omovertebral bone is progressive, as it is associated with calcification and with limitation of shoulder range of motion.
Treatment options for Sprengel deformity associated with omovertebral bone are influenced by different factors, including the degree of limitation of shoulder abduction, presence of myelopathy symptoms and age of the patient.6 Surgical correction in adult patients presenting with deformity is controversial. Doita et al4 described a good result in terms of shoulder range of motion after surgical correction in 2 adult patients. A less satisfactory result has been associated with late presentation of Sprengel deformity. However, Gillespie B et al7 reported good outcomes after surgical correction for a patient with late presentation of Sprengel deformity.
In the literature, only one case of an adult patient with Sprengel deformity and omovertebral bone presented with sign and symptoms of cervical myelopathy has been reported.6 This was managed with spinal decompression and resection of the omovertebral bone. Here, we described the first case for a pediatric patient with Sprengel deformity presented with myelopathy secondary to compression by the omovertebral bone.
The decision to proceed with surgery in cases of mild Sprengel deformity with the omovertebral bone is challenging due to the lack of clearly established guidelines. In our illustrated condition, the surgical option was chosen based on the clinical and radiological picture of the patient.
In conclusion, presentation of Sprengel deformity and omovertebral bone with myelopathic manifestation is rare. Here, we described a pediatric patient with cervical myelopathy secondary to compression by the omovertebral bone associated with mild sprengel deformity. Due to the lack of established guidelines for the management of mild Sprengel deformity, the surgical decision is usually challenging. Further descriptive studies are encouraged to formulate when it is indicated to proceed to surgical intervention rather than conservative option.
Footnotes
Disclosure. The authors declare no conflicting interests, support or funding from any drug company.
- Received July 7, 2020.
- Accepted March 17, 2020.
- Copyright: © Neurosciences
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