Correlation between ultrasonic and radiographic imaging of developmental dysplasia of the hip.

PURPOSE: The correlation between the degree of developmental hip dysplasia (DDH) measured on ultrasound images compared with that measured on radiographs is not clear. Most studies have compared ultrasonography (US) and radiographic images made at different times of follow-up. In this study the correlation between US images and radiographs of the hip made on the same day was evaluated. METHODS: US images and radiographs of both hips of 74 infants, who were treated for stable DDH, were reviewed in a retrospective study. Only infants who had an US examination and a radiograph on the same day were included. RESULTS: The correlation between α-angle of Graf and femoral head coverage on US was strong (p ≤ 0.0001). Weak correlations were found between the acetabular index of Tönnis on radiographs and α-angle of Graf on US (p = 0.049) and between acetabular index of Tönnis on radiographs and femoral head coverage of Morin on US (p = 0.100). CONCLUSION: This study reports on the correlation between US and radiographic imaging outcomes, both made on the same day in patients for treatment and follow-up of DDH. LEVEL OF EVIDENCE: IV.

Assessment of the relationship between joint laxity and migration of the hip in children with Down syndrome.

PURPOSE: The aim of this cross-sectional cohort study is to describe the incidence of joint laxity and the correlation between joint laxity and radiological migration of the hip in children with Down syndrome. METHODS: Sixty-five children (2-19 years) with Down's syndrome were examined for joint laxity. For each subject, laxity scores for joints were carried out with the Bulbena method. Plane pelvic radiographs were used to determine the migration of the hip, according to Reimer's migration index. RESULTS: In this study, 26 out of 65 children with Down's syndrome (40 %) were diagnosed with general joint laxity. On the radiographs of the hips we found a mean Reimer's Migration Index of 5.2 % for all the subjects. Children with general joint laxity showed a lower Reimer's Migration Index (2.1 %). No significant correlation was found between general joint laxity and migration of the hip. CONCLUSIONS: This study showed no relationship between joint laxity and migration of the hip in children with Down's syndrome. This implicates that we were not able to prove that joint laxity is the major factor in developing hip migration in children with Down's syndrome.

Decrease in outpatient department visits and operative interventions due to bisphosphonates in children with osteogenesis imperfecta.

INTRODUCTION: Bisphosphonates are currently the medical treatment most often used in children with osteogenesis imperfecta (OI). The purpose of this retrospective pre-post study was to evaluate the efficacy of treatment with bisphosphonates. We measured the effect by evaluating the number of outpatient department consultations and operative interventions before and after treatment with bisphosphonates in children with OI. METHODS AND MATERIALS: Outpatient department consultation and operative intervention frequencies before and after treatment with bisphosphonates were registered. Children who had at least 2 years of medical records before treatment and at least 2 years after treatment were used in this study. RESULTS: Of 118 children who were treated with bisphosphonates, 51 (23 boys and 28 girls) fulfilled the inclusion criteria. Statistical analysis revealed a significant decrease in outpatient department consultations (P < 0.000) and operative intervention (P < 0.003) before and after bisphosphonate treatment. CONCLUSION: The pre-post design of our study shows a significant reduction of the number of outpatient department consultations and operative interventions in patients with OI after treatment with bisphosphonates.

Spinal fusion in children with spina bifida: influence on ambulation level and functional abilities.

The aim of this study was to determine the influence of spinal fusion on ambulation and functional abilities in children with spina bifida for whom early mobilization was stimulated. Ten children (three males and seven females) with myelomeningocele were prospectively followed. Their mean age at operation was 9.3 years (standard deviation (SD): 2.4). Spinal curvature was measured according to Cobb. Pelvic obliquity and trunk decompensation were measured as well. The ambulation level was scored according to Hoffer, and functional abilities, as well as the amount of caregiver assistance, were documented using the Pediatric Evaluation of Disability Inventory. All patients were assessed before surgery and three times after surgery, with a total follow-up duration of 18 months after surgery. After spinal fusion, magnitude of primary curvature decreased significantly (p=0.002). Pelvic obliquity and trunk decompensation did not change. In spite of less immobilization as compared with other reported experiences, ambulation became difficult in three out of four patients who had been able to ambulate prior to surgery. Functional abilities and amount of caregiver assistance concerning self-care (especially regarding dressing upper and lower body, and self-catheterization) and mobility (especially regarding transfers) showed a nonsignificant trend to deterioration within the first 6 months after surgery, but recovered afterwards. From pre-surgery to 18 months after surgery, functional skills on self-care showed borderline improvement (p=0.07), whereas mobility did not (p=0.2). Mean scores on caregiver assistance improved significantly on self-care (p=0.03), and borderline on mobility (p=0.06), meaning that less caregiver assistance was needed compared with pre-surgery. The complication rate was high (80%). In conclusion, within the first 6 months after spinal fusion, more caregiver assistance is needed in self-care and mobility. It takes about 12 months to recover to pre-surgery level, while small improvement is seen afterwards. After spinal fusion, ambulation often becomes difficult, especially in exercise walkers. These findings are important for health-care professionals, in order to inform and prepare the patients and their parents properly for a planned spinal fusion.

Osteogenesis imperfecta in childhood: effects of spondylodesis on functional ability, ambulation and perceived competence.

We studied the effects of spondylodesis on spinal curvature, functional outcome, level of ambulation and perceived competence in 11 children with osteogenesis imperfecta (OI). Mean age at surgical intervention was 13.1 years (SD 2.5 years) and follow-up amounted to 3.4 years (SD 2.3 years). Spinal curvature was measured according to Cobb. The level of ambulation was scored according to the modified criteria of Bleck. Functional abilities and the amount of parental assistance were scored using the Dutch version of the Pediatric Evaluation of Disability Inventory (PEDI). Perceived competence was measured using the Harter Self-Perception Profile for Children. The amount of fatigue, spinal pain and presence of subjective dyspnea were scored with a visual analog scale. The median progression per year before spondylodesis was 6.1 degrees (interquartile range 2.9 degrees -12.9 degrees ) and after the spondylodesis it was 5.0 degrees (interquartile range 1.6 degrees -11.0 degrees ). No significant progression or regression in the level of ambulation was found. Perceived competence improved slightly. In the total score of the perceived competence, a borderline significant increase was found ( P-value 0.068). We concluded that spinal fusion in children with OI does not materially influence functional ability and level of ambulation. Self-perceived competence seemed to improve after surgery. The amount of pain, fatigue and subjective dyspnea seemed to diminish after spinal surgery. Progression of scoliosis proceeded, as did development of spinal curvature at the junction of the spondylodesis. Therefore, oral or intravenous bisphosphonates before and after spinal surgery should be considered.

Osteogenesis imperfecta in childhood: MR imaging of basilar impression.

OBJECTIVE: To determine on radiographs the presence of Basilar Impression (BI) in children with Osteogenesis Imperfecta (OI). To confirm this sign and altered geometrical relationships of the craniocervical junction in course of time with magnetic resonance imaging (MRI). METHODS AND PATIENTS: In a cohort study of 130 patients with OI (OI type I: 85; OI type III: 21; OI type IV: 24) lateral radiographs of the skull and cervical spine were made in a standardised way. MRI scans were performed when BI was suspected based upon protrusion of the odontoid above Chamberlain's line. Intracranial abnormalities as well as the basal angle were described. Neurological examination was performed in patients with conclusive BI at MRI-scan. RESULTS AND DISCUSSION: In eight patients BI could be confirmed by MRI-scan. None of the children had or developed in time neurological symptoms or signs. Follow up of BI by MRI scans was done in seven patients (mean: 5 years; range: 2-6 years). No alteration of intracranial findings were seen at subsequent investigation, although in one child Chamberlain's line increased from 8 (first MRI) to 15 mm (last MRI). BI can be diagnosed by radiographs but in the extreme osteoporotic bone and altered anatomy of the craniocervical junction of children with OI MRI is preferable. As intracranial pathology can be demonstrated by MRI, also a relation can be laid to possible neurological symptoms and signs at clinical examination. CONCLUSION: In our cohort study no alteration of the intracranial contents was seen at subsequent MRI scans. Although anatomic deformations exist in BI, no neurological symptoms or signs were present in our study and no operative reconstruction had to be performed. Periodical MRI-scan has not been of influence on the clinical decision making process. At the moment we perform a MRI-scan if BI is suspected at lateral skull radiographs. The MRI images serve as reference findings to anticipate on possible future symptoms and signs of neurological deficit.

Hemi-epiphysiodesis for unclassified congenital scoliosis: immediate results and mid-term follow-up.

Complex spinal anomalies involved in congenital scoliosis consist of a jumble of vertebral defects. Progressive scoliotic curves are frequently encountered in these patients. We evaluated the results of hemi-epiphysiodesis for this patient population. A series of ten patients with unclassified scoliosis, involving multiple hemivertebrae and/or bars, was reviewed retrospectively. Hemi-epiphysiodesis was the primary treatment in all patients. Mortality, complications and wound healing problems did not occur. The average Cobb angle for all patients changed from 54 degrees (range: 40 degrees -74 degrees), preoperatively, to 58 degrees (range: 30 degrees -104 degrees), postoperatively. Applying the criterion of a minimum change of 20 degrees, to take into account measurement variability, an epiphysiodesis effect was achieved in two procedures, progression was arrested in six procedures and failure occurred in two procedures. The mean rate of Cobb angle change per annum decreased from 2.9 degrees (range: -35 degrees to 14 degrees), preoperatively, to 2.4 degrees (range: -4 degrees to 13 degrees), following surgery. Repeat surgery was necessitated by coexisting progressive kyphosis and pseudoarthrosis in one patient, and involved extension of primary epiphysiodesis in two patients. A mean intervention-free period of 58 months was established. These results suggest that hemi-epiphysiodesis stabilized the unclassified congenital scoliosis. Being under 5 years of age, having a thoracolumbar curve location, and the absence of coexisting kyphosis were found to be associated with a more favorable outcome.