14 research outputs found

    Spinal and pelvic corrections in a patient with spondylocostal dysplasia syndrome and hemimyelomeningocele

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    Congenital malformation complex of the spine and the spinal cord can be a syndromic entity rather than a symptom complex. The spinal cord lesion is usually bilaterally symmetrical, but, there are occasional cases with one or more hemivertebrae, often associated with a central bony spur splitting the cord (diastematomyelia), in which one leg is virtually normal while the other is severely paralysed. Hemimyelomeningocele over the lumbar area may be associated with extensive spine malsegmentation compatible with the diagnosis of spondylocostal dysplasia syndrome. In this report, we present a 3-year-old girl who underwent neurological evaluation and spinal imaging studies for extensive spine malsegmentation compatible with spondylocostal dysostosis syndrome associated with hemimyelomeningocele. She had a series of corrective orthopaedic interventions to reconstruct her pelvic girdle and spine deformities, with a satisfactory outcome

    Extraspinal ossifications after implantation of vertical expandable prosthetic titanium ribs (VEPTRs).

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    INTRODUCTION Though developed for thoracic insufficiency syndrome, the spinal growth-stimulating potential and the ease of placement of vertical expandable titanium ribs (VEPTRs) has resulted in their widespread use for early-onset spine deformity. Observation of implant-related ossifications warrants further assessment, since they may be detrimental to the function-preserving non-fusion strategy. PATIENTS AND METHODS Radiographs (obtained pre and post index procedure, and at 4-year follow-up) and the records of 65 VEPTR patients from four paediatric spine centres were analysed. Ossifications were classified as type I (at anchor points), type II (along the central part) or type III (re-ossification after thoracostomy). RESULTS The average age at the index procedure was 6.5 years (min 1, max 13.7). The most prevalent spine problem was congenital scoliosis (37) with rib fusions (34), followed by neuromuscular and syndromic deformities (13 and 8, respectively). Idiopathic and secondary scoliosis (e.g. after thoracotomy) were less frequent (3 and 4, respectively). Forty-two of the 65 (65 %) patients showed ossifications, half of which were around the anchors. Forty-five percent (15/33) without pre-existing rib fusions developed a type II ossification along the implant. Re-ossifications of thoracostomies were less frequent (5/34, 15 %). The occurrence of ossifications was not associated with patient-specific factors. CONCLUSIONS Implant-related ossifications around VEPTR are common. In contrast to harmless bone formation around anchors, ossifications around the telescopic part and the rod section are troublesome in view of their possible negative impact on chest cage compliance and spinal mobility. This potential side effect needs to be considered during implant selection, particularly in patients with originally normal thoracic and spinal anatomy

    Electromagnetic navigation reduces radiation exposure for retrograde drilling in osteochondrosis dissecans of the talus

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    Background!#!Retrograde drilling in osteochondrosis dissecans (OCD) is a widely used surgical intervention. A radiation-free electromagnetic navigation system (ENS)-based method was compared with the standard freehand fluoroscopic (SFF) method regarding clinical applicability.!##!Methods!#!We performed a clinical cohort study at a department of Orthopaedics in a Level 1 children's hospital with 40 patients (20 SFF and 20 ENS). Retrograde drilling of the talar dome was used in patients with unstable medial OCD (MRI stage 2 according to Hepple's revised classification; stage 2 according to the International Cartilage Repair Society). The outcome measurements were: (a) Intraoperative fluoroscopy exposure and length of surgery and (b) Postoperative serial follow-up MRIs every 6 months.!##!Results!#!22 female and 18 male patients aged 13.8 ± 1.6 years (range: 11-17 years) were included. Using the ENS technique, length of surgery was significantly reduced to 20.2 ± 6.4 min compared to 36.1 ± 11.8 min (p < 0.01) for the SFF technique. The average x-ray radiation time for the SFF technique was 23.5 ± 13.5 sec and 1.9 ± 1.7 sec for the ENS technique (p < 0.01). Radiation exposure was significantly reduced from 44.6 ± 19.7 mSv (SFF technique) to 5.6 ± 2.8 mSv (ENS technique) (p < 0.01). Intraoperative perforation of cartilage occurred once in the SFF group. Correct placement of the drilling channel was verified in all patients on follow-up MRI after six months and a timely healing was seen after two years.!##!Conclusions!#!The ENS method provides for a significant reduction in length of surgery and radiation exposure. ENS was without intraoperative cartilage perforation. The clinical and radiological follow-up parameters are comparable for SFF- and ENS-guided retrograde drilling.!##!Trial registration!#!WF - 085/20, 05/2020 'retrospectively registered' https://www.aerztekammer-hamburg.org/ethik_kommission.html

    The Use of Bovine Xenogeneic Bone Graft for Dega Pelvic Osteotomy in Children with Hip Dysplasia: A Retrospective Study of 147 Treated Hips

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    Backgrounds: Dega pelvic osteotomy is commonly used to correct acetabular dysplasia in children with open triradiate cartilage. The use of bovine xenogeneic bone graft (Tutobone®) for Dega osteotomy has not been reported so far. This study aimed to determine the clinical and radiological outcome in a large series of children with hip dysplasia who were treated by Dega osteotomy using a bovine xenogeneic block for stabilisation. Methods: A retrospective, single-centre study was conducted including 101 patients (147 hips) with different underlying diseases. The acetabular angle of Hilgenreiner (AA) and the lateral center-edge angle (LCA) were analysed to quantify the correction of acetabular indices. Graft incorporation was assessed using the Goldberg scoring system. Results: the mean preoperative AA improved from 28.1 (SD: 6.7) to 14.7 (SD: 5.1) after surgery (p < 0.001). The mean preoperative LCA improved from 9.9 (SD: 6.7) to 21.8 (SD: 6.8) postoperatively (p < 0.001). Both indices remained stable at the one-year follow-up examination. Graft incorporation was excellent with a mean Goldberg score of 6.6. Heterotopic ossification occurred in one hip without clinical relevance. Graft-related complications were not noted. Conclusions: Dega osteotomy using Tutobone® is safe and effective in the treatment of acetabular dysplasia in children independent of the underlying disease

    Recombinant Human Bone Morphogenetic Protein-2 (rhBMP-2) for the Treatment of Nonunion of the Femur in Children and Adolescents: A Retrospective Analysis

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    Background. The aim of this study was to examine clinical and radiographic healing after rhBMP-2 application in children and adolescents presenting with nonunion of the femur and to investigate the safety of rhBMP-2 use in these cases. Materials and Methods. We reviewed the medical records of five patients with a mean age of 11 years (5.4 to 16.2) with nonunion of the femur who were treated with rhBMP-2 and internal fixation using a locking plate at a single institution. Particular attention was paid to identify all adverse events that may be due to rhBMP-2 use. Results. Union occurred in four of five patients at a mean of 12.1 months (7.9 to 18.9). The locking plates were removed after a mean of 16 months (11 to 23). One patient had nonunion due to deep infection. After a mean follow-up of 62.5 months (17 to 100), union was still evident in all four patients and they were fully weight-bearing without pain. Discussion. In this retrospective study, rhBMP-2 combined with a locking plate has been used successfully to treat children and adolescents with nonunion of the femur in four of five cases. One major complication was thought to be possibly related to its use

    Hip pathologies in mucopolysaccharidosis type III

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    Background!#!Mucopolysaccharidosis type III (MPS III) comprises a group of rare lysosomal storage diseases. Although musculoskeletal symptoms are less pronounced than in other MPS subtypes, pathologies of hip and spine have been reported in MPS III patients. The purpose of this study was to describe hip pathologies and influencing parameters in MPS III patients.!##!Methods!#!A retrospective chart review was performed for 101 MPS III patients. Thirty-two patients met the inclusion criteria of enzymatically or genetically confirmed diagnosis and anteroposterior radiograph of the hips. Modified Ficat classification, Wiberg's center-edge angle, and Reimer's migration percentage were measured.!##!Results!#!The mean age at data assessment was 11.0 years (SD 5.7). Osteonecrosis of the femoral head was observed in 17/32 patients. No statistically significant association was found between these changes and age, sex, or MPS III subtype. Patients with a severe phenotype showed significantly higher rates of osteonecrosis (14/17) than patients with an intermediate phenotype. Hip dysplasia was present in 9/32 patients and was significantly associated with osteonecrosis of the femoral head (p = 0.04).!##!Conclusions!#!The present study demonstrates a high rate of hip pathologies in MPS III patients. Hip dysplasia and severe phenotype were significantly correlated with osteonecrosis of the femoral head. Therefore, radiographs of the hips are highly recommended in baseline and follow-up assessments of MPS III patients.!##!Trial registration!#!Retrospectively registered

    Selective posterior thoracic fusion by means of direct vertebral derotation in adolescent idiopathic scoliosis: effects on the sagittal alignment

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    The objectives of this retrospective study were to evaluate the effect of direct vertebral derotation on the sagittal alignment of the spine after selective posterior thoracic fusion for Lenke Type I adolescent idiopathic scoliosis (AIS). Preservation of the sagittal alignment has become critical in the management of spinal deformity. Better coronal and rotational corrections in posterior selective thoracic fusion for AIS have been reported with direct vertebral derotation as compared with the simple rod rotation technique. A greater lordogenic effect has been anticipated with direct vertebral derotation; however, data comparing those two techniques in terms of correction in the sagittal plane are still lacking. Standing full-spine PA and standard lateral serial X-rays of a total of 30 consecutive patients with adolescent idiopathic scoliosis treated between 2002 and 2008 at a single institution were evaluated. All the patients had Lenke Type I curves and underwent selective posterior thoracic fusion with pedicle screw instrumentation. Patients who were treated with additional osteotomies and concave or convex thoracoplasty or concomitant anterior releases were excluded. Minimum follow-up period was 24 months. Preoperative and postoperative coronal and sagittal spinal alignments in both the groups were compared. In 13 patients, the correction was achieved by means of a simple rod rotation (SRR). In 17 patients, the technique of direct vertebral derotation (DVD) was used. Scoliosis correction averaged 67 and 69%, respectively, and was similar in both groups (p > 0.05). Thoracic kyphosis and lumbar lordosis remained unchanged in the SRR group (p > 0.1). In the direct vertebral derotation group, a significant decrease of both thoracic kyphosis and lumbar lordosis of 8.1° and 11.8°, respectively, was observed (p < 0.0001). Global sagittal balance remained within normal limits in all the patients at the latest follow-up. Decrease in thoracic kyphosis and lumbar lordosis should be taken into account when using direct vertebral derotation for selective posterior thoracic fusion in AIS. In order to preserve sagittal alignment in these patients, ultra hard rods or maneuvers that pull posteriorly the concave side of the spine, thus avoiding the application of additional flattening forces should be considered
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