Outcomes of growing rod surgery for severe compared with moderate early-onset scoliosis: a matched comparative study.

Aims: The aim of this study was to compare the outcomes of surgery using growing rods in patients with severe versus moderate early-onset scoliosis (EOS). Patients and Methods: A review of a multicentre EOS database identified 107 children with severe EOS (major curve ≥ 90°) treated with growing rods before the age of ten years with a minimum follow-up of two years and three or more lengthening procedures. From the same database, 107 matched controls with moderate EOS were identified. Results: The mean preoperative major curve was 101° (90 to 139) in the severe group and 67° (33° to 88°) in the moderate group (p < 0.001), which was corrected at final follow-up to 57° (10° to 96°) in the severe group and 40° (3° to 85°) in the moderate group (p < 0.001). T1-S1 height increased by a mean of 54 mm (-8 to 131) in the severe group and 27 mm (-4 to 131) in the moderate group at the initial surgery (p < 0.001), and by 50 mm (-17 to 200) and 54 mm (-11 to 212), respectively, during distraction (p = 0.84). The mean number of complications per patient was 2.6 (0 to 14) in the severe group and 1.9 (0 to 10) in the moderate group (p = 0.040). Five patients (4.7%) in the severe group and three (2.8%) in the moderate group developed a neurological deficit postoperatively (p = 0.47). Conclusion: Severe EOS can be treated effectively using growing rods, but the risk of complications is high. Cite this article: Bone Joint J 2018;100-B:772-9.

Arteriovenous fistula formation after a closed proximal tibial fracture in a child.

Arteriovenous fistula formation after a closed extremity fracture is rare. We present the case of an 11-year-old boy who developed an arteriovenous fistula between the anterior tibial artery and popliteal vein after closed fractures of the proximal tibia and fibula. The fractures were treated by closed reduction and casting. A fistula was diagnosed 12 weeks after the injury. It was treated by embolisation with coils. Subsequent angiography and ultrasonography confirmed patency of the popliteal vein and anterior and posterior tibial and peroneal arteries, with no residual shunting through the fistula. The fractures healed uneventfully and he returned to full unrestricted activities 21 weeks after his injury.

Surgical treatment of congenital kyphosis.

STUDY DESIGN: In this study, 26 cases of congenital kyphosis and kyphoscoliosis treated surgically were retrospectively reviewed. OBJECTIVE: To assess the clinical outcomes and surgical indications for posterior only versus anteroposterior surgery in the child. SUMMARY OF BACKGROUND DATA: Congenital kyphosis usually is progressive without surgical intervention. Current recommended treatment includes posterior arthrodesis for deformities of less than 50 degrees to 60 degrees, and anterior release or decompression, anterior fusion, and posterior instrumented arthrodesis for large deformities and cord compression. METHODS: Cases involving myelodysplasia, spinal dysgenesis, and skeletal dysplasia were excluded from the study. Kyphoscoliosis was included if the kyphotic deformity was greater than the scoliotic deformity. Patients were grouped by age and surgical technique. The patients in group P1 underwent posterior arthrodesis at an age younger than 3 years, and those in group P2 underwent the procedure at an age older than 3 years. The patients in group AP1 underwent anterior and posterior procedures at an age younger than 3 years, and those in group AP2 underwent the procedures at an age older than 3 years. The preoperative deformity, complications, and postoperative deformity correction were analyzed. There were nine Type 1 (failure of formation), nine Type 2 (failure of segmentation), and eight Type 3 (mixed) deformities. Four patients had associated spinal dysraphism. Three patients with Type 1 deformities had clinical or radiographic evidence of cord compression. RESULTS: In Group P1, five patients at an average age of 16 months underwent posterior arthrodesis alone for an average kyphotic deformity of 49 degrees. The immediate postoperative correction improved over a period of 6 years and 9 months by an additional 10 degrees, resulting in a final deformity of 26 degrees. Pseudarthrosis developed in two patients, requiring fusion mass augmentation or anterior arthrodesis. Neither patient was instrumented. In Group P2, five patients at an average age of 13 years and 7 months underwent posterior arthrodesis with instrumentation for kyphotic deformity of 59 degrees. Approximately 30 degrees of intraoperative correction was achieved safely using compression instrumentation and positioning. No further correction occurred with growth. The final residual kyphotic deformity was 29 degrees after a follow-up period of 4 years and 5 months. In Group AP1, seven patients underwent anterior release or vertebra resection for deformity correction and posterior arthrodesis for an average kyphotic deformity of 48 degrees at the age of 16 months. There were no iatrogenic neurologic injuries. The final residual kyphotic deformity was 22 degrees after a follow-up period of 6 years and 3 months. In Group AP2, nine patients underwent anterior release or decompression with posterior arthrodesis for kyphotic deformity of 77 degrees at the age of 11 years and 6 months. The deformity was corrected to 37 degrees, with no significant loss over a follow-up period of 5 years and 2 months. There were two postoperative neurologic complications. CONCLUSIONS: After reviewing their experience, the authors made the following observations: 1) The pseudarthrosis rate was low even without routine augmentation of fusion mass if instrumentation was used; 2) gradual correction of kyphosis may occur with growth in patients younger than 3 years with Types 2 and 3 deformities after posterior fusion, but appears to be unpredictable; 3) the risk of neurologic injury with anterior and posterior fusion for kyphotic deformity was associated with greater age, more severe deformity, and preexisting spinal cord compromise.

Early failure of Pavlik harness treatment for developmental hip dysplasia: clinical and ultrasound predictors.

A cohort of 93 patients with developmental dysplasia of the hip (DDH) treated with a Pavlik harness were evaluated to determine predictors of treatment failure. Failure was defined as failure to achieve or maintain hip reduction in the Pavlik harness. Of 93 patients (137 hips), 17 (26 hips) failed Pavlik harness treatment. Univariate risk factors for failure included bilaterality, initial clinical exam, and initial ultrasound (US) percent coverage. Clinical exam and initial percent coverage were multivariate risk factors for failure. Among initially clinically dislocatable hips, a low initial US alpha angle correlated with an increased likelihood of failure. All (6/6) patients with an initially irreducible hip and an initial coverage of <20% by US eventually failed treatment. Gender, side of pathology, and age at diagnosis and initiation of treatment did not correlate with failure. Irreducibility by physical exam combined with US coverage of <20% identified a patient group that uniformly failed Pavlik harness treatment. These patients may be candidates for alternative bracing, traction, or closed or open reduction.

Role of flexion-extension radiographs in blunt pediatric cervical spine injury.

OBJECTIVE: To determine whether flexion-extension cervical spine radiography (FECSR) is abnormal in children who have sustained blunt cervical spine injury (CSI) when standard cervical spine radiography (SCSR) demonstrates no acute abnormalities. METHODS: This was a blinded radiographic review of 129 patients < or = 16 years of age evaluated at an academic pediatric trauma center during July 1990-March 1996. All patients had SCSR (anteroposterior/lateral views) and FECSR performed for a trauma-related event within seven days of injury. RESULTS: Of 46 patients without acute abnormalities on SCSR, one patient (with final clinical diagnosis of "no CSI") had acute abnormalities on FECSR (95% CI = 0.06% to 11.5%). Of 50 patients with isolated loss of lordosis on SCSR, no patient had acute abnormalities on FECSR (95% CI = 0% to 5.8%). The FECSR review revealed no acute abnormalities in 75 of 83 patients (90.4%) with suspicious findings for CSI viewed on SCSR (95% CI = 81.9% to 95.7%). Complications during FECSR were noted in one patient with transient paresthesias (0.8%) (95% CI = 0.02% to 4.2%). CONCLUSIONS: In children who underwent acute radiographic evaluation of blunt cervical spine trauma, FECSR was unlikely to be abnormal when no acute abnormality or isolated loss of lordosis was evident on SCSR. In a subset of patients with suspicious findings for occult CSI on SCSR, FECSR was useful in ruling out ligamentous instability in the acute, posttrauma setting.

Spinal fusion in patients with congenital heart disease. Predictors of outcome.

The strong association between congenital heart disease and spinal deformity is well established, but data on the risks and outcome of spinal fusion surgery in patients with congenital heart disease are scarce. The purpose of this study was to identify predictors of perioperative risk and outcome in a large series of children and adolescents with congenital heart disease who underwent spinal fusion for scoliosis or kyphosis. In the authors' retrospective analysis of 74 consecutive patients with congenital heart disease undergoing spinal fusion, there were two deaths (2.7%) and 18 significant complications (24.3%) in the perioperative period. Preoperative cyanosis (arterial oxygen saturation < 90% at rest) with uncorrected or incompletely corrected congenital heart disease was associated with both deaths. Complications occurred in nine of 18 (50%) patients with cyanosis and in 11 of 56 (20%) patients without cyanosis. As judged by multivariate analysis the best predictors of perioperative outcome were the overall physical status of the patient as represented by the American Society of Anesthesiologists' preoperative score and a higher rate of intraoperative blood loss. Seventeen of 43 patients (40%) with an American Society of Anesthesiologists score of 3 or higher experienced complications including two perioperative deaths. Successful spinal fusion and correction were achieved in 97% of patients. Children and adolescents with congenital heart disease can undergo elective spinal fusion with risks that relate to overall cardiac status. Careful assessment of preoperative status by pediatric cardiologists and cardiac anesthesiologists familiar with surgical treatment of patients with congenital heart disease will assist the orthopaedic surgeon in providing the most realistic estimate of risk.

Occipitocervical fusions in children. Retrospective analysis and technical considerations.

This report presents a retrospective analysis of the authors' experience with occipitocervical fusions in children and adolescents during the last 2 decades. A description of an operative technique devised by the senior author (JEH), and a comparison of the results using this and other methods of fusion are given. Twenty-three patients underwent occipitocervical fusion. Fifteen of the patients were operated on using the authors' technique. To achieve stable fixation of the distal cervical vertebra a threaded Kirschner wire was passed transversely through the spinous process; occipital fixation was achieved by the traditional method of wiring corticocancellous bone graft to the skull through burr holes. The occipital wires then were wrapped around the Kirschner wire and the graft was cradled in the resulting nest. Halo immobilization was used in 10 patients for an average of 12.5 weeks (range, 6-24 weeks). Twenty-two patients achieved successful fusion at an average followup of 5.8 years (range, 1-14.33 years). Several complications, including transient quadriplegia in one patient, pseudarthrosis in two (one of which persists), hardware fixation failure in one, unintended distal extension of the fusion, pneumonia, wound infection, halo pin infection, skin breakdown under the halo vest, hydrocephalus, cerebrospinal fluid leak, and traumatic fusion fracture were encountered. Results using the technique described herein are comparable with or better than the results reported in the previous literature, and the results of the patients in this series in whom the technique was not used.

Adverse urologic consequences of spinal cord resection at the time of kyphectomy: value of preoperative urodynamic evaluation.

Four children with thoracic level paraplegia and severe myelokyphosis underwent distal spinal cord resection at the time of kyphectomy. All four children were continent before spinal cord resection and became incontinent immediately afterward. Two children in retrospect had preoperative urodynamics that showed intact innervation to the external sphincter, and two had no preoperative urodynamic studies. All four had evidence of lower urinary tract denervation on urodynamic studies performed after cord resection. The urologic and urodynamic consequences of spinal cord resection at the time of kyphectomy in children with myelodysplasia are reviewed. Resection of the distal spinal cord in thoracic level myelodysplasia may cause postoperative incontinence in some previously continent patients. Preoperative urodynamic evaluation and urologic consultation is recommended. If individual evaluation indicates that residual sacral function is beneficial to urologic management, kyphectomy without cord resection is preferable.

Spinal deformity in myelodysplasia. Correction with posterior pedicle screw instrumentation.

STUDY DESIGN: A retrospective review of transpedicular instrumentation used in a series of 24 patients with myelodysplastic spinal deformities and deficient posterior elements. OBJECTIVE: To describe the usefulness and efficacy of these instruments in the treatment of complicated myelodysplastic spinal deformity. METHODS: The mean preoperative scoliosis was 75.7 degrees (range, 39-130 degrees) in the 22 patients with scoliotic deformities; 4 patients with thoracic hyperkyphoses averaged 70.5 degrees (range, 46-90 degrees) and 10 patients with lumbar kyphoses averaged 80.5 degrees (range, 42-120 degrees). The instrumentation extended to the sacrum in 4 patients and the pelvis in 9; 10 patients also underwent anterior release and fusion and 7 underwent concomitant spinal cord detethering. At an average follow-up of 4.0 years (2.0-7.7 years; one patient died at 8 months), all patients have fused (with the exception of two lumbosacral pseudarthroses). RESULTS: At last follow-up, deformity measured 32.1 degrees scoliosis (range, 6-85 degrees), 30.8 degrees thoracic kyphosis (range, 24-35 degrees), and 0.0 degree lumbar kyphosis (range, 35 degrees kyphosis to 29 degrees lordosis). Three patients lost some neurologic function after surgery; two recovered within 6 months and one has incomplete recovery. No ambulatory patient lost the ability to walk. Five patients required additional surgical procedures; in three cases, there was instrumentation breakage associated with pseudarthrosis or unfused spinal segments. CONCLUSIONS: Pedicle screw instrumentation is uniquely suited to the deficient myelodysplastic spine. Compared with historical control subjects, these devices have proven capable of significant correction of both scoliotic and kyphotic deformities. This instrumentation appears particularly useful in preserving lumbar lordosis in all patients and may preserve more lumbar motion in ambulatory myelodysplasia patients.

Lumbar pedicle screws versus hooks. Results in double major curves in adolescent idiopathic scoliosis.

STUDY DESIGN: A retrospective assessment of the effectiveness of lumbar pedicle screws versus laminar hooks in lumbar curve correction with double major curves in adolescent idiopathic scoliosis. OBJECTIVE: To determine if pedicle screw fixation of the lumbar spine has any advantage compared with multiple laminar hook instrumentation in the treatment of double major curves in adolescent idiopathic scoliosis. SUMMARY OF BACKGROUND DATA: Although hooks have been used most commonly, pedicle screws may offer advantages in correction and maintenance of reduction of the lumbar curve in adolescent idiopathic scoliosis. METHODS: A consecutive series of 39 patients with double major curves underwent thoracic and lumbar instrumentation by a single surgeon. Lumbar pedicle screws and hooks were used in 20 patients (Group S) and in 19 patients only lumbar hooks were used (Group H). Thoracic Cotrel-Dubousset instrumentation with hooks was the same in both groups. Preoperative age, gender, bracing, and Cobb angles were similar in both groups. Preoperative, 1-month postoperative, and latest follow-up standing posteroanterior and lateral spine radiographs were blinded to the surgeon and lumbar instrumentation covered to hide its identity. Measurements included Cobb angles, preoperative flexibility, lumbar and thoracic apical vertebral deviation, and reduction of lateral tilt and lateral displacement of the first free lumbar vertebra below the instrumentation. Percent correction, maintenance of correction at follow-up, and total levels fused were calculated. RESULTS: The mean follow-up was 3.5 years (range, 2-8 years), which was similar for Groups H and S. Pedicle screws appear to offer some advantage in lumbar curve correction, maintenance of correction, and correction of the uninstrumented spine below the fusion when compared with the use of hooks alone. Horizontalization of the first free lumbar vertebra below the instrumentation percent correction of tilt: 62% screws vs. 11% hooks; P = 0.0003), residual tilt (8 degrees screws vs. 17 degrees hooks; P = 0.004), and loss of horizontalization at follow-up (5% screws vs. 26% hooks) were dramatically better for the group using screws. Lumbar curve correction (72% screws vs. 60% hooks; P = 0.026), loss of lumbar curve correction (5% screws vs. 13% hooks), and correction of lateral apical vertebral deviation (2.2-cm screws vs. 1.5-cm hooks or 63% vs. 31%; P = 0.013) were better when screws were used. There was no significant difference in loss of correction of the thoracic curves (35% vs. 37%) or any difference in loss of correction of lateral displacement of the thoracic apical vertebra (12% vs. 14%). There was no difference in total levels fused, operative blood loss, operative time, or ultimate patient outcome. No patients in either group had spinal imbalance at latest follow-up. There were no complications related to pedicle screw placement. Two cases of transient postoperative superior mesenteric artery syndrome (duodenal obstruction by the superior mesenteric artery) in the pedicle screw group are attributed to acute correction of the lumbar scoliosis and thoracolumbar kyphosis with resultant lordosis at the thoracolumbar junction. CONCLUSIONS: Lumbar pedicle screws may offer greater lumbar curve correction, better maintenance of correction, and greater correction of the uninstrumented spine below double major curves. No complications were associated with the placement of pedicle screws.

Surgery of the spine in myelodysplasia. An overview.

Significant spinal deformity is particularly common in nonambulatory patients with myelodysplasia. Progressive deformity may be caused by congenital anomalies, paralytic collapse, hip contractures, or spinal cord tethering. Existing or projected functional impairment should be the principle indication for treatment. Surgical treatment is complicated by poor soft tissue coverage, associated contractures, lack of sensation, weak bone, and absence of posterior elements. Successful fusion can be achieved by circumferential (anterior and posterior) fusion and current rigid segmental instrumentation. The unique deformities and bony anatomy require individualized techniques to achieve fixation.

A meta-analysis of the efficacy of non-operative treatments for idiopathic scoliosis.

With use of data culled from twenty studies, members of the Prevalence and Natural History Committee of the Scoliosis Research Society conducted a meta-analysis of 1910 patients who had been managed with bracing (1459 patients), lateral electrical surface stimulation (322 patients), or observation (129 patients) because of idiopathic scoliosis. Three variables - the type of treatment, the level of maturity, and the criterion for failure - were analyzed to determine which had the greatest impact on the outcome. We also examined the effect of the type of brace that was used and the duration of bracing on the success of treatment. The number of failures of treatment in each study was determined by calculating the total number of patients who had unacceptable progression of the curve (as defined in the study), who could not comply with or tolerate treatment, or who had an operation. The percentage of patients who completed a given course of treatment without failure, adjusted for the sample sizes of the studies in which that treatment was used, yielded the weighted mean proportion of success for that treatment. The weighted mean proportion of success was 0.39 for lateral electrical surface stimulation, 0.49 for observation only, 0.60 for bracing for eight hours per day, 0.62 for bracing for sixteen hours per day, and 0.93 for bracing for twenty-three hours per day. The twenty-three-hour regimens were significantly more successful than any other treatment (p < 0.0001). The difference between the eight and sixteen-hour regimens was not significant, with the numbers available. Although lateral electrical surface stimulation was associated with a lower weighted mean proportion of success than observation only, the difference was not significant, with the numbers available. This meta-analysis demonstrates the effectiveness of bracing for the treatment of idiopathic scoliosis. The weighted mean proportion of success for the six types of braces included in this review was 0.92, with the highest proportion (0.99) achieved with the Milwaukee brace. We found that use of the Milwaukee brace or another thoracolumbosacral orthosis for twenty-three hours per day effectively halted progression of the curve. Bracing for eight or sixteen hours per day was found to be significantly less effective than bracing for twenty-three hours per day (p < 0.0001).

Segmental spinal dysgenesis: early neurologic deterioration and treatment.

Segmental spinal dysgenesis is a rare congenital condition of the lumbar or thoracolumbar spine that can be associated with significant progressive or permanent neurologic defects, including paraplegia. In the past, both bracing and surgery have been recommended. Of the seven children we have seen with this problem, three have lost motor function between presentation and the time of surgery. Our experience suggests that to prevent catastrophic neurologic deterioration, surgery is indicated once the diagnosis is made.

Increasing lordosis of the occipitocervical junction after arthrodesis in young children: the occipitocervical crankshaft phenomenon.

Five children were treated before age 6 years with occipitocervical fusion for occipitocervical instability. Long-term (average, 11.8 years; range, 8.4-14.5 years) follow-up revealed increasing lordosis across the fused segment in four of the patients, a finding we here refer to as the occipitocervical crankshaft phenomenon. On average, occipitocervical lordosis increased 1.06 degrees per level fused per year until skeletal maturity. Although such a progression might be expected, to our knowledge this is the first report of its occurrence. Compensatory subaxial motion was able to overcome this increase in all of the patients. We recommend occipitocervical fusion in a neutral or slightly flexed position in the very young child to account for this predictable increase in lordosis.

Chronic physeal fractures in myelodysplasia: magnetic resonance analysis, histologic description, treatment, and outcome.

Thirteen myelodysplastic children with 19 chronic physeal fractures were treated. All were treated with prolonged immobilization (average, 5.8 months; range, 3-18 months) in either braces or casts; four of the fractures required operative fixation to facilitate healing. All were healed at 4.8-years follow-up but, in four of the fractures, the growth plate closed prematurely. Three of the children underwent magnetic resonance imaging (MRI) of the injured physes, and one underwent physeal biopsy as part of her operative epiphysiodesis. Histologic analysis revealed three distinct zones of physeal pathoanatomy: a normal zone of proliferation; a thickened, disorganized zone of hypertrophy; and a vascularized zone of fibrous tissue adjacent to the metaphysis. On MRI, there was thickening of the physis and irregularity of the zone of provisional calcification. The physeal cartilage and the juxtametaphyseal fibrovascular tissue enhanced with gadolinium. These findings corroborate earlier mechanistic proposals for physeal injury in myelodysplasia: chronic stress or trauma to the poorly sensate limb produces micromotion at the zone of hypertrophy, yielding a widened, disorganized physis, and leading to fracture, displacement, and delayed union.

A prospective evaluation of idiopathic left thoracic scoliosis with magnetic resonance imaging.

Twenty-nine consecutive patients with idiopathic left thoracic scoliosis were prospectively studied using magnetic resonance imaging (MRI). T1-weighted sagittal and axial images were obtained on all patients from the brainstem to the tip of the conus. Two patients (7%) had a syrinx on MRI. The remaining 27 patients had normal MRIs. The prevalence of brainstem and spinal cord anomalies was much less common than reported in previous retrospective reviews.

The Pavlik harness and developmental dysplasia of the hip: has ultrasound changed treatment patterns?

Monitoring Pavlik harness therapy for developmental dysplasia of the hip (DDH) has been used at our institution since 1986. In this study, we compared Pavlik therapy for DDH without ultrasound monitoring (group A) to similar therapy with ultrasound monitoring (group B). Both groups had similar age at diagnosis. Treatment failure was recognized earlier in group B than in group A (4.9 vs. 9.3 weeks). The total number of radiographs was significantly decreased in group B. The duration of therapy was less in group B than in group A. Successfully treated hips had an average increase in alpha angle of 8.4 degrees per month. The average failure rate in hips resting in a dislocated position at the onset of Pavlik therapy was unchanged by Pavlik monitoring.

Progressive kyphosis and neurologic compromise complicating spondylothoracic dysplasia in infancy (Jarcho-Levin syndrome).

STUDY DESIGN: This is a case report and review of the literature. OBJECTIVE: To review the orthopedic literature regarding the spinal abnormalities found with spondylothoracic dysplasia, and to present the history and management of severe kyphosis with neurologic impairment in an infant with spondylothoracic dysplasia. SUMMARY OF BACKGROUND DATA: Spondylothoracic dysplasia (Jarcho-Levin syndrome) is a rare form of short-limbed dwarfism characterized by extensive vertebral and chest-wall abnormalities. Although the spinal anomalies generally are radiographically severe, no patient previously reported in the English orthopedic literature has required surgical stabilization. METHODS: The clinical findings, course, and surgical management of an infant with spondylothoracic dysplasia and severe congenital kyphosis are presented. This patient required anterior decompression and anterior/posterior spinal fusion to obtain stabilization. The follow-up from the index surgical procedure was 2 years. RESULTS: The patient underwent an initial anteroposterior fusion and decompression at 5 months of age. She underwent revision of both anteroposterior procedures 7 months later because of pseudarthroses and a progressive kyphosis. Her residual neurologic abnormality was minimal and she appeared to have a stable fusion at 24 months post-index procedure. CONCLUSIONS: The spinal abnormalities associated with spondylothoracic dysplasia may not be as benign as previously reported. Sagittal, as well as frontal, plane deformities may exist secondary to multiple hemivertebrae. Longer life span, possibly due to improved pulmonary care, may increase the incidence of deformities requiring intervention.