Progressive Thoracic Scoliosis Research Articles

Assessment of malalignment at early stage in adolescent idiopathic scoliosis: a longitudinal cohort study.

Our objective was to assess abnormalities of the odontoid-hip axis (OD-HA) angle in a mild scoliotic population to determine whether screening for malalignment would help predict the distinction between progressive and stable adolescent idiopathic scoliosis (AIS) at early stage. All patients (non-scoliotic and AIS) underwent a biplanar X-ray between 2013 and 2020. In AIS, inclusion criteria were Cobb angle between 10° and 25°; Risser sign lower than 3; age higher than 10years; and no previous treatment. A 3D spine reconstruction was performed, and the OD-HA was computed automatically. A reference corridor for OD-HA values in non-scoliotic subjects was calculated as the range [5th-95th percentiles]. A severity index, helping to distinguish stable and progressive AIS, was calculated and weighted according to the OD-HA value. Eighty-three non-scoliotic and 205 AIS were included. The mean coronal and sagittal OD-HA angles in the non-scoliotic group were 0.2° and -2.5°, whereas in AIS values were 0.3° and -0.8°, respectively. For coronal and sagittal OD-HA, 27.5% and 26.8% of AIS were outside the reference corridor compared with 10.8% in non-scoliotic (OR = 3.1 and 3). Adding to the severity index a weighting factor based on coronal OD-HA, for thoracic scoliosis, improved the positive predictive value by 9% and the specificity by 13%. Analysis of OD-HA suggests that AIS patients are almost three times more likely to have malalignment compared with a non-scoliotic population. Furthermore, analysis of coronal OD-HA is promising to help the clinician distinguish between stable and progressive thoracic scoliosis.

Less-Invasive Approach to Early-Onset Scoliosis—Surgical Technique for Magnetically Controlled Growing Rod (MCGR) Based on Treatment of 2-Year-Old Child with Severe Scoliosis

Spinal deformities in children can be caused by various etiologies, such as congenital, syndromic, neuromuscular, or idiopathic. Early-onset scoliosis (EOS) is diagnosed before the age of ten years, and when the curvature continues to progress and exceeds a Cobb angle of 60-65 degrees, surgical treatment should be considered. Initial minimally invasive surgery and the implantation of magnetically controlled growing rods (MCGRs) allows for the noninvasive distraction of the spine, growing, and avoids multiple operations associated with the classic distractions of standard growing rods. A 2-year-old girl was admitted to our clinic with rapidly progressive thoracic scoliosis. The major curve of the thoracic spine Cobb angle was 122° at 30 months. No congenital deformities were detected. The surgical technique was the less-invasive percutaneous and subfascial implantation of MCGRs, without long incisions on the back and the non-invasive ambulatory lengthening of her spine over the next 4 years. MCGR is a safe procedure for EOS patients. It is extremely effective at correcting spinal deformity; controlling the growth and curvature of the spine as the child develops during growth; reducing the number of hospitalizations and anesthesia; and minimizing the physical and mental burden of young patients, parents, and their families.

Scoliosis in Carey-Fineman-Ziter Syndrome: Clinical Course, Association with Pierre Robin Sequence and Treatment

Objective − We present a female patient with Carey-Fineman-Ziter Syndrome (CFZS) and 4 patients with Pierre Robin sequence who developed a progressive thoracic scoliosis in early child life. Material and Methods − We reviewed the medical notes and spinal radiographs of these 5 patients who were treated for progressive scoliosis, underwent a posterior spinal fusion and were followed in our service to skeletal maturity. Results − We describe the patients’ response to treatment including bracing which was unsuccessful, and scoliosis correction. We compared the evolution of scoliosis and surgical outcome between the patient with CFZS and our 4 patients with Pierre-Robin sequence and hypotonia who were managed for scoliosis under our care in order to understand possible aetiological relations between the underlying myopathic condition and the development of scoliosis. Conclusion − Scoliosis in children with CFZS and Pierre Robin sequence occurred in early childhood with rapid progression around puberty and poor effectiveness of bracing. We suggest that generalised hypotonia is a likely causative factor in the development and rapid deterioration of scoliosis in these 2 conditions. Spinal surgery through a posterior spinal fusion produced excellent deformity correction and functional outcomes that were maintained through to skeletal maturity in all 5 patients.

Scoliosis associated with HGPPS syndrome: What do we know?

Horizontal gaze palsy with progressive scoliosis (HGPPS) syndrome is a rare genetic abnormality causing cranial dysinnervation manifesting as absence of conjugate lateral eye movements, nystagmus, and scoliosis. While the genetics, imaging abnormalities, and ocular manifestations have been well described in literature, the spinal deformity has not been addressed adequately. An 11-year-old girl presented with progressive thoracic scoliosis who on evaluation was detected to have all the features of HGPPS syndrome. The patient underwent posterior correction of scoliosis uneventfully. A thorough literature search was performed to understand the descriptors of spinal deformity and its surgery in HGPPS syndrome. The spinal deformity in HGPPS resembles adolescent idiopathic scoliosis. Rapidly progressive right thoracic scoliosis was the usual pattern. Surgical and anesthetic considerations during surgery are similar to idiopathic scoliosis. These children tolerate surgery and anesthesia well. Neuromonitoring changes during surgery need to be interpreted correctly in the background of uncrossed sensory and motor tracts.

Minodronate treatment improves low bone mass and reduces progressive thoracic scoliosis in a mouse model of adolescent idiopathic scoliosis.

Recent studies have shown an association between osteopenia and adolescent idiopathic scoliosis (AIS) and implied that osteopenia plays a causative role in AIS development. This study aimed to determine if minodronate (MIN) treatment could prevent curve progression by increasing bone mass in a thoracic restraint (TR) mouse model, which develops causes the development of thoracic scoliosis similar to human AIS. A total of 100 young female C57BL6J mice were divided into four groups: (1) control with vehicle (CON/VEH; n = 20), (2) control with MIN (CON/MIN; n = 20), (3) TR with vehicle (TR/VEH; n = 30), or (4) TR with MIN (TR/MIN; n = 30). MIN (0.01 mg/kg/week) and vehicle were administered intraperitoneally to their respective groups. TR was performed at age 4 weeks, and the mice were sacrificed at age 9 weeks. Body weights, spine radiographs, femoral bone mineral density (BMD), serum bone marker levels, and histomorphometry of the cancellous bone of the thoracic vertebrae were analyzed. TR significantly reduced weight gain in the TR/VEH group relative to the CON/VEH group. TR also induced osteoporosis with accelerated bone resorption, as indicated by decreases in femoral BMDs and thoracic cancellous bone volume and increases in serum bone resorption marker levels and histomorphometric resorption parameters in the TR/VEH group. MIN partially improved body weight gain and improved poor bone structure relative to the TR/VEH group by suppressing high bone resorption in the TR/MIN mice. MIN significantly reduced the curve magnitudes, as indicated by a 43% lower curve magnitude in the TR/MIN mice than in the TR/VEH mice (17.9 ± 8.9° vs. 31.5 ± 13.1°; p< 0.001). The administration of MIN increased bone mass and reduced the severity of scoliosis in the TR mice. MIN was suggested as a possible inhibitor of scoliosis development.

Novel porcine experimental model of severe progressive thoracic scoliosis with compensatory curves induced by interpedicular bent rigid temporary tethering.

The tether release model will be used to evaluate corrective non-fusion technologies in future investigations. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 36:174-182, 2018.

A shallow chest correlates with the aortic position in the normal spine: features resembling those observed in structural scoliosis.

BackgroundRight thoracic curvature, rib cage deformities and aortic left shift are features of adolescent idiopathic scoliosis that are correlated with each other. We recently reported that disturbance of ribcage development results in progressive thoracic scoliosis in mice. Recently, it has been confirmed that the normal spine exhibits right thoracic curvature and rib cage deformities and that these deformities worsen during the adolescent period. The purpose of this study was to examine whether rib cage deformities correlate with thoracic side curvature in the normal spine, as observed in scoliosis, which is important basic knowledge needed to elucidate the causative factors of adolescent idiopathic scoliosis.MethodsTo examine the relationship between rib cage deformities and thoracic side curvature in the normal spine, CT scans of 148 consecutive adult females were examined. The anteroposterior chest dimension, aortic location and rib cage rotation were measured on CT scans obtained at the T8 level. The thoracic side curvature (T5-T12) was also measured on chest radiographs.ResultsThe anteroposterior chest dimension exhibited a significant correlation with aortic left shift. The aortic location and rib cage rotation were correlated, and the rib cage rotation and thoracic side curvature were correlated.ConclusionsThere was a significant correlation between a shallow chest and the aortic position, between the aortic position and the rib cage rotation and between the rib cage rotation and the thoracic side curvature in the normal spine. These findings suggest the possibility that rib cage development is one of the causative factors of adolescent idiopathic scoliosis.

Disturbance of Rib Cage Development Causes Progressive Thoracic Scoliosis

The pathomechanism underlying idiopathic scoliosis remains unclear, and, to our knowledge, a consistent and relevant animal model has not been established previously. The goal of this study was to examine whether a disturbance of rib cage development is a causative factor for scoliosis and to establish a nonsurgical mouse model of progressive scoliosis. To examine the relationship between rib cage development and the pathogenesis of progressive scoliosis, a plastic restraint limiting anteroposterior rib cage development was placed on the chest of four-week-old mice. All mice were evaluated with whole-spine radiographs, and the severity of scoliosis was consecutively measured. The rib cage rotation angle and the anteroposterior chest dimension were measured with use of micro-computed tomography scanning. To examine whether the imbalanced load transmission through the ribs to the vertebral body was involved in our model, we performed a rib-neck osteotomy in a subgroup of the mice. The thoracic restraint did not provoke spinal curvature immediately after it was applied, but nine of ten mice that wore the restraint but did not have rib osteotomy gradually developed progressive scoliosis. Radiographs and computed tomography images showed a right thoracic curvature, vertebral rotation, and narrow chest in the mice that had worn the restraint for eleven weeks but did not have rib osteotomy even after the restraint was removed. The anteroposterior chest dimension was significantly correlated with both the curve magnitude and the rib cage rotation angle. The progression of spinal deformity was observed only during the adolescent growth spurt, and it plateaued thereafter. The left-side rib osteotomy led to the development of progressive left-thoracic curvature, whereas the bilateral rib osteotomy did not cause scoliosis, even with restraint wear. We established a nonsurgical experimental model of progressive scoliosis and also demonstrated that a rib cage deformity with an imbalanced load to the vertebral body resulted in progressive structural scoliosis.

Hémi-hypertrophie et scoliose révélatrices d’une malformation de Chiari de type 1 avec syringomyélie

We report the case of a 9-year-old boy with progressive thoracic scoliosis and crossed hemihypertrophy who was discovered with a Chiari 1 malformation and syringomyelia. These disorders are connected by complex physiopathological mechanisms; their association deserves attention. This observation reviews the importance of the clinical examination, particularly the neurological exam, in childhood scoliosis. The features suggesting a neurogenic background of spine deformation should be sought. Scoliosis with hemihypertrophy can be the sign of an underlying neurological abnormality.

Computer navigation-assisted spinal fusion with segmental pedicle screw instrumentation for scoliosis with Rett syndrome: a case report.

Scoliosis is a common clinical manifestation of Rett syndrome, a neurodevelopmental disorder that almost exclusively affects females. The spinal curve in patients with Rett syndrome is typically a long C curve of a neuromuscular type. As the onset of the scoliosis is very early and shows rapid progression, early surgical intervention has been recommended to prevent a life-threatening collapsing spine syndrome. However, there are high perioperative risks in Rett syndrome patients who undergo spinal surgery, such as neurological compromise and respiratory dysfunction due to rigid spinal curve. We herein report the surgical result of treating severe rapid progressive thoracic scoliosis in a 16-year-old girl with Rett syndrome. Posterior segmental pedicle screw fixation was performed from T1 to L3 using a computer-assisted technique. Post-operative radiography demonstrated a good correction of the curve in both the sagittal and coronal alignment. There were no postoperative complications such as neurological compromise. The patient had maintained satisfactory spinal balance as of the 3-year follow-up examination.

Progressive Thoracic Scoliosis Due to a Large Plexiform Neurofibroma

Progressive Thoracic Scoliosis Due to a Large Plexiform Neurofibroma

Imagerie de la neurofibromatose de type 1

Neurofibromatosis type 1 (NF1) is the most common of all the phakomatoses. It is an autosomal dominant disorder, with about 50% of patients being new mutations. NF1 is diagnosed based on the presence of well established diagnostic criteria. Prominent cutaneous manifestations include cafe-au-lait spots, freckling and cutaneous neurofibromas. CNS lesions are frequent and imaging is valuable for diagnosis, treatment and follow-up of patients. Tumors of the central nervous system are frequent. Optic nerve glioma usually affects younger patients with clinical symptoms in one third of cases. MRI shows fusiform enlargement with variable enhancement of the optic nerve. These tumors are usually non-aggressive with good prognosis. Other gliomas and astrocytomas can occur as well, usually midline in location, that also generally have good prognosis. Non-tumoral white matter lesions, referred as unidentified bright objects or UBO's, are frequently observed, typically in the basal ganglia and posterior fossa structures. These lesions are usually seen during childhood and they typically diminish with age. The distinction between UBO's and other tumors may be difficult to achieve at imaging, and a malignant evolution may very rarely be observed. Patients with NF1 may have hydrocephalus and dural sac anomalies leading to meningocele formation. Neurofibromas and plexiform neurofibromas involve peripheral nerves and nerve sheaths. Plexiform neurofibromas may cause radicular symptoms. They more frequently involve the lumbosacral plexus. Neurofibromas are homogeneous oval shaped tumors that may extend into the spinal canal. Neurofibrosarcoma is the main cause of death of NF1 patients less than 40 years of age. It may develop de novo or from sarcomatous degeneration of a pre-existing plexiform neurofibroma. It should be suspected in patients with new onset of symptoms or patients with changing symptoms. At imaging, it is characterized by a large heterogeneous tumor invading adjacent structures. Osseous lesions have been described including progressive thoracic scoliosis, vertebral anomalies (posterior scalloping is very suggestive), long bones anomalies with frequent bowing of the tibia, sometimes resulting in pseudarthrosis, and rib anomalies with ribbon ribs. Vascular lesions may occur resulting in arterial hypertension and aneurysm formation.