As fast as an X-ray: real-time magnetic resonance imaging for diagnosis of idiopathic scoliosis in children and adolescents.

BACKGROUND: Idiopathic scoliosis is common in adolescence. Due to the rapid growth of the spine, it must be monitored closely with radiographs to ensure timely intervention when therapy is needed. As these radiographs continue into young adulthood, patients are repeatedly exposed to ionizing radiation. OBJECTIVE: This study aimed to investigate whether real-time magnetic resonance imaging (MRI) is equivalent to conventional radiography in juvenile idiopathic scoliosis for determining curvature, rotation and the Risser stage. Additionally, the time requirement should be quantified. MATERIALS AND METHODS: Children with idiopathic scoliosis who had postero-anterior whole-spine radiography for clinical indications were included in this prospective study. A real-time spine MRI was performed at 3 tesla in the supine position, capturing images in both the coronal and sagittal planes. The scoliosis was assessed using Cobb angle, rotation was evaluated based on Nash and Moe criteria, and the Risser stage was determined for each modality. The correlations between modalities and a correction factor for the Cobb angle between the standing and supine position were calculated. RESULTS: A total of 33 children (aged 5-17 years), who met the inclusion criteria, were recruited. The Cobb angle (R2 = 0.972; P < 0.01) was positively correlated with a correction factor of 1.07 between modalities. Additionally, the degree of rotation (R2 = 0.92; P < 0.01) and the Risser stage (R2 = 0.93; P < 0.01) demonstrated a strong correlation. CONCLUSION: Real-time MRI is equivalent to conventional radiography in determining baseline parameters. Furthermore, it is radiation-free and less time-consuming.

Congenital cervical spine malformation due to bi-allelic RIPPLY2 variants in spondylocostal dysostosis type 6.

RIPPLY2 is an essential part of the formation of somite patterning during embryogenesis and in establishment of rostro-caudal polarity. Here, we describe three individuals from two families with compound-heterozygous variants in RIPPLY2 (NM_001009994.2): c.238A > T, p.(Arg80*) and c.240-4 T > G, p.(?), in two 15 and 20-year-old sisters, and a homozygous nonsense variant, c.238A > T, p.(Arg80*), in an 8 year old boy. All patients had multiple vertebral body malformations in the cervical and thoracic region, small or absent rib involvement, myelopathies, and common clinical features of SCDO6 including scoliosis, mild facial asymmetry, spinal spasticity and hemivertebrae. The nonsense variant can be classified as likely pathogenic based on the ACMG criteria while the splice variants must be classified as a variant of unknown significance. With this report on two further families, we confirm RIPPLY2 as the gene for SCDO6 and broaden the phenotype by adding myelopathy with or without spinal canal stenosis and spinal spasticity to the symptom spectrum.

[Etiology, epidemiology, prognosis and biomechanical principles of neuromuscular scoliosis]. / Ätiologie, Epidemiologie, Prognose und biomechanische Besonderheiten neuromuskulärer Skoliosen.

Deformity due to neuromuscular disease is often progressive and associated with reduced vital capacity. In general, all treatment should be performed in specialized centers, since invasive measures are associated with an increased morbidity compared to adolescent scoliosis. Derived from the etiology and the resulting biomechanical basis (characteristics), important aspects and considerations arise for all healthcare institutions from the examination interval to the duration of conservative therapy and initiation of surgical therapy. Proper monitoring and assessment are key components to identify curve progression and to achieve preservation or improvement of basic functions.

[Bilateral idiopathic chondrolysis of the hips - A case report of an adolescent patient]. / Idiopathische Chondrolyse beider Hüftgelenke ­ Fallbericht bei einer adoleszenten Patientin.

The diagnosis and treatment of idiopathic chondrolysis of the hips are described in the case of a 12-year-old girl. The patient presented with intermittent pain and extreme dysfunction of both hips. After clinical examination and pelvic X­ray, MRI showed the typical changes of idiopathic chondrolysis. Purely conservative treatment with systematic physiotherapy and regular application of a non-steroidal anti-inflammatory drug produced a good clinical result.

Treatment of genu valgum in children by means of temporary hemiepiphysiodesis using eight-plates: short-term findings.

BACKGROUND: Idiopathic genu valgum is a frequently diagnosed growth disorder in adolescence. Whenever the possibilities of conservative therapy have been exhausted, leg straightening by means of hemiepiphysiodesis has become the standard form of treatment. Because of their flexible screw-plate connection, eight-Plates have been reported in the literature to lead to lower complications regarding implant loosening and fracture compared to other implants. The aim of this retrospective survey was to analyse our own patient population who were treated for genu valgum by means of temporary hemiepiphysiodesis near the knee using eight-Plates to modulate growth. METHODS: Between July 2007 and July 2015, 198 eight-Plates were implanted near the knee in 132 children suffering from genu valgum to modulate growth. Depending on the deformity analysis, an eight-Plate was implanted on the distal medial femur and/or the proximal medial tibia. By December 2015, they had been removed from 105 patients. The etiology of genu valgum was mainly idiopathic or associated withobesity. Evaluation was carried out clinically and radiologically (whole-leg X-ray in standing) including determination of the joint angles. RESULTS: The median follow-up period was about 46 months (12-102 months). The median age at implantation was 12.7 +/-6.76 years. Of the 105 patients, 45.7% (n = 48) were girls. The eight-Plates remained in place for a median period of 13 +/-1.76 months. Irrespective of the location of hemiepiphysiodesis, the intermalleolar distance was corrected to a median of 0 +/-2.1 cm while the anatomical femorotibial angle was corrected by on average 9 +/-2.7 °Mechanical lateral distal femoral angle changed an average 7 +/- 7.72 degrees. Medial proximal tibial angle changed an average 4 +/- 6.02 degrees. Complications necessitating surgery occurred in 2.8% of cases (1 wound infection, 3 corrective osteotomies following overcorrection). CONCLUSION: Temporary hemiepiphysiodesis using eight-Plates is a gentle, simple and effective procedure used to treat genu valgum by modulating growth. Slight overcorrection is desirable due to the rebound phenomenon, especially in young patients with high growth potential and risk groups such as obese children. In adolescents with only low growth potential (older than 14 years), owing to the low correction potential, the indication should be strictly reviewed and the possible failure of therapy should be discussed with the patient. No differences were observed regarding the location of the implanted eight-Plates (femoral or tibial).

Treatment of Severe Kyphoscoliosis in Children with Mucopolysaccharidosis Type I (Pfaundler-Hurler Syndrome) Using the Growing Rod Technique: A Case Series with Mid-Term Results.

BACKGROUND: Patients with mucopolysaccharidosis type I (MPS I) have a good life expectancy due to early therapeutic options, such as stem cell therapy. Stem cell therapy can prevent the progression of some skeletal malformations. In contrast, the progression of thoracolumbar kyphoscoliosis, genua vara, and hip dysplasia cannot be influenced. We present 3 cases of children with MPS I with thoracolumbar kyphosis/kyphoscoliosis treated with a growing rod system. CASE DESCRIPTION: The medical records and radiologic imaging of 3 children with a diagnosis of MPS I and kyphosis/kyphoscoliosis of the lumbar spine treated between 2007 and 2019 were retrospectively analyzed. Two children presented with a kyphoscoliosis, and 1 child had a combination of severe anterolisthesis and kyphoscoliosis. Surgery to correct the kyphosis and dorsal stabilization was performed in all patients after exhausted conservative treatment. There were no neurologic complications. Postoperative treatment and aftercare included a corset for 4 months and physical therapy. In all 3 patients, distraction surgery of the lumbar stabilization was done twice at a mean interval of 1 year. CONCLUSIONS: If conservative treatment fails and surgery is necessary, an individual approach is needed. Dorsal stabilization with pedicle screws using a growing rod technique is an option for the correction of thoracolumbar/lumbar kyphosis in children with MPS I. However, fusion should be prevented initially or should be kept as short as possible. We achieved acceptable correction of the spinal deformity using the growing rod technique. Finally, surgery with correction and fusion is necessary after exhausted correction potential.