Idiopathic talipes equinovarus is the most common congenital defect characterized by the presence of a congenital dysplasia of all musculoskeletal tissues distal to the knee. For many years, the treatment has been based on extensive surgery after manipulation and cast trial. Owing to poor surgical results, Ponseti developed a new treatment protocol consisting of manipulation with cast and an Achilles tenotomy. The new technique requires 4 years of orthotic management to guarantee good results. The most recent studies have emphasized how difficult it is to comply with the orthotic posttreatment protocol. Poor compliance has been attributed to parent's low educational and low income level. The purpose of the study is to evaluate if poor compliance is due to the complexity of the orthotic use or if it is related to family education, cultural, or income factors. Fifty-three patients with 73 idiopathic talipes equinovarus feet were treated with the Ponseti technique and followed for 48 months after completing the cast treatment. There was a male predominance (72%). The mean age at presentation was 1 month (range: 1 wk to 7 mo). Twenty patients (38%) had bilateral involvement, 17 patients (32%) had right side affected, and 16 patients (30%) had the left side involved. The mean time of manipulation and casting treatment was 6 weeks (range: 4 to 10 wk). Thirty-eight patients (72%) required Achilles tenotomy as stipulated by the protocol. Recurrence was considered if there was a deterioration of the Dimeglio severity score requiring remanipulation and casting. Twenty-four out of 73 feet treated by our service showed the evidence of recurrence (33%). Sex, age at presentation, cast treatment duration, unilateral or bilateral, severity score, the necessity of Achilles tenotomy, family educational, or income level did not reveal any significant correlation with the recurrence risk. Noncompliance with the orthotic use showed a significant correlation with the recurrence rate. The noncompliance rate did not show any correlation with the patient demographic data or parent's education level, insurance, or cultural factors as proposed previously. The use of the brace is extremely relevant with the Ponseti technique outcome (recurrence) in the treatment of idiopathic talipes equinovarus. Noncompliance is not related to family education, cultural, or income level. The Ponseti postcasting orthotic protocol needs to be reevaluated to a less demanding option to improve outcome and brace compliance.

Background: Spondylothoracic dysplasia is a condition in which bilateral chest wall deformity due to costovertebral rib fusion with shortening of the thoracic spine results in severe thoracic insufficiency syndrome and early death. Little is known about the long-term respiratory natural history of this disorder and the specific anatomic deformity. Methods: We conducted a multicenter prospective and retrospective study of patients with spondylothoracic dysplasia. Medical evaluations, respiratory history, physical examination findings, radiographs, computed tomographic scans, and pulmonary function tests were studied. Anatomic, radiographic, and functional parameters for the disorder were established to determine the natural history of the thoracic insufficiency syndrome. Results: Twenty-eight patients were identified. Eight patients had died in the neonatal period, and twenty were evaluated (eleven prospectively and nine retrospectively). The survivors were doing well clinically, but the average spirometric values were 27.9% of the predicted normal value for the forced vital capacity (FVC), 29.5% of the predicted normal value for the forced expiratory volume in the first second (FEV1), and 0.92 for the FEV1/FVC ratio, demonstrating a severe restrictive respiratory pattern. The computed tomographic scan lung volumes were an average of 28% of the expected values for age and gender. The thorax was stiff from rib fusion and was severely shortened posteriorly, averaging 24.2% of the predicted normal length. The thoracic spine was predominantly composed of block vertebrae, whereas in the lumbar region there were multiple hemivertebrae. Minimal scoliosis was seen, and there were no neurological deficits. Conclusions: Spondylothoracic dysplasia has a unique pathoanatomy of volume depletion deformity of the thorax with chest wall stiffness, resulting in thoracic insufficiency syndrome. Clinical tolerance of the restrictive lung disease in this disorder is impressive, but no clear reason has yet been identified for the clinical pulmonary health in the face of severe restrictive lung disease. Patients who survive infancy show no progression of congenital anomalies and can have a good quality of life. This disease may serve as a model of the natural history of thoracic insufficiency syndrome due to growth inhibition of the thoracic spine either as a result of congenital causes or secondary to surgical fusion early in life. Level of Evidence: Prognostic Level IV. See Instructions to Authors for a complete description of levels of evidence.

The Vertical Expandable Prosthetic Titanium Rib (VEPTR) is a technique developed for the treatment of progressive early onset scoliosis. This vertically placed device uses distraction to indirectly elongate the spine and chest, stabilizing the progression of the spinal deformity while preserving spinal growth. Thoracic spine and chest wall deformity are usually correlated; therefore, elongation of the chest wall will increase the space available for the lung and improve respiratory mechanics in patients with early onset scoliosis. We conducted a retrospective study of 17 patients with early onset scoliosis treated with the VEPTR technique. The medical records, imaging studies, and follow-up physical examinations were evaluated. The patient population consisted of 17 primary VEPTR implantations and 33 expansion surgeries with a mean follow-up of 25 months. Our results show that there was an improvement in the coronal plane deformity between the presurgical and postsurgical Cobb angles, preoperative Cobb angle of 59 degrees (range 38-77) to postoperative 35 degrees (range 10-70), resulting in an average decrease of 59% in the Cobb angle (P<0.001). The thoracic kyphosis was maintained at anatomically normal values. The surgical technique preserved the space available for the lung. The complication rate was 13%, which includes infection, device migration, and rib fracture. The analysis of the data shows that the natural history of the progressive spinal deformity was improved in all patients. This preliminary report reaffirms that the VEPTR implantation is a safe and efficient method for the treatment of early onset scoliosis.

Background Spondylocostal dysplasia (SCD) constitutes a heterogeneous patient group with multiple vertebral formations and segmentation defects of the entire spine, with asymmetric rib malformations. Respiratory failure has been reported in spondylocostal dysplasia secondary to thoracic insufficiency syndrome. The vertical expandable prosthetic titanium rib (VEPTR) reconstructs the chest wall to address the thoracic insufficiency seen in this patient population. The purpose of this study is to evaluate spinal deformity correction and respiratory function outcomes in a spondylocostal dysplasia population treated with VEPTR. Methods A cohort of 20 patients with spondylocostal dysplasia and 2-year follow-up were evaluated from a multicenter IDE study of 214 patients who had surgery with the VEPTR device. Data collected included gender, nonskeletal malformations, age at surgery, number of procedures, estimated blood loss, length of stay, and surgical time. Clinical and radiographic parameters were collected, and respiratory function was assessed. Results In 14 of 20 patients (70%), spinal deformity was controlled evidenced by a decrease of the initial Cobb coronal angle at last follow-up. Fourteen patients (70%) maintained their oxygen level throughout treatment. At preoperative and last evaluation, assisted ventilation rating (AVR) scores showed that 5 patients improved their level of ventilation and 14 patients maintained their AVR level at room air. One patient decreased his level from supplemental oxygen to night ventilation. Mean thoracic spinal length (growth) by year was 0.82 cm. No mortality occurred in this group of patients. Conclusions VEPTR implantation in SCD allows continued thoracic spine growth while controlling progressive spine deformity. The improved AVR ratings after surgery suggest a beneficial effect on the natural history of TIS in this population. Mortality and complication rate seem acceptable in this high-risk population of SCD patients. Level of Evidence Therapeutic study, Level IV, (case series, no comparison group).