Aims. Although atlantoaxial rotatory fixation (AARF) is a common cause of torticollis in children, the diagnosis may be delayed. The condition is characterised by a lack of rotation at the atlantoaxial joint which becomes fixed in a rotated and subluxed position. The management of children with a delayed presentation of this condition is controversial. This is a retrospective study of a group of such children. Patients and Methods. Children who were admitted to two institutions between 1988 and 2014 with a diagnosis of AARF were included. We identified 12 children (four boys, eight girls), with a mean age of 7.3 years (1.5 to 13.4), in whom the duration of symptoms on presentation was at least four weeks (four to 39). All were treated with halo traction followed by a period of cervical immobilisation in a halo vest or a Minerva jacket. We describe a simple modification to the halo traction that allows the child to move their head whilst maintaining traction. The mean follow-up was 59.6 weeks (24 to 156). Results. Despite the delay in referral, the subluxation was successfully reduced in all children. Only two children required atlantoaxial fusion. Conclusion. The results of our study suggest that normal anatomy with restoration of movement may be achieved even in cases of AARF presenting late, obviating the need for fusion. We also show a simple modification to the halo traction that allows the child to move their head about while still maintaining traction. Take home message: Prompt diagnosis and management with halo traction (with a simple modification as described) is associated with good results in patients with AARF who present late. Cite this article: Bone Joint J 2016;98-B:715–20

We evaluated the use of surgical stabilisation for atlantoaxial subluxation after a follow-up of 24 years in 50 rheumatoid patients who had some degree of pain but no major neurological deficit. The mortality of patients treated by atlantoaxial fusion was significantly lower than for those who received conservative treatment. The deaths resulted from infection or comorbid conditions. The significantly high relative risks of mortality from conservative treatment compared with surgical treatment were mutilating disease and susceptible factors on both of the HLA-DRB1 alleles. Relief from pain and neurological and functional recovery were better, and the radiological degree of atlantoaxial translocation was less in those who were surgically treated compared with those who were not. Two patients had superficial local infections after surgery. We conclude that prophylactic atlantoaxial fusion is better than conservative treatment in these patients

We present seven children with atlantoaxial rotatory fixation (AARF) of more than three months’ duration after an injury to the upper cervical spine. The deformity was irreducible by skull traction. MRI and MR angiography (MRA) of the vertebral arteries were performed in four children. The patients were neurologically intact. Thrombosis of the ipsilateral vertebral artery was noted in two patients. The deformity was gradually corrected and stabilised after transoral release of the atlantoaxial complex, skull traction and posterior atlantoaxial fusion. Soft-tissue interposition and contractures within the atlantoaxial complex prevented closed reduction. MRI and MRA of the vertebral arteries were useful in elucidating the pathology of chronic atlantoaxial rotatory fixation

Transarticular screw fixation with autograft is an established procedure for the surgical treatment of atlantoaxial instability. Removal of the posterior arch of C1 may affect the rate of fusion. This study assessed the rate of atlantoaxial fusion using transarticular screws with or without removal of the posterior arch of C1. We reviewed 30 consecutive patients who underwent atlantoaxial fusion with a minimum follow-up of two years. In 25 patients (group A) the posterior arch of C1 was not excised (group A) and in five it was (group B). Fusion was assessed on static and dynamic radiographs. In selected patients CT imaging was also used to assess fusion and the position of the screws. There were 15 men and 15 women with a mean age of 51.2 years (23 to 77) and a mean follow-up of 7.7 years (2 to 11.6). Stable union with a solid fusion or a stable fibrous union was achieved in 29 patients (97%). In Group A, 20 patients (80%) achieved a solid fusion, four (16%) a stable fibrous union and one (4%) a nonunion. In Group B, stable union was achieved in all patients, three having a solid fusion and two a stable fibrous union. There was no statistically significant difference between the status of fusion in the two groups. Complications were noted in 12 patients (40%); these were mainly related to the screws, and included malpositioning and breakage. The presence of an intact or removed posterior arch of C1 did not affect the rate of fusion in patients with atlantoaxial instability undergoing C1/C2 fusion using transarticular screws and autograft. Cite this article: Bone Joint J 2013;95-B:972–6

We reviewed the outcome of a retrospective case series of eight patients with atlantoaxial instability who had been treated by percutaneous anterior transarticular screw fixation and grafting under image-intensifier guidance between December 2005 and June 2008. The mean follow-up was 19 months (8 to 27). All eight patients had a solid C1–2 fusion. There were no breakages or displacement of screws. All the patients with pre-operative neck pain had immediate relief from their symptoms or considerable improvement. There were no major complications. Our preliminary clinical results suggest that percutaneous anterior transarticulation screw fixation is technically feasible, safe, useful and minimally invasive when using the appropriate instruments allied to intra-operative image intensification, and by selecting the correct puncture point, angle and depth of insertion

In normal, physiological circumstances there is ample room in the spinal canal to accommodate the spinal cord. Our study aimed to identify the degree of compromise of the spinal canal which could be anticipated in various atlantoaxial pathological states. We examined paired atlas and axis vertebrae using high-definition radiography and simultaneous photography in both normal and simulated pathological orientations in order to measure the resultant dimension of the spinal canal and its percentage occlusion. At the extreme of physiological axial rotation (47°) the spinal canal is reduced to 61% of its cross-sectional area in neutral rotation. The spinal cord is thus safe from compromise. Atlantoaxial subluxation of up to 9 mm reduces the area of the spinal canal, in neutral rotation, to 60% with no cord compromise. Any rotation is, however, likely to cause cord compression. The mechanism of fixation in atlantoaxial rotatory subluxation could be explained by bony interlocking of the facet joint, reproducible in dry bones

Fracture of the atlas is rare in children. We report a case of fracture of the atlas through a synchondrosis of the anterior arch complicated by atlantoaxial rotatory fixation in a four-year-old girl

Three-dimensional CT was used to examine the atlantoaxial joint of a nine-year-old girl who presented with fixed rotation of the head 3.5 months after an apparently spontaneous acute torticollis. The method provided clear, anatomical images to show the site, extent and direction of the type-I rotatory fixation (Fielding and Hawkins 1977). The 3-D reconstructions helped to guide manipulation which was successful in this late case

Ankylosing spondylitis (AS) is a progressive multisystem chronic inflammatory disorder. The hallmark of this pathological process is a progressive fusion of the zygapophyseal joints and disc spaces of the axial skeleton, leading to a rigid kyphotic deformity and positive sagittal balance. The ankylosed spine is unable to accommodate normal mechanical forces, rendering it brittle and susceptible to injury. Traumatic hyperextension injury of the cervical spine leading to atlantoaxial subluxation (AAS) in AS patients can often be fatal. We report a non-traumatic mechanism of injury in AS progressing to AAS attributable to persistent hyperextension, which resulted in fatal migration of C2 through the foramen magnum. Cite this article: Bone Joint J 2013;95-B:206–9

Aims

To determine the normal values and usefulness of the C1/4 space available for spinal cord (SAC) ratio and C1 inclination angle, which are new radiological parameters for assessing atlantoaxial instability in children with Down syndrome.

Traumatic atlanto-occipital dislocation in adults is usually fatal and survival without neurological deficit is rare. The surgical management of those who do survive is difficult and controversial. Most authorities recommend posterior occipitoaxial fusion, but this compromises cervical rotation. We describe a case in which a patient with a traumatic atlanto-occipital disruption but no neurological deficit was treated by atlanto-occipital fusion using a new technique consisting of cancellous bone autografting supported by an occipital plate linked by rods to lateral mass screws in the atlas. The technique is described in detail. At one year the neck was stable, radiological fusion had been achieved, and atlantoaxial rotation preserved. The rationale behind this approach is discussed and the relevant literature reviewed. We recommend the technique for injuries of this type

Fractures of the odontoid in children with an open basilar synchondrosis differ from those which occur in older children and adults. We have reviewed the morphology of these fractures and present a classification system for them. There were four distinct patterns of fracture (types IA to IC and type II) which were distinguished by the site of the fracture, the degree of displacement and the presence or absence of atlantoaxial dislocation. Children with a closed synchondrosis were classified using the system devised by Anderson and D’Alonzo. Those with an open synchondrosis had a comparatively lower incidence of traumatic brain injury, a higher rate of missed diagnosis and a shorter mean stay in hospital. Certain subtypes (type IA and type II) are likely to be missed on plain radiographs and therefore more advanced imaging is recommended. We suggest staged treatment with initial stabilisation in a Halo body jacket and early fusion for those with unstable injuries, severe displacement or neurological involvement

Posterior cervical wiring is commonly performed for patients with spinal instability, but has inherent risks. We report eight patients who had neurological deterioration after sublaminar or spinous process wiring of the cervical spine; four had complete injuries of the spinal cord, one had residual leg spasticity and three recovered after transient injuries. We found no relation between the degree of spinal canal encroachment and the severity of the spinal-cord injury, but in all cases neurological worsening appeared to have been caused by either sublaminar wiring or spinous process wiring which had been placed too far anteriorly. Sublaminar wiring has substantial risks and should be used only at atlantoaxial level, and then only after adequate reduction. Fluoroscopic guidance should be used when placing spinous process wires especially when the posterior spinal anatomy is abnormal

Structural defects of the posterior arch of the atlas are rare, and range from clefts of variable location and size to more extensive defects such as complete agenesis. These abnormalities are usually incidental radiological findings. We present a case of a fracture of the anterior arch of the atlas associated with a congenital abnormality of the posterior arch.

Increasing innovation in rapid prototyping (RP) and additive manufacturing (AM), also known as 3D printing, is bringing about major changes in translational surgical research.

This review describes the current position in the use of additive manufacturing in orthopaedic surgery.

Cite this article: Bone Joint J 2018;100-B:455-60.

The effect of rheumatoid arthritis on the anatomy of the cervical spine has not been clearly documented. We studied 129 female patients, 90 with rheumatoid arthritis and 39 with other pathologies (the control group). There were 21 patients in the control group with a diagnosis of cervical spondylotic myelopathy, and 18 with ossification of the posterior longitudinal ligament. All had plain lateral radiographs taken of the cervical spine as well as a reconstructed CT scan. The axial diameter of the width of the pedicle, the thickness of the lateral mass, the height of the isthmus and internal height were measured. The transverse diameter of the transverse foramen (d1) and that of the spinal canal (d2) were measured, and the ratio d1/d2 calculated.

The width of the pedicles and the thickness of the lateral masses were significantly less in patients with rheumatoid arthritis than in those with other pathologies. The area of the transverse foramina in patients with rheumatoid arthritis was significantly greater than that in the other patients. The ratio of d1 to d2 was not significantly different. A high-riding vertebral artery was noted in 33.9% of the patients with rheumatoid arthritis and in 7.7% of those with other pathologies. This difference was statistically significant. In the rheumatoid group there was a significant correlation between isthmus height and vertical subluxation and between internal height and vertical subluxation.

We present the case of an 83-year-old man who developed quadriparesis and respiratory embarrassment following osteomyelitis at the occipito-atlantoaxial junction. He had developed an abscess at this site after an earlier urinary infection with methicillin-resistant staphylococcus aureus. Stabilisation of the neck and antibiotic therapy led to an almost complete neurological recovery without recourse to anterior surgery.

The management of joint replacement in lysosomal storage diseases has not been well reported. We present three patients with progressive degenerative changes of the hips who required bilateral total hip replacement in early childhood. The stature of the patients make it essential to have access to appropriately scaled prostheses. Consideration has to be given to associated disorders of the skeleton which must be carefully screened to ensure safety in providing appropriate anaesthesia as well as ensuring that there is no cardiac abnormality. In one patient, a periprosthetic fracture was sustained in one hip in the early post-operative course requiring internal fixation.

The patient made a full recovery and all six hips were clinically and radiologically satisfactory at mid-term review.

We present the case of a 15-year-old boy with symptoms due to Klippel–Feil syndrome. Radiographs and CT scans demonstrated basilar impression, occipitalisation of C1 and fusion of C2/C3. MRI showed ventral compression of the medullocervical junction. Skull traction was undertaken pre-operatively to determine whether the basilar impression could be safely reduced. During traction, the C3/C4 junction migrated 12 mm caudally and spasticity resolved. Peri-operative skull-femoral traction enabled posterior occipitocervical fixation without decompression. Following surgery, cervical alignment was restored and spasticity remained absent. One year after surgery he was not limited in his activities. The surgical strategy for patients with basilar impression and congenital anomalies remains controversial. The anterior approach with decompression is often recommended for patients with ventral compression of the medullocervical region, but such procedures are technically demanding and carry a significant risk of complications. Our surgical strategy was an alternative solution. Prior to a posterior cervical fixation, without decompression, skull traction was used to confirm that the deformity was reducible and effective in resolving associated myelopathy.

Patients with skeletal dysplasia are prone to developing advanced osteoarthritis of the knee requiring total knee replacement (TKR) at a younger age than the general population. TKR in this unique group of patients is a technically demanding procedure owing to the deformity, flexion contracture, generalised hypotonia and ligamentous laxity. We retrospectively reviewed the outcome of 11 TKRs performed in eight patients with skeletal dysplasia at our institution using the Stanmore Modular Individualised Lower Extremity System (SMILES) custom-made rotating-hinge TKR. There were three men and five women with mean age of 57 years (41 to 79). Patients were followed clinically and radiologically for a mean of seven years (3 to 11.5). The mean Knee Society clinical and function scores improved from 24 (14 to 36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to 80) and 50 (22 to 74), respectively, at final follow-up. Four complications were recorded, including a patellar fracture following a fall, a tibial peri-prosthetic fracture, persistent anterior knee pain, and aseptic loosening of a femoral component requiring revision. Our results demonstrate that custom primary rotating-hinge TKR in patients with skeletal dysplasia is effective at relieving pain, with a satisfactory range of movement and improved function. It compensates for bony deformity and ligament deficiency and reduces the likelihood of corrective osteotomy. Patellofemoral joint complications are frequent and functional outcome is worse than with primary TKR in the general population.