Artificial intelligence data-driven 3D model for AIS.

Scoliosis is a 3D deformation of the spinal column, characterized by a lateral deviation of the spine, accompanied by axial rotation of the vertebrae. Adolescent Idiopathic Scoliosis (AIS), is the most common type, affecting children between ages 8 to 18 when bone growth is at its maximum rate. The selection of the most appropriate treatment options is based on the surgeon's experience. So, developing a clinically validated patient-specific model of the spine would aid surgeons in understanding AIS in early stages and propose an efficient method of treatment for the individual patient. This project steps include: Developing a clinically validated patient-specific Reduced Order Finite Element Model (ROFEM) of the spine, predicting AIS progression using data mining and proposing a method of treatment. First we implement FE synergistically with bio-mechanical information, image processing and data science techniques to improve predictive ability. Initial geometry of the spine will be extracted from the x-ray images from different planes and imported to FEM software to generate the spine model and perform analysis. A RO model is developed based on the detailed spinal FEM. Next, a neural network is used to predict the spinal curvature. The ability to predict the severity of AIS will have an immense impact on the treatment of AIS-affected children. Access to a predictive and patient-specific model will enable the physicians to have a better understanding of spinal curvature progression. Consequently, the physicians will be able to educate families, choose the most appropriate treatment option and asses for surgical intervention.

Standards in anterior spine surgery in pediatric patients with neuromuscular scoliosis.

The authors reviewed 111 patients with neuromuscular disease who underwent anterior spine surgery for correction of scoliosis. An overall complication rate of 44.1% was found, 21.6% major and 22.5% minor. Pulmonary complications were the most common major complications, urinary tract infections the most common minor complications. The rate of complications was greater in patients with cerebral palsy, thoracoabdominal and transthoracic approaches, staged procedures, operative blood loss >1,000 mL, or previous spine surgery. In addition, statistical analysis confirmed that curve magnitude >100 degrees degrees was a risk factor for complications.

Evaluating congenital spine deformities for intraspinal anomalies with magnetic resonance imaging.

SUMMARY: The incidence of intraspinal abnormalities associated with congenital spinal anomalies as detected by magnetic resonance imaging (MRI) is becoming better defined. In this study, 41 nonrandomized children with congenital spinal deformities (excluding myelomeningocele) who underwent complete MR evaluation were reviewed. Of the 41 congenital spinal deformities, 37 demonstrated congenital scoliosis, with failure of formation in 19, failure of segmentation in 4, and mixed defects in 14. The remaining four deformities were cases of congenital kyphosis. Thirteen patients with congenital spine anomalies were noted to have intraspinal abnormalities identified by MRI: tethered cord in 12 patients, syringomyelia in 3 patients, and diastematomyelia in 5 patients. Of the 12 patients with tethered cord, 2 patients had neurologic deficits. Urorectal anomaly was one of the most common associated findings (15%). Considering an incidence of intraspinal anomalies of 31% and as clinical manifestations may not be initially detectable, MRI is recommended in patients with congenital spinal deformity as part of the initial evaluation even in the absence of clinical findings.

Neural tube defects and the 13q deletion syndrome: evidence for a critical region in 13q33-34.

Neural tube defects (NTD) are common findings in the 13q deletion syndrome, but the relationship between the 13q- syndrome and NTDs is poorly understood. We present a child with a 13q deletion and lumbosacral myelomeningocele. This was a boy with microcephaly, telecanthus, minor facial anomalies, and ambiguous genitalia. Cytogenetic and fluorescence in situ hybridization analysis showed a de novo 46,XY,del(13)(q33.2-->qter) with no visible translocation. By using microsatellite markers, the deletion breakpoint was mapped to a 350-kb region between D13S274 and D13S1311 and was paternal in origin. An analysis of 13q deletions with NTDs, including the present case, suggests that a deletion in 13q33-34 is sufficient to cause an NTD. The deletions associated with NTDs are distal to and nonoverlapping with the previously defined critical region in 13q32 for the major malformation syndrome [Brown et al., 1999: Am J Hum Genet 57: 859-866]. Our analysis also suggests that one or more genes in 13q33-34 produces NTDs by haploinsufficiency.

Quantitative assessment with SPECT imaging of stress injuries of the pars interarticularis and response to bracing.

The evaluation and management of acute spondylolysis remains unclear in part because of outcome data that are primarily subjective. The aim of this study was to evaluate and monitor these patients objectively using quantitative single-photon emission computed tomography (SPECT). Thirty-four patients were so observed clinically between 1987 and 1996 and were studied with an initial and at least one follow-up SPECT scintigram. Initial radiographs and planar bone scans failed to demonstrate the pars lesion in 53 and 19% of the patients, respectively. The average SPECT ratio before brace treatment was 1.45. After treatment, this ratio significantly decreased to 1.27 (p = 0.03). A subset of patients remained symptomatic at follow-up. Their reduction in SPECT ratio averaged only 2.8% as compared with 13% for the remainder of the patients (p = 0.01). Patients diagnosed and braced in the early, more active stage of the condition (with greater intensity on SPECT) had more predictable symptom relief. An initial SPECT ratio of >1.5 was associated with complete symptom resolution after brace treatment. Patients treated with activity restriction only (>3 months) before bracing were more likely to have persistent symptoms and more modest improvement on SPECT (p = 0.01). These data, which use SPECT scintigraphy, support prompt treatment with brace immobilization for acute spondylolysis in children and adolescents.

Anterior only fusion for scoliosis in patients with myelomeningocele.

A series of patients with single major scoliosis curvatures attributable to spina bifida treated by anterior only spinal fusion was studied for 2 years to determine whether the infection rate could be decreased, adequate correction and pelvic balance could be provided, and posterior surgery could be avoided in these patients. Anterior surgery alone was performed for thoracolumbar scoliosis greater than 45 degrees if the compensatory thoracic curve was less than 40 degrees and there was no significant junctional kyphosis. Fourteen patients were treated at a mean age of 11.9 years (range, 7-16 years), with a mean curve of 64 degrees (range, 51 degrees-85 degrees), and motor levels distributed from T10-L4. Thirteen patients had prior neurosurgery for tether, syrinx, or Arnold-Chiari malformation. The spine was fused over a mean of seven vertebrae. A 3/16 inch Texas Scottish Rite Hospital rod was used most commonly (10 patients). Blood loss averaged 1100 cc. The mean curve correction was 57% at 40 months after surgery. Loss of correction occurred primarily by adding on outside the instrumented area. Mean pelvic obliquity was improved from 16 degrees to 9 degrees. There was one superficial infection. Results were good in five patients, fair in four, and poor in five. Failures were attributable to proximal decompensation in two patients who required revision surgery (two), neurologic deterioration in two, and screw pullout in one. Both patients with decompensation had syringomyelia. Both patients with neurologic deterioration had large curves (> 75 degrees). Both patients recovered after rod removal. Retrospectively, by eliminating patients with syrinx or with a curve greater than 75 degrees, all poor results would be eliminated. Anterior only fusion and instrumentation may have significant advantages, but only for selected patients with thoracolumbar curves less than 75 degrees, compensatory curves less than 40 degrees, no increased kyphosis, and no syrinx. Quadriceps function should be monitored. On the basis of this preliminary experience, continued use of this approach using stricter selection seems warranted.

Kyphosis deformity in myelomeningocele.

Management of the child with myelomeningocele and kyphosis is an extreme challenge to the orthopedic surgeon and spine surgeon on many fronts. Delayed or observation treatment may result in loss of functional independence and self-esteem. Early surgical correction may result in loss of truncal height, intra-abdominal upward volume effect on the diaphragm, and loss of pulmonary capacity. Late surgical reconstruction may be associated with significant morbidity and mortality. Early surgical intervention with preservation of growth may prove safer and result in improved function and independence.

Modification of Cotrel-Dubousset's original hook constructs for idiopathic scoliosis.

We performed a retrospective review of 41 patients (ages 9-18 years) who underwent posterior spinal fusion with either Isola or Cotrel-Dubousset (CD) instrumentation to determine whether the presence of an apical hook on the thoracic convexity affected initial and long-term sagittal and coronal correction in adolescent idiopathic scoliosis surgery. A study group of 38 female and three male patients was evaluated (2-5 years of follow-up). Twenty-three patients (Group A) were treated with an up-going hook at the convex apex of the thoracic curve, and 18 patients (Group B) with similar curves were instrumented without an apical hook. Results showed that Group A's average preoperative coronal curve of 48 degrees decreased to 17 degrees , whereas Group B's preoperative average of 52 degrees decreased to 25 degrees . At follow-up, no statistical significance was noted in either coronal curve correction (p = 0.203) or sagittal kyphosis (p = 0.38) between Groups A and B. We conclude that omission of the up-going hook at the apex of the thoracic convexity can reduce postoperative discomfort in patients undergoing posterior spinal fusion, without sacrificing curve correction or balance.

Pediatric spinal deformity.

Pediatric spinal deformity is a common problem facing the pediatrician and orthopedic surgeon. Most commonly seen is idiopathic scoliosis. Early diagnosis and treatment are important. Although the etiology of idiopathic scoliosis remains unclear, there continues to be a search for genetic markers and studies for the modes of inheritance. Idiopathic scoliosis is more clearly understood recently and is recognized as a complex three-dimensional deformity. Prognostic indicators for juvenile scoliosis have been identified. Surgical management of idiopathic scoliosis continues to evolve, and now a thoracoscopic endoscopic technique is available. Long-term follow-up has demonstrated generally satisfactory results with spinal fusion surgery. Other syndromes such as Klippel-Feil, familial dysautonomia, and Marfan syndrome demonstrate high rates of scoliotic deformities. Most are unresponsive to bracing and most often require surgical intervention. Although primary spinal neoplasms are uncommon, most are benign, and outcomes are generally satisfactory.

The influence of forearm crutches on pelvic and hip kinematics in children with myelomeningocele: don't throw away the crutches.

Gait analysis was performed on 16 children with high-sacral-level myelomeningocele who walked with and without crutches to evaluate the influence of crutches on their unique walking pattern. All of the patients used solid ankle-foot orthoses (AFOs). Deviations in coronal and transverse planes improved with assisted walking. The timing of stance phase pelvic depression and the magnitude of stance phase hip abduction improved with crutch walking. Pelvic rotation, which was seven times the normal range of motion during no-crutch walking, decreased to four times normal with crutches. Walking velocity was not significantly different between conditions. The results demonstrated that deviations in pelvic and hip kinematics are related to muscle weakness and improve with crutch use. Crutches enable the patient to transfer some weight bearing to their upper extremities which decreases the demand on weak lower-extremity musculature. This allows them to maintain functional ambulation with a closer to normal gait pattern.

Tethered cord syndrome in low motor level children with myelomeningocele.

The clinical presentation of tethered spinal cord and the results of tethered cord release were examined in a group of 30 low motor level (L3 and below) children with a history of myelomeningocele without concomitant CNS complications. Changes in orthopedic and/or neurologic status formed the basis of consideration for tethered cord release. Clinically, these patients presented with a new onset or recently progressing scoliosis, spasticity with or without contractures, decrease in motor function and low back pain at the site of closure. One or more of these findings was present in all cases and led to the suspicion of tethered spinal cord. The diagnosis of tethered cord was confirmed in all cases by MRI or CT myeolography. In order to isolate tethering as the etiology for the patients' clinical deterioration, patients with concomitant CNS complications, e.g. shunt dysfunction or hydromyelia were excluded from the study. Twenty-nine such patients, of an initial 59, who would have otherwise been considered, were excluded on the basis of this criteria of concomitant CNS complications. The results of release 1 year after the procedure were as follows: regarding scoliosis, in 75% of cases the curve either remained stable or decreased by more than 10 degrees, with 25% experiencing curve progression of > 10 degrees. The most recent follow-up in this group revealed that 11.8% experienced a decrease in curvature of >10 degrees; 47.1% remained stable, and 41.2% ultimately progressed 10 degrees. In the group with spasticity, 43.8% improved; 56.3% remained stable, and none worsened. Most (78.6%) of the children who had experienced a decline in motor function improved postoperatively, and all those with back pain experienced complete resolution. In conclusion, tethered cord release in symptomatic low lumbar and sacral level children with myelomeningocele appears to be of benefit, especially with respect to stabilization of scoliosis in selected patients, back pain at the site of closure, and prior decline in motor function. Results in the cases with spasticity were more equivocal.

Spina bifida.

Spina bifida (myelomeningocele) is the most common major birth defect among live-born infants. It is now recognized that half of those cases are preventable if folate is given periconceptionally. Epidemiology is discussed, together with an overview of approaches to orthopedic problems from birth to maturity.

Orthopedic aspects of myelodysplasia in children.

A review of new developments in spina bifida shows that many cases are preventable by administration of periconceptual folic acid, reducing the relative risk by up to 86%. Currently, however, 50% of women of reproductive age have no folic acid in their usual diet. The Centers for Disease Control as recommended folic acid at 0.4 mg/d for all women anticipating pregnancy. The long-term outcomes of sacral level patients show a surprising decline in adult ambulatory status. Adult ambulatory status in spina bifida is significantly predictive (89% accurate) by two variables: motor level and sitting balance. Studies regarding hip dislocation, foot deformities in thoracic level children, the prevalence of spondylolisthesis, and surgical treatment of kyphosis are also reviewed.

Freeman-Sheldon syndrome (whistling face syndrome) and cranio-vertebral junction malformation producing dysphagia and weight loss.

The whistling face syndrome has been reported, with complications of the respiratory system and feeding problems due to microstomia, since 1938. Dysphagia, however, has not previously been reported as a complication of whistling face syndrome. We report a 13-year-old with the whistling face syndrome and dysphagia of late onset who has the typical whistling face, scoliosis and multiple joint contractures. Dysphagia occurred as a result of compression at the cervicomedullary junction by a cranio-vertebral junction malformation and impingement by the tip of the odontoid process. This child had undergone multiple surgical procedures for cleft palate, clubfeet, contractures of the hip and knee and scoliosis. Halo traction and posterior occipital-cervical decompression and fusion successfully treated the dysphagia.

The orthopaedic manifestations of prune-belly (Eagle-Barrett) syndrome.

Forty children were managed for prune-belly syndrome between 1979 and 1989, and twenty-five of them had musculoskeletal abnormalities. The musculoskeletal abnormalities were primary in twenty-two children, secondary to renal osteodystrophy in one, and both primary and secondary to renal osteodystrophy in two. Thirteen children had marked abnormality of the hip, and congenital dislocation was typically resistant to conventional treatment. Scoliosis was seen in seven patients. Pectus excavatum (a chest-wall deformity) was seen in eleven patients, including five of the six who had an idiopathic-like curve. Although prune-belly syndrome is uncommon, the diagnosis necessitates a thorough orthopaedic evaluation because of the high prevalence of associated musculoskeletal abnormalities.

Femoral shaft fractures in children treated with early spica cast.

We retrospectively reviewed 51 patients aged 3-11 years with femoral shaft fractures selected for treatment with early spica cast immobilization. Shortening greater than 20 mm was the most common complication, occurring in 22 (43%) of the 51 patients. Factors associated with unacceptable shortening were shortening at the time of spica cast application greater than 10 mm, shortening greater than 20 mm at initial examination, and increasing age. Achieving less than 1 cm shortening at the time of cast application and close follow-up during the first 2 weeks after cast application are advised in order to achieve an acceptable final outcome.

The natural history of congenital kyphosis in myelomeningocele. A review of 51 children.

The progression of congenital lumbar kyphosis in myelomeningocele is a well-known problem, but rates of progression are not well documented in the literature. Fifty-one children with congenital kyphosis and myelomeningocele were followed for an average of 4.8 years. Minimum follow-up was 1 year. Group I (35 patients) had initial radiographs at 1 year of age or less. Group II (16 patients) had radiographs taken after the age of 1 year. Curves less than or equal to 90 degrees in Group I progressed 7.7 degrees/yr; those greater than 90 degrees progressed 12.1 degrees/yr. Curves less than or equal to 90 degrees and greater than 90 degrees progressed at similar rates, regardless of initial curve magnitude: 6.4 degrees/yr and 6.7 degrees/yr, respectively. No correlation existed among the rate of curve progression, the frequency of shunt revisions, or the presence of vertebral anomalies, aside from the dysraphism.