Hammer Buur (boxturnip7)

A 16-year-old male presented with primary complaint of worsening dorsal spinal kyphoscoliosis (SKS) for 3 years. More recently, he developed spasticity in legs, breathlessness on mild exertion, and sleep apneas. Apart from SKS, investigations revealed rotatory atlantoaxial dislocation. Atlantoaxial fixation resulted in rapid recovery from all symptoms including from spinal deformity. Observations in this patient suggest that rotatory dislocation can be a cause of spinal deformity.We present a report of two patients having the association of omovertebra, Sprengel's deformity of the shoulder and Klippel-Feil abnormality with craniovertebral junctional instability. Our literature survey did not locate any report of such association. Significance of bone alterations is analyzed. Two young patients presented with neck pain, torticollis, webbed neck, and spastic quadriparesis. In both patients, the investigations revealed basilar invagination, Klippel-Feil abnormality and Sprengel's deformity of the shoulder. Apart from these relatively common associations, both the patients had omovertebral bone that extended from the transverse process of C5 vertebra to scapula. Following atlantoaxial stabilization surgery, the patients rapidly recovered from all symptoms. Musculoskeletal abnormalities at the craniovertebral junction that include Klippel-Feil abnormality, Sprengel's shoulder, and omovertebra are secondary alterations to primary atlantoaxial instability.A relatively rare report of an 8-year-old girl with Maroteaux-Lamy syndrome that is Type VI mucopolysaccharidosis who presented with symptoms of spastic quadriparesis related to atlantoaxial instability is presented. Atlantoaxial stabilization resulted in rapid and sustained neurological recovery.Giant cell reparative granuloma (GCRG) is a benign nonneoplastic granulomatous lesion and is rare in the cranial bone. We present a pediatric case of this lesion arising from the condyle and lower clivus. A 9-year-old girl presented with slowly progressive hoarseness and dysphagia. She showed left glossopharyngeal, vagus, and hypoglossal nerve palsy. An osteolytic lesion around the lower clivus and condyle joint was accompanied by deformation of the craniovertebral junction. An endoscopic endonasal approach was used to decompress the cranial nerve and confirm the pathological finding. The lesion around the condyle was not resected to preserve occipito-cervical stability. The residual lesion has been observed carefully for 6 months, and regrowth has not occurred. GCRG is a rare granulomatous lesion in the cranial bone. This case is the first report of a pediatric clival GCRG. Treating pediatric GCRG may be helpful.A spectrum of vertebral artery (VA) anomalies have been described with or without an associated congenital craniovertebral junction (CVJ) anomalies. C3 segmental VA, where the VA enters the dura at the level of C2/3 intervertebral foramen is an extremely rare anomaly. We report two cases of congenital CVJ anomaly (irreducible in one with C2/3 fusion and reducible in the other; without any subaxial fusion but with articular agenesis at C2/3 joint on the anomalous artery side). Computed tomographic angiography revealed intraspinal intradural entry of VA through the C2/3 intervertebral foramen on the right side with the contralateral artery found crossing the atlanto-axial joint. Both the patients underwent posterior approach and C2 was spared from instrumentation in both cases. Postoperatively, the patient with irreducible dislocation recovered well while the patient with reducible dislocation expired, possibly secondary to the thrombosis of the dominant VA from C2/3 foraminal encroachment. C3 segmental VA may be advantageous in aggressively exposing the C1/2 joint but instrumentation of C2 or C3 needs caution in view of the possibility of VA injury. Our experience shows that VA may be endangered even while exposing and protecting the artery. For such cases, we recommend posterior