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Background: The purpose of the present study was to evaluate changes in pulmonary function, caused by preoperative halo-pelvic traction (HPT) for the treatment of extremely severe and rigid kyphoscoliosis, with use of 3-dimensional computed tomography (3D-CT) reconstruction and pulmonary function tests (PFTs)., Methods: Twenty-eight patients with severe and rigid scoliosis (Cobb angle, >100°) underwent preoperative HPT and staged posterior spinal fusion. CT, radiographic assessment, and PFT were performed during pre-traction and post-traction visits. The changes in total lung volume were evaluated with use of 3D-CT reconstruction, and the changes in pulmonary function were evaluated with PFTs at each time point. Differences were analyzed with use of 2-tailed paired Student t tests, and correlations were analyzed with use of Spearman rank tests., Results: None of the patients had pulmonary complications during traction, and all radiographic spinal measurements improved significantly after HPT. The main Cobb angle was corrected from 143.30° ± 20.85° to 62.97° ± 10.83° between the pre-traction and post-traction evaluations. Additionally, the C7-S1 distance was lengthened from 280.48 ± 39.99 to 421.26 ± 32.08 mm between the pre-traction and post-traction evaluations. Furthermore, 3D lung reconstruction demonstrated a notable increase in total lung volume (TLV) (from 1.30 ± 0.25 to 1.83 ± 0.37 L) and maximum lung height (from 176.96 ± 27.44 to 202.31 ± 32.45 mm) between the pre-traction and post-traction evaluations. Moreover, PFTs showed that total lung capacity (TLC) improved between the pre-traction and post-traction evaluations (from 2.06 ± 0.32 to 2.98 ± 0.82 L) and that the changes in T1-T12 distance and maximum lung height were correlated with changes in TLV (p = 0.0288 and p = 0.0007, respectively)., Conclusions: The application of HPT is a safe and effective method for improving pulmonary function in patients with extremely severe and rigid scoliosis before fusion surgery. The TLV as measured with CT-based reconstruction was greatly increased after HPT, mainly because of the changes in thoracic height., Level of Evidence: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article ( http://links.lww.com/JBJS/H703 )., (Copyright © 2023 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: The Classification of Early-Onset Scoliosis (C-EOS) allows providers to differentiate patients, for clinical and research purposes, on the basis of the etiology of their disease as well as radiographic parameters. The Early Onset Scoliosis Questionnaire (EOSQ) is the first disease-specific, parent-reported HRQOL (health-related quality-of-life) outcome measure for this condition. We sought to determine the influence of the C-EOS etiology designation, radiographic parameters, and medical comorbidities on EOSQ scores to differentiate quality of life in this heterogeneous patient population. We hypothesized that baseline EOSQ scores for patients with EOS would be strongly affected by the C-EOS etiology designation., Methods: The analysis included prospectively enrolled patients with EOSQ scores recorded in a multicenter EOS database prior to intervention for the EOS. EOSQ scores were compared across C-EOS etiologies, severity of disease based on radiographic measurements, and patient comorbidities prior to scoliosis intervention., Results: Six hundred and ten patients with EOS were available for analysis; 119 had congenital, 201 had idiopathic, 156 had neuromuscular, and 134 had syndromic EOS. In multivariate analysis, neuromuscular and syndromic etiologies were associated with lower scores than congenital and idiopathic etiologies in many EOSQ domains including general health, transfer, daily living, fatigue/energy level, and emotion. Patients with neuromuscular EOS had the lowest EOSQ scores in general. Congenital and idiopathic EOS did not differ from each other in any EOSQ domain. Coronal Cobb and kyphosis angles had significant inverse but generally weak correlations with EOSQ domains. Individual medical comorbidities had a minor effect on certain domains while American Society of Anesthesiologists (ASA) class and total number of comorbidities had inverse correlations with most domains., Conclusions: The underlying etiology of EOS appears to have a significant influence on the parent-reported HRQOL outcomes of the disease. Specifically, syndromic and neuromuscular C-EOS diagnoses are associated with lower EOSQ scores before treatment compared with congenital and idiopathic diagnoses. Radiographic measurements of severity have a relatively small influence on EOSQ scores. These baseline differences in C-EOS-designated etiology should be accounted for in studies comparing outcomes of treatment for this heterogeneous patient population., Level of Evidence: Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence., Competing Interests: Disclosure: This project was supported by the Angela S. M. Kuo award through the Pediatric Orthopaedic Society of North America. Funding was directed to the PSSG. On the Disclosure of Potential Conflicts of Interest forms, which are provided with the online version of the article, one or more of the authors checked “yes” to indicate that the author had a relevant financial relationship in the biomedical arena outside the submitted work; “yes” to indicate that the author had a patent and/or copyright, planned, pending, or issued, broadly relevant to this work; and “yes” to indicate that the author had other relationships or activities that could be perceived to influence, or have the potential to influence, what was written in this work (http://links.lww.com/JBJS/G291)., (Copyright © 2021 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Cervical kyphosis may occur with neurofibromatosis type I (NF1) and is often associated with vertebral dysplasia. Outcomes of cervical spinal fusion in patients with NF1 are not well described because of the rarity of the condition. We aimed to (1) characterize the clinical presentation of cervical kyphosis and (2) report the outcomes of posterior and anteroposterior cervical fusion for the condition in these children., Methods: The medical records and imaging studies of 22 children with NF1 who had undergone spinal fusion for cervical kyphosis (mean, 67°) at a mean age of 11 years and who had been followed for a minimum of 2 years were reviewed., Results: Thirteen children presented with neck pain; 10, with head tilt; 9, with a previous cervical laminectomy or fusion; and 5, with a neurologic deficit. Two patients had spontaneous dislocation of the mid-cervical spine without a neurologic deficit. Eleven had scoliosis, with the major curve measuring a mean of 61°. Nine patients underwent posterior and 13 underwent anteroposterior surgery. Twenty-one received spinal instrumentation, and 1 was not treated with instrumentation. Preoperative halo traction was used for 9 patients, and it reduced the mean preoperative kyphosis by 34% (p = 0.0059). At the time of final follow-up, all spinal fusion sites had healed and the cervical kyphosis averaged 21° (mean correction, 69%; p < 0.001). The cervical kyphosis correction was significantly better after the anteroposterior procedures (83%) than after the posterior-only procedures (58%) (p = 0.031). Vertebral dysplasia and erosion continued in all 17 patients who had presented with dysplasia preoperatively. Thirteen patients had complications, including 5 new neurologic deficits and 8 cases of junctional kyphosis. Nine patients required revision surgery. Junctional kyphosis was more common in children in whom ≤5 levels had been fused (p = 0.054)., Conclusions: Anteroposterior surgery provided better correction of cervical kyphosis than posterior spinal fusion in children with NF1. Erosion of vertebral bodies continued during the postoperative follow-up period in all patients who had presented with dysplastic changes preoperatively. The cervical spine should be screened in all children with NF1. Fusion should include at least 6 levels to prevent junctional kyphosis., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence., (Copyright © 2016 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Congenital anomalies of the ribs and chest wall as well as Sprengel deformity of the shoulder are often associated with congenital deformities of the spine. It has been suggested that rib anomalies may adversely affect the progression of the spinal deformity., Methods: We conducted a retrospective study of the medical records and spine radiographs of 620 consecutive patients with congenital deformities of the spine; 497 patients (80%) had scoliosis; eighty-eight patients (14%), kyphoscoliosis; and thirty-five patients (6%), kyphosis. The rib anomalies were classified into simple and complex, and the presence of a Sprengel deformity of the shoulder was recorded. The rate of scoliosis deterioration without treatment before the age of eleven years, as well as the patient age and curve size at the time of surgery, was compared for different types of vertebral abnormalities in patients with and without rib anomalies., Results: A total of 119 patients (19.2%) had rib anomalies, which were most commonly associated with congenital scoliosis (111 patients; 93%) and were much less frequently associated with congenital kyphoscoliosis or kyphosis (eight patients). The rib anomalies were simple in ninety-five patients and complex in twenty-four. Eighty-five patients (71%) with rib abnormalities had a scoliosis due to a unilateral failure of vertebral segmentation, and seven patients had mixed or unclassifiable vertebral anomalies. In contrast, only sixteen of 203 patients with a scoliosis due to a hemivertebra alone had rib anomalies. The rib anomalies were most frequently associated with a thoracic or thoracolumbar scoliosis (102 patients; 92%) and occurred on the concavity in eighty-two patients (74%), the convexity in twenty-two patients (20%), and were bilateral in seven patients. The Sprengel deformity occurred in forty-five patients and most frequently in association with a thoracic scoliosis due to a unilateral failure of vertebral segmentation (twenty-seven patients). No significant difference was detected in the rate of curve progression without treatment in patients with and without rib anomalies. The only exception was the mean age at the time of surgery, which was higher for patients with a unilateral unsegmented bar without rib anomalies (p = 0.005). In addition, no significant difference was found with regard to any tethering effect due to the site of the rib fusions on the concavity of the scoliosis, i.e., whether they were in close approximation to the spine or were more lateral (p > 0.05)., Conclusions: Congenital rib anomalies occur most commonly on the concavity of a thoracic or thoracolumbar congenital scoliosis that is due to a unilateral failure of vertebral segmentation, and they do not appear to have an adverse effect on curve size or rate of progression.

Background: Congenital kyphosis and kyphoscoliosis are much less common than congenital scoliosis. However, they are potentially more serious because compression of the spinal cord and paraplegia sometimes develop. The goals of the present study were to document the natural history of congenital kyphosis and kyphoscoliosis and to determine the stage at which the natural progression should be interrupted by treatment., Methods: We reviewed the medical records and radiographs of the spine of 112 consecutive patients. Sixty-eight patients had a type-I kyphosis due to an anterior failure of vertebral-body formation, twenty-four had a type-II kyphosis due to an anterior failure of vertebral-body segmentation, and twelve had a type-III kyphosis due to a combination of anomalies; the deformities of the remaining eight patients could not be classified. Eighty-five skeletally immature, untreated patients were first evaluated at a mean age of six years and nine months (range, two months to sixteen years and three months), and twenty-seven patients were skeletally mature at the time of the first visit. Sixty-three of the eighty-five skeletally immature patients were observed without any treatment for a mean period of six years and six months (range, one to sixteen years) before skeletal maturity, and the remaining twenty-two patients had a posterior arthrodesis of the spine soon after the initial visit. At skeletal maturity, forty-one patients had not been treated and sixty-eight had had an arthrodesis of the spine. The remaining three patients had not yet reached skeletal maturity at the time of the most recent follow-up., Results: The apex of the kyphosis was seen at all levels but was most frequent between the tenth thoracic and the first lumbar level (seventy-four patients; 66 percent). There was no relationship between the severity of the kyphosis and its location in the spine. Progression of the curve was most rapid during the adolescent growth spurt and stopped only at skeletal maturity. Progression was most rapid and the magnitude of the curve was the greatest in type-III kyphosis (twelve patients) followed by type-I kyphosis due to a posterolateral quadrant vertebra (thirty-nine patients), a posterior hemivertebra (eight patients), a butterfly vertebra (fifteen patients), and a wedged vertebra (six patients). A kyphosis due to two adjacent type-I vertebral anomalies progressed more rapidly and produced a more severe deformity than did a similar single anomaly. The prognosis for type-II kyphosis was variable and was much more severe when an anterolateral unsegmented bar had produced a kyphoscoliosis (nine patients) than it was when a midline anterior bar had produced a pure kyphosis (fifteen patients), which usually progressed slowly. Spontaneous neurological deterioration due to compression of the spinal cord occurred in ten patients (seven of whom had a type-I kyphosis and three of whom had an unclassifiable anomaly) at a mean age of thirteen years and eight months, and one other patient (with an unclassifiable anomaly) had spastic paraparesis at the age of twenty-eight years., Conclusions: Congenital kyphosis and kyphoscoliosis are uncommon deformities with the potential to progress rapidly, resulting in severe deformity and possible neurological deficits. A thorough knowledge of the natural history is essential in the planning of appropriate and timely treatment to prevent progression of the deformity and neurological complications.

Four patients who had Larsen syndrome and cervical kyphosis were managed operatively and followed for an average of seventy months (range, forty to ninety-two months). The preoperative cervical kyphosis ranged from 35 to 65 degrees. The patients had had a posterior cervical arthrodesis alone when they were infants, at an average age fo fourteen months (range, ten to sixteen months). In three infants, the kyphosis either stabilized (one patient) or reversed into lordosis (two patients). Thus, the kyphosis corrected gradually by continued anterior growth in the presence of a solid posterior fusion. In the fourth infant, the kyphosis progressed to 110 degrees because of pseudarthrosis. This child had anterior decompression and arthrodesis for an acute neurological deficit. We believe that cervical kyphosis is sometimes present but not diagnosed in patients who have Larsen syndrome. Early diagnosis followed by operative stabilization should help such patients avoid neurological deficits. Posterior cervical arthrodesis alone, performed in infancy, provided stability and the opportunity for the gradual correction of the deformity by continued anterior growth in three of our four patients.

The results of operative treatment of scoliosis were reviewed for twenty-two patients (ten boys and twelve girls) who had familial dysautonomia, an autosomal recessive disorder affecting primarily Ashkenazi Jews. The indication for operative intervention was progressive kyphoscoliosis to 45 degrees or more in a skeletally immature patient for whom bracing had failed. The mean age at the time of the operation was fifteen years and five months (range, eight years and two months to nineteen years). Seventeen patients had a thoracic curve with a mean preoperative Cobb angle of 69 degrees (range, 47 to 112 degrees), and five patients had a double major curve with a mean preoperative Cobb angle of 71 degrees (range, 42 to 87 degrees) for the cephalad curves and 60 degrees (range, 45 to 72 degrees) for the caudad curves. Twenty patients had a rigid kyphosis; in fourteen, the apex was at the seventh thoracic vertebra or more cephalad. Two patients had a lordoscoliosis. The mean preoperative kyphosis was 64 degrees (range, 12 to 110 degrees) in the thirteen patients who had a thoracic curve and for whom information regarding kyphosis was available, and it was 70 degrees (range, 54 to 84 degrees) in the five patients who had a double major curve. Postoperior spinal arthrodesis and instrumentation was performed in all patients. Two patients had an anterior arthrodesis as well because of the severity and rigidity of the curve. Allograft bone was used in eighteen patients. Postoperatively, all patients were managed with a body cast or with a custom-molded thoracolumbar brace.(ABSTRACT TRUNCATED AT 250 WORDS)

The results of resection of the lordotic segment cephalad to the apical vertebra of a kyphotic deformity in thirty-nine patients who had had a myelomeningocele were reviewed retrospectively. The operations were performed between 1973 and 1984, when the patients were an average of six years and ten months old (range, one year and five months to twenty years old). The average duration of follow-up was eleven years and one month (range, five years and one month to seventeen years and four months). The average preoperative kyphosis was 111 degrees (range, 77 to 151 degrees) and the average postoperative kyphosis was 40 degrees (range, 2 to 88 degrees). The preoperative deformity was reduced an average of 64 per cent (range, 0 to 98 per cent). At the latest follow-up evaluation, the average kyphosis was 62 degrees (range, -25 to 100 degrees). The measurements were obtained from lateral radiographs that were made with the patient sitting preoperatively, immediately postoperatively, at one year, and yearly until the time of the latest follow-up evaluation. At the most recent examination, thirty-four patients had a partial loss of correction, with twenty-five of them having maintained at least 50 per cent of the correction. The five remaining patients had an improvement in the alignment by an average of 26 degrees. Only two patients had an increase in kyphosis compared with the preoperative deformity. Thirty-seven patients had an average increase of 3.2 centimeters (range, 0.2 to 8.2 centimeters) in the height of the lumbar spine; the two remaining patients, who had a decrease in height, had operative intervention after reaching skeletal maturity.

Twenty-two patients with Scheuermann's kyphosis were treated with correction, Harrington instrumentation (in twenty-one), and posterior spine fusion. This procedure relieved pain and corrected deformity in all patients but loss of correction of 5 degrees or more occurred in sixteen of the twenty-two patients, including all those whose initial kyphosis was greater than 70 degrees. The incidence of complications was so high that this procedure should ordinarily be performed only for disabling pain or spinal cord compression.

In this series of nine patients with diastrophic dwarfism, multiple problems with the spine were noted. Scoliosis was present in all patients and usually became rapidly progressive in the preadolescent years. The scoliosis had a strong tendency to become severe and rigid, with a large rotatory component. Early brace treatment appeared to have merit. Cervical kyphosis was noted in four patients; it resolved in three patients, but progressed in one to a 130-degree deformity, quadriplegia, and death. All patients in this series had spina bifida occulta in the cervical spine. Six of the nine patients demonstrated a narrowed interpedicular distance below the third lumbar vertebra, but none had spinal stenosis.

The cast syndrome, recently called the superior mesenteric-artery syndrome, is a well recognized complication that can occur after a body cast has been applied. We are reporting the cases of three patients who had this syndrome following surgical correction of spinal deformity. The complication failed to resolve with the usual non-operative measures but did resolve with treatment by total parenteral nutrition.

We reviewed forty-five patients with congenital constriction bands with an average follow-up of 15.8 years. The family history and associated anomalies were recorded and particular attention was paid to temperature gradients and neurocirculatory deficits with respect to band location. No family history of band constrictions was elicited. Most of our patients were products of first pregnancies and of young mothers. Deformity of the nails was a constant finding in patients with distally located bands. All patients demonstrated at least one other anomaly, most commonly a malformation of the hand. Neural deficits were noted in 23 per cent of the patients, while circulatory findings appeared in 16 per cent. A significant temperature gradient averaging 2.38 degrees Celsius was noted in twenty patients in whom the bands were located proximally. All patients with neural deficits demonstrated a significant temperature gradient. Staged z-plasty was the procedure of choice in surgical release of constriction bands.

Of 400 patients with the diagnosis of neurofibromatosis on their hospital records, 141 actually had the disease. The presence of at least two of the following features was considered diagnostic: positive family history; positive biopsy; a minimum of six cafe-au-lait spots, each with a diameter of at least 1.5 centimeters; and multiple subcutaneous neurofibromas. Scoliosis was present in thirty-seven patients (26 per cent), most commonly associated with cafe-au-lait spots (thirty-five patients). In many of the patients with scoliosis there were associated medical and surgical problems. Although no standard pattern of spinal deformity could be identified, a sharp single right thoracic curve involving more than five vertebrae was the most common. For the whole group the initial measurement of the scoliosis averaged 42 degrees. Double curves were more sever, buth kyphosis was uncommon and no cases of paraplegia were recorded. In patients with progressive scoliosis, the best results were obtained with early Harrington instrumentation and posterior spine fusion. Progression of the scoliosis was observed both before treatment and postoperatively. The amount of progression was not necessarily related to the severity of other manifestations of neurofibromatosis, and was not significantly dependent on the length of the curve.

A survey conducted by the Scoliosis Research Society found eighty-seven patients with acute neurological complications resulting from the treatment of scoliosis. The incidence of these complications was determined to be 0.72 per cent. Seventy-four major complications involving the spinal cord were reported, half of them complete paraplegia and half partial paraplegia. Thirty-six per cent recovered completely, 32 per cent had partial recovery, and 32 per cent had no return of function. Thirteen minor complications involving cranial and peripheral nerves were reported. Major complications occurred in forty-two cases of posterior spine fusion with Harrington instrumentation and in twenty cases of posterior spine fusion without instrumentation. Six patients became paraplegic following skeletal traction alone.

Of seven patients with diastrophic dwarfism, all had cervical spina bifida occulta. Two had cervical kyphosis, which was severe in one. Scoliosis was present in five patients and was always progressive. Lumbar lordosis was seen in all seven patients and was not progressive.