BackgroundPatients with adolescent idiopathic scoliosis (AIS) are usually investigated by serial imaging studies during the course of treatment, some imaging involves ionizing radiation, and the radiation doses are cumulative. Few studies have addressed the correlation of spinal deformity captured by these different imaging modalities, for which patient positioning are different. To the best of our knowledge, this is the first study to compare the coronal, axial, and sagittal morphology of the scoliotic spine in three different body positions (upright, prone, and supine) and between three different imaging modalities (X-ray, CT, and MRI).MethodsSixty-two AIS patients scheduled for scoliosis surgery, and having undergone standard pre-operative work-up, were included. This work-up included upright full-spine radiographs, supine bending radiographs, supine MRI, and prone CT as is the routine in one of our institutions. In all three positions, Cobb angles, thoracic kyphosis (TK), lumbar lordosis (LL), and vertebral rotation were determined. The relationship among three positions (upright X-ray, prone CT, and supine MRI) was investigated according to the Bland-Altman test, whereas the correlation was described by the intraclass correlation coefficient (ICC).ResultsThoracic and lumbar Cobb angles correlated significantly between conventional radiographs (68° ± 15° and 44° ± 17°), prone CT (54° ± 15° and 33° ± 15°), and supine MRI (57° ± 14° and 35° ± 16°; ICC ≥0.96; P < 0.001). The thoracic and lumbar apical vertebral rotation showed a good correlation among three positions (upright, 22° ± 12° and 11° ± 13°; prone, 20° ± 9° and 8° ± 11°; supine, 16° ± 11° and 6° ± 14°; ICC ≥0.82; P < 0.001). The TK and LL correlated well among three different positions (TK 26° ± 11°, 22° ± 12°, and 17° ± 10°; P ≤ 0.004; LL 49° ± 12°, 45° ± 11°, and 44° ± 12°; P < 0.006; ICC 0.87 and 0.85).ConclusionsAlthough there is a generalized underestimation of morphological parameters of the scoliotic deformity in the supine and prone positions as compared to the upright position, a significant correlation of these parameters is still evident among different body positions by different imaging modalities. Findings of this study suggest that severity of scoliotic deformity in AIS patients can be largely represented by different imaging modalities despite the difference in body positioning.

PurposeThe management of proximal femoral deformity in fibrous dysplasia (FD) is a challenge to the orthopaedic surgeon. The purpose of this study was to analyze the various presentations of FD of proximal femur and the results of the various treatment modalities for the same.MethodsThis is a retrospective cohort study of 23 patients (24 femora) with FD who underwent surgery for the proximal femur. The study sample included 14 males, nine females. Ten patients had a monostotic disease, eight patients had polyostotic disease, and five patients had McCune-Albright syndrome.ResultsGroup 1: shepherd crook deformity—included five patients who underwent femoral neck osteotomy. Four patients had intramedullary (IM) nailing with neck cross-pinning and all patients showed union. One patient was stabilized with external fixation, which failed. Group 2: nine patients (ten femora) presented with frank pathological fracture. Nine underwent fixation with IM nailing, one with locking plate and screws. Three patients had to undergo more than one procedure and all fractures showed good union. Group 3: nine patients who presented with bone cyst and pain. All patients underwent biopsy; four of them had curettage with bone graft.ConclusionShepherd crook deformity can be treated by a well-planned osteotomy and fixation with intramedullary implants with neck cross-pinning. Frank pathological fractures fixation with an intramedullary nail has excellent results even if not accompanied by resolution of the fibrodysplastic lesion. More than one procedure may be required. External fixation is not an optimal choice for fixation of femoral osteotomies in FD.

4.

Level 4.

The 22q11.2 Deletion Syndrome (22q11.2DS) is the most common microdeletion syndrome with an estimated prevalence of 1:4,000 live births. 22q11.2DS is known to have wide phenotypic variability, including orthopaedic manifestations. The purpose of this systematic review is to increase the awareness of orthopaedic manifestations associated with 22q11.2DS. This systematic review was performed according to the PRISMA Guidelines. Original epidemiological studies on the prevalence of orthopaedic manifestations within 22q11.2DS were systematically searched for in PubMed and EMBASE. The included articles were scored according to a risk-of-bias tool, a best-evidence synthesis was performed and the prevalence data was extracted. Sixty-nine published manuscripts described 58 orthopaedic manifestations in a total of 6,055 patients. The prevalence of at least one cervical or occipital anomaly is 90.5-100% (strong evidence). Fourteen studies (n = 2,264) revealed moderate evidence for a wide scoliosis prevalence of 0.6-60%. Two studies demonstrated that 5-6.4% of all 22q11.2DS patients required surgical scoliosis correction. Fifteen studies (n = 2,115) reported a 1.1-13.3% prevalence of clubfoot with moderate evidence. Other reported orthopaedic manifestations are patellar dislocation (10-20%), juvenile rheumatic arthritis (3.75%), impaired growth and skeletal anomalies like polydactyly (1.0-3.7%), syndactyly (11-11.8%), butterfly vertebrae (11.1%) and 13 ribs (2-19%). Orthopaedic findings are important manifestations of the 22q11.2DS, both in bringing patients to diagnostic attention and in requiring surveillance and appropriate intervention. Data on these manifestations are scattered and incomprehensive. Routinely screening for cervical anomalies, scoliosis, and upper and lower limb malformations is recommended in this vulnerable group of patients.