Orthopaedic manifestations of familial dysautonomia. A review of one hundred and thirty-six patients

BACKGROUND: Familial dysautonomia is a hereditary multisystemic disease primarily affecting people of Ashkenazi Jewish descent. Musculoskeletal problems are related to gait disorders, spinal deformities, foot deformities, fractures, and arthropathies. METHODS: The charts and radiographs of 136 patients who ranged in age from three months to forty-six years (mean, sixteen years) were reviewed. Sixty-four patients were available for follow-up examination. RESULTS: Spinal deformity was the most common orthopaedic problem and was diagnosed in seventy-eight patients starting at the age of four years, with a prevalence of 86 percent (forty-eight of fifty-six) by the age of fifteen years. Forty-one (53 percent) of the seventy-eight patients had scoliosis only, thirty-four (44 percent) had kyphoscoliosis, and three (4 percent) had kyphosis only. Bracing was accompanied by emotional, pulmonary, and skin problems, leading to a high rate of noncompliance and progression of the curve. Twenty-four patients had an operation at a mean age of thirteen years (range, five to eighteen years): twenty patients had posterior spinal arthrodesis, and four had combined anterior and posterior arthrodesis. Fifteen patients had a total of nineteen complications, of which seven were systemic and twelve were related to the spinal fixation. Eight patients had revision surgery. At the time of the surgery, scoliosis was corrected from a mean of 55 degrees to a mean of 35 degrees and kyphosis was corrected from a mean of 69 degrees to a mean of 61 degrees. After a mean duration of follow-up of sixty-five months, scoliosis measured 49 degrees (range, 18 to 62 degrees) and kyphosis measured 67 degrees (range, 30 to 115 degrees). Postoperative progression of the deformity was caused by failure of the instrumentation or progression in unfused segments. Walking was delayed in 72 percent (ninety-four) of the 130 patients who were of walking age. All sixty-four of the patients who were examined had an ataxic gait. Foot deformities were found in sixteen patients, six of whom were treated surgically. Two patients had Charcot joints. Fifty-five patients sustained at least one fracture before skeletal maturity, with a mean of 1.5 fractures per patient. All but one of the fractures was treated nonoperatively, and fracture-healing was often accompanied by profuse callus formation. CONCLUSIONS: Spinal deformity is common in patients with familial dysautonomia. Bracing is of questionable benefit, and surgical intervention should be considered once curve progression is well documented. Arthrodesis should be extended as far proximally as possible to prevent junctional kyphosis. Swelling and warmth in a limb should raise suspicion of an undiagnosed fracture.     

Progression of scoliosis in Duchenne muscular dystrophy

We reviewed the clinical charts and spinal radiographs of fifty-one boys who had Duchenne muscular dystrophy, had not had surgical treatment of the spine, and had been followed until death. All had scoliosis. None of the following variables was useful in predicting which curves would become severe: age when the patient initially walked, age when he ceased walking, age at onset of spinal collapse, surgical release of the iliotibial bands, or age at the time of death. Radiographs were made within eighteen months before death for thirty-three patients; in thirty-one of them, the final curve exceeded 40 degrees and in seventeen, 90 degrees. For the remaining eighteen patients, final radiographs were made more than eighteen months before death; at that time, eight of them already had a curve of more than 90 degrees. Although there was a relationship between extension of the lumbar spine and severity of scoliosis at the time of final follow-up, early maintenance of the lumbar spine in extension rarely prevented the development of a severe curve. For most of the patients who had a severe curve, sitting was difficult and was accompanied by breakdown of the skin and pain. When a patient's curve exceeded 35 degrees, the vital capacity usually was less than 40 per cent of the predicted normal value. Therefore, when walking becomes impossible for boys who have Duchenne muscular dystrophy, routine spinal arthrodesis should be considered.     

Comparison of survival analysis between surgical and non-surgical treatments in Duchenne muscular dystrophy scoliosis

BACKGROUND CONTEXTThere are still controversies about the effects of spinal surgeries for Duchenne muscular dystrophy (DMD) scoliosis on functional outcome, respiratory function, and the survival rate.PURPOSEThe purpose of this retrospective investigation was to compare the clinical course over time between the patients who were treated surgically and those who were treated nonsurgically. Through this comparison, we tried to determine how surgical treatment could affect the functional status, pulmonary function, and survival rate in patients with DMD scoliosis.STUDY DESIGN/SETTINGSingle-center retrospective cohort study.PATIENT SAMPLEWe reviewed the clinical data of 199 male patients with DMD scoliosis who were followed up at our center for an average of 6.4 years between 2003 and 2017.OUTCOME MEASURESThe basic radiologic parameters evaluated include the Cobb angle and pelvic obliquity on a whole spine X-ray. Further, the Swinyard scale for functional status, forced vital capacity (FVC) for respiratory function, and mortality were compared between the surgical group and nonsurgical group.METHODSThe radiologic parameters and Swinyard scale stage were compared between the surgical group and nonsurgical group at baseline and 2, 5, and 10 years. For the FVC, serial changes every year were investigated in both groups. Mortality was surveyed between the surgical group and nonsurgical group.RESULTSOf the 199 patients, 99 patients underwent the instrumented spinal fusion surgery and 100 patients in the nonsurgical group opted for conservative management. Radiologic results of the two groups were not different at baseline, but during the follow-up periods, the surgical group demonstrated better Cobb angles and pelvic obliquities. The surgical group showed a better functional status than did the nonsurgical group (6.7±0.9 versus [vs.] 7.2±0.7, p<.001). These functional differences between the groups were continuously observed during the follow-up period. Similarly, the FVC at baseline was higher in the surgical group than in the nonsurgical group (1005.7±421.4 mL vs. 787.3±574.1 mL, p=.005). Although FVC in the nonsurgical group consistently decreased during the follow-up (4.8% decrease/year), FVC in the surgical group increased up to the 2-year follow-up period compared with the baseline value and decreased during the follow-up period (2.8% decrease/year). Mortality was higher in the nonsurgical group than in the surgical group (n=22/100, 22.0% vs. n=8/99, 8.1%; p<.001) during an average follow-up duration of 6.4 years. Mean survival was longer in the surgical group than in the nonsurgical group (12.2 years vs. 8.3 years, hazard ratio=2.43, p=.02).CONCLUSIONSSpinal surgery for DMD scoliosis improved the FVC for approximately 2 years postoperatively compared to non-surgical treatment. The surgical group had a better functional status and FVC at baseline than the non-surgical group. The positive effect of surgical treatment on the FVC is owing to scoliosis correction, which delayed the decrease of FVC and consequently extended the survival rate of the patients with DMD scoliosis.     

Superior mesenteric artery syndrome in children and adolescents with spine deformities undergoing corrective surgery

OBJECTIVE: Obstruction of the third part of the duodenum by the superior mesenteric artery (SMA) is associated with spinal manipulation in the surgical or conservative management of scoliosis. The purpose of the present study was to investigate the prevalence of SMA syndrome in a cohort of 165 consecutive pediatric patients who underwent spine deformity surgery and had minimum 2-year follow-up. METHODS: The study group comprised 85 patients with idiopathic scoliosis, 20 patients with neuromuscular and 18 patients with miscellaneous or syndromic scoliosis, and 42 patients with congenital spine deformities. Posterior spine arthrodesis was performed in 94 patients, combined anteroposterior in 60 patients, and anterior spinal fusion in 11 patients. RESULTS: We identified four patients who developed SMA syndrome postoperatively. These were all markedly underweight adolescent girls; two patients had adolescent idiopathic scoliosis, one had neuromuscular scoliosis, and one congenital scoliosis. Third-generation instrumentation systems with derotational effect were used in three patients. The spine arthrodesis in the patient with neuromuscular scoliosis was performed using bone graft followed by application of a spinal jacket. The symptoms developed at a mean of 3.7 days post surgery and included nausea, vomiting, increased nasogastric aspirates, and abdominal pain and distension. Conservative management with prolonged nasojejunal feedings achieved resolution of the symptoms in all but one patient, who required derotation of the duodenum and jejunum. There was no evidence of recurrence of the condition in any patient. The prevalence of SMA syndrome in our series was 2.4%. CONCLUSIONS: This work draws attention to the significance of the prevention of the condition by recognizing patients who are at a higher risk. An early diagnosis of SMA syndrome will allow for application of conservative methods and will increase the chances for a successful outcome.     

The treatment of scoliosis in cerebral palsy by posterior spinal fusion with Luque-rod segmental instrumentation

Thirty-three patients who had cerebral palsy and scoliosis underwent posterior spinal fusion with Luque-rod segmental instrumentation. The mean curve measured 65 degrees preoperatively and 30 degrees postoperatively. The mean loss of correction was 3 degrees after a mean length of follow-up of forty months. As of the time of writing, pseudarthrosis had not developed in any patient. No patient had a broken rod or an increased neurological deficit. There were no major pulmonary complications. The most common early postoperative complication was wound infection, which occurred in five patients, none of whom required removal of the hardware. We concluded that posterior spinal fusion with Luque-rod segmental instrumentation is an effective method of obtaining and maintaining correction of a spinal curve, with minimum complications, in patients who have cerebral palsy and progressive scoliosis.     

Pulmonary function before and after anterior spinal surgery in adult idiopathic scoliosis.

BACKGROUND: Little is known about the long term effects of anterior spinal surgery on pulmonary function in adult patients with idiopathic scoliosis. A study was therefore undertaken of pulmonary function before and after anterior spinal surgery in this group of patients. METHODS: Fourteen patients (12 women) of mean age 26.5 years (range 17-50, 10 > or = 20 years) were studied. All 14 patients underwent thoracotomy and anterior arthrodesis, and five also underwent posterior arthrodesis. Scoliosis curves and lung volumes were measured preoperatively and at a mean follow up of 32 months (range 14-49) after surgery. RESULTS: At long term follow up after surgery the mean (SD) Cobb angle of the thoracic curve improved from 59 degrees (25 degrees) to 39 degrees (18 degrees), a correction of 31%. Despite this there was a fall in mean forced vital capacity (FVC) of 0.21 litres (95% confidence interval 0.04 to 0.38). In the patients who underwent anterior surgery without posterior surgery (n = 9) there was a fall in mean FVC of 0.31 litres (95% confidence interval 0.12 to 0.50); mean forced expiratory volume in one second and total lung capacity were also reduced and there was an increase in mean residual volume. CONCLUSIONS: Forced vital capacity is reduced at long term follow up in adult patients with idiopathic scoliosis who undergo anterior spinal surgery. The fall in FVC is small and is unlikely to be of clinical significance in patients with reasonable lung function in whom surgery is planned for prevention of curve progression or improvement of cosmetic appearance and pain. However, surgical intervention should not be undertaken in an attempt to improve pulmonary function.     

Management of neuromuscular spinal deformities with Luque segmental instrumentation

Forty-six patients who had a neuromuscular spinal deformity were treated with arthrodesis and Luque segmental spinal instrumentation and were followed for an average of three years. Twenty-two patients had cerebral palsy and twenty-four had another neuromuscular disease. In thirty-nine patients, the arthrodesis was extended to the sacrum. Eleven patients who had severe scoliosis as well as pelvic obliquity and decompensation of the torso had a combined anterior and posterior arthrodesis; the other thirty-five patients had a one-stage posterior procedure. Preoperatively, the average scoliosis was 74 degrees; this was corrected to 39 degrees at follow-up. Final corrections were similar for scoliosis and were better for pelvic obliquity and decompensation of the torso in patients who had combined anterior and posterior arthrodesis. The results for scoliosis and pelvic obliquity in patients who had a spastic deformity were similar to the results in patients who had a flaccid deformity. Correction of decompensation of the torso was better in patients who had a spastic deformity. Postoperatively, a brace was used in half of the patients in each group; this did not appear to affect the amount of correction in either group, although the result may have been influenced by the selection process. The rate of complications was 48 per cent. Pseudarthrosis occurred in three patients (6.5 per cent). There were no major neurological deficits related to the correction or to the use of sublaminar wires. Three patients died, one in the immediate postoperative period and the other two at eighteen months and four years after the original procedure, due to causes unrelated to the operation.     

神经纤维瘤病性脊柱侧凸的治疗

目的回顾性研究45例神经纤维瘤病性脊柱侧凸患者的外科治疗结果,分析侧凸分类与手术效果的关系。方法45例神经纤维瘤病性脊柱侧凸患者,平均年龄14.2岁,平均随访时间6.8年。其中6例为非营养不良型侧凸,39例为营养不良型侧凸。后者按照顶椎位置分为3组:胸弯组、胸腰弯组、单纯腰弯组。根据侧凸类型和位置选择后路脊柱融合或前后路脊柱融合。所有病例行完整的影像学检查,并进行侧弯角度与手术效果之间关系的评估。结果术中平均出血600 m l,平均输血670 m l。在非营养不良组冠状面和矢状面的矫正率分别为61.77%和40.19%,随访平均Cobb角丢失分别为2.9°和5.2°。营养不良组冠状面和矢状面的矫正率胸弯组分别为48.91%和47.74%,随访平均Cobb角丢失分别为4.8°和3.6°;胸腰弯组分别为58.40%和52.00%,随访平均Cobb角丢失分别为6.3°和5.0°。腰弯组冠状面矫正率为65.10%,随访平均Cobb角丢失为12.8°。在45例患者中有6例随访时出现大于10°的角度丢失,3例出现内固定失败,6例进行了共7次翻修手术。结论神经纤维瘤病性脊柱侧凸治疗较为复杂,难度较大。早期积极的手术治疗,是治疗此类侧凸尤其是营养不良型NF-1型侧凸的关键。     

Pulmonary function in adolescent idiopathic scoliosis relative to the surgical procedure

BACKGROUND: The long-term pulmonary function of patients with adolescent idiopathic scoliosis undergoing surgical correction is uncertain. To our knowledge, no report has demonstrated the changes in pulmonary function five years or more following spinal arthrodesis with use of modern segmental spinal instrumentation techniques for the treatment of all types of adolescent idiopathic scoliosis in a similar adolescent population. METHODS: One hundred and eighteen patients with adolescent idiopathic scoliosis undergoing surgical treatment at a single institution were evaluated with pulmonary function tests to assess the absolute and percent-predicted value of forced vital capacity and forced expiratory volume in one second at the preoperative examination and at regular intervals postoperatively. The patients were divided into four groups depending upon the surgical procedure: Group 1 comprised forty-nine patients who had posterior spinal arthrodesis with iliac crest bone graft; Group 2, forty-one patients who had posterior spinal arthrodesis with thoracoplasty; Group 3, sixteen patients who had open anterior spinal arthrodesis with a rib resection thoracotomy; and Group 4, twelve patients who had combined anterior and posterior spinal arthrodesis with a rib resection thoracotomy and iliac crest bone graft, respectively. RESULTS: A comparison of absolute pulmonary function values from the preoperative and final follow-up evaluations demonstrated a significant (p < 0.0001) increase in both the forced vital capacity and the forced expiratory volume in one second for Group 1, whereas no change was seen in those values for Groups 2, 3, and 4. A comparison of the changes in the percent-predicted pulmonary function values demonstrated significant (p < 0.05) decreases in forced vital capacity and forced expiratory volume in one second for Groups 2, 3, and 4, except for the latter value for Group 4, whereas Group 1 had no change. CONCLUSIONS: Patients who have had any type of chest cage disruption during the surgical treatment of adolescent idiopathic scoliosis demonstrate no change in the absolute value and a significant decline in the percent-predicted value of pulmonary functions at five years following surgery. Chest cage preservation is recommended to maximize both absolute and percent-predicted pulmonary function values after surgical treatment of adolescent idiopathic scoliosis.     

The associated effects of untreated unilateral hip dislocation in cerebral palsy scoliosis

INTRODUCTION: The presence of a unilateral hip dislocation in children with cerebral palsy (CP) may cause problems with sitting imbalance, pressure ulcers, and hip pain. There is a dynamic interplay between hip dislocation, pelvic obliquity (PO), and scoliosis. The effect of an untreated unilateral hip dislocation on the rate of curve progression of CP scoliosis has not been defined in the literature. The purpose of this study is to investigate the effect of unilateral hip dislocation on PO and the rate of curve progression in children with spastic quadriplegic CP. METHODS: Patients with spastic quadriplegic CP who had spine radiographs at the time of initial presentation with scoliosis and at the latest follow-up were evaluated. Twenty-three children with spastic CP who had an untreated unilateral hip dislocation and scoliosis constituted the study group. The control group consisted of 83 quadriplegic CP patients with scoliosis and well-located hips. The rate of curve progression, incidence of PO, and the rate of PO progression at follow-up were compared between the 2 groups. RESULTS: The mean ages of patients with a unilateral dislocation and with well-located hips at initial radiograph were 10.4 and 10.5 years, respectively. The mean follow-up was 3.5 years. The mean rate of scoliosis curve progression in patients with a unilateral hip dislocation was 12.9 degrees per year. In the control group, the mean progression rate was 12.2 degrees per year. The incidence of PO at follow-up was 74% in scoliotic patients with a dislocation and 63% in scoliotic patients with normal hips. Using repeated-measures analysis of variance, unilateral hip dislocation was found to have no significant effect on scoliosis progression; however, progression of PO was significantly increased in the hip dislocation group (P < 0.05). Pelvic obliquity was corrected after posterior spinal fusion to the sacrum with pelvic fixation, without reducing the hip(s) at the same surgery. CONCLUSION: Unilateral hip dislocation causes a significant increase of PO but does not affect the rate of scoliosis curve progression.     

The effect of spinal fusion on the long-term outcome of idiopathic scoliosis. A case-control study.

We have investigated the effect of multisegmental spinal fusion on the long-term functional and radiological outcome in patients with scoliosis. We compared these patients both with those whose spine had not been fused, and with a control group. We studied 68 patients with idiopathic scoliosis (34 operative and 34 non-operative) who had been followed up for a minimum of five years after treatment. They were matched for age (mean 44 years) and Cobb angle (mean 54 degrees) at follow-up. An age- and gender-matched control group of 34 subjects was also recruited. All participants completed a questionnaire to assess spinal function and to grade the severity of back pain using a numerical rating scale. Radiographs of the spine were taken in the patients with scoliosis and lumbar degenerative changes were recorded. The spinal function scores for the patients with scoliosis who had had a fusion were similar to those who had not. Both scoliosis groups, however, had lower scores than the control group (p < 0.001). The frequency and severity of back pain were lower for patients with scoliosis and fusion than for those without, but higher for both scoliosis groups compared with the control group. Radiographs showed similar degenerative changes in both scoliosis groups.     

ISIS scanning: a useful assessment technique in the management of scoliosis

The value of surface topographical measurements in the assessment of curve progression in adolescent idiopathic scoliosis is demonstrated in a group of 51 patients. Cobb angles at the commencement of the study ranged from 10 degrees -55 degrees (mean 34.5 degrees), and the mean follow-up period was greater than 2 years. The surface shape method correctly identified curve evolution in 84% of the patient group. Of these, the eight patients who underwent spinal instrumentation were correctly predicted as candidates for surgery.     

Adult idiopathic scoliosis treated with Luque or Harrington rods and sublaminar wiring

We reviewed the results of spinal arthrodesis that was performed, with sublaminar wires that were attached to either double L-shaped Luque rods or to a Harrington rod, in forty-two adults who had idiopathic scoliosis. The minimum length of follow-up was two years; the maximum, five years; and the average, three years. The average scoliosis measured 67 degrees preoperatively, was corrected to 37 degrees at operation, and was 44 degrees at the time of follow-up. The final correction averaged 34 per cent. A single Harrington rod and multiple sublaminar wires were used in thirty-one patients. Eighteen of the thirty-one patients had a posterior arthrodesis only and thirteen, a preliminary anterior arthrodesis followed by a posterior arthrodesis. Eleven patients had instrumentation with double L-shaped Luque rods; six of them had posterior arthrodesis only and five, a preliminary anterior arthrodesis followed by a posterior arthrodesis. One patient had neurological deficit that was related to the operation. Three patients had a pseudarthrosis, which was in the lumbar area in all of them. All three patients had had only a posterior operation. No statistically significant difference in the amount of final correction was demonstrated between the subgroups.     

Spinal deformities in Noonan syndrome: a clinical review of sixty cases.

BACKGROUND: Skeletal abnormalities, including spinal deformities, in Noonan syndrome have been described, but no detailed and systematic study of such spinal deformities has been presented in the literature. METHODS: The cases of sixty patients with Noonan syndrome were reviewed retrospectively, and the general appearance, growth disturbance, and mental status of the patients were documented. Spinal deformities were evaluated radiographically, and the frequency, pattern, and severity of the curves were documented. RESULTS: Spinal deformity was present in eighteen (30%) of the sixty patients. Two patients had congenital spinal deformity. Of the remaining sixteen patients with scoliosis, nine had a single thoracic curve, four had a single thoracolumbar curve, and three had a double major curve. Thoracic lordosis was also present in three of these sixteen patients. No patient had only increased kyphosis or lordosis. The mean age when the spinal deformities were detected was nine years; seven deformities were detected before the age of seven years. Overall, surgery was recommended to eleven of the eighteen patients; it was recommended for the treatment of scoliosis (mean, 68.5 degrees; range, 45 degrees to 125 degrees ) in eight patients and for the treatment of an associated thoracic lordosis (8 degrees, 15 degrees, and 18 degrees ) in three. Seven of the eleven patients underwent spinal arthrodesis. The operation was deferred in one patient because malignant hyperthermia developed during the induction of anesthesia. CONCLUSIONS: Scoliosis with an associated thoracic lordosis occurs more frequently in Noonan syndrome than has been reported previously. Since the deformities tend to develop early and are relatively severe, a clinical and, if necessary, radiographic assessment of the spine with careful follow-up should be performed for early detection and treatment of spinal deformity. Although malignant hyperthermia is rare, all patients with Noonan syndrome should be considered to be at risk for the development of this complication before operative treatment.     

Transpedicular eggshell osteotomies for congenital scoliosis using frameless stereotactic guidance

STUDY DESIGN: Three cases of multiplanar congenital scoliosis corrected by a transpedicular eggshell osteotomy using frameless stereotactic guidance are reported. OBJECTIVE: To show an alternative surgical technique for correcting and fusing complex congenital spinal curves. SUMMARY OF BACKGROUND DATA: Children with congenital scoliosis can have progressive curves causing significant multiplanar deformities. In older children, traditional approaches to spinal corrective surgery including fusion in situ, convex growth arrest, and hemivertebra excision can achieve only limited corrections. METHODS: Three older patients who had congenital scoliosis with significant multiplanar curves underwent one-stage posterior transpedicular eggshell osteotomy. Three-dimensional reconstructive images and frameless stereotactic guidance were used for preoperative selection of osteotomy levels and accurate placement of pedicle screws. After completion of the osteotomy, closure was obtained with extension of the hips. RESULTS: The average coronal correction of the major curve was 28.7 degrees (range, 22-33 degrees ). The average correction of the plumb line or lateral displacement from the center of the trunk was 4.8 cm (range, 3-7.5 cm). A significant kyphotic deformity was corrected 38 degrees, and a pelvic tilt was reduced from 7 to 3 cm. CONCLUSIONS: One-stage posterior reduction eggshell osteotomy can be used to correct a sagittal and/or coronal congenital spinal curve imbalance. Frameless stereotactic guidance for solid pedicle screw fixation was essential to the achievement of rigid spinal stabilization before arthrodesis. Transpedicular eggshell osteotomy is a technique that should be considered for older patients who have congenital scoliosis with multiplanar spinal abnormalities.     

Adolescent idiopathic scoliosis treated by Harrington-rod distraction and fusion

One hundred and thirty-three patients who had adolescent idiopathic scoliosis were treated by insertion of a single Harrington distraction rod and spinal fusion. Postoperative immobilization consisted of six months in a below-the-shoulder cast. The mean preoperative curve was 50 degrees, with a range of 30 to 110 degrees. The mean final curve was 35 degrees, with a range of 19 to 63 degrees. There were no neurological injuries and no deep wound infections. Twelve patients required further surgery for complications, all of which were treated successfully. The duration of follow-up ranged from twenty-four to seventy-two months. At final follow-up, all patients had resumed their normal preoperative activities without limitations.     

Surgical treatment of scoliosis in Marfan syndrome: guidelines for a successful outcome

Twenty-three patients with Marfan syndrome who had a posterior spinal arthrodesis, with or without instrumentation, autogenous iliac crest bone graft, and postoperative immobilization were retrospectively reviewed to formulate guidelines for the treatment of scoliosis. Patients in group 1 (7 patients) had both the primary and secondary curves arthrodesed, whereas patients in group 2 (16 patients) underwent selective arthrodesis of the entire primary and a partial arthrodesis of the secondary curves. Thirteen patients had triple curves, nine had double curves, and one patient had a single curve. At the time of follow-up all patients in group 1 had minimal or no progression of their curves, whereas 5 patients in group 2 had minimal or no progression and 11 had marked progression of at least one curve. There were no pseudarthroses or hardware failures. The authors recommend arthrodesing both the primary and secondary curves in all patients with Marfan syndrome.     

Crankshaft effect after posterior spinal fusion and unit rod instrumentation in children with cerebral palsy

Radiographs and charts were reviewed for all children with cerebral palsy who underwent posterior-only spinal fusion with the unit rod for neuromuscular scoliosis by the senior author from 1989 through 1996. Fifty patients were found to have an open triradiate cartilage at the time of fusion. A single observer obtained measurements of the preoperative, postoperative, and most recent spine films using the standard Cobb angle. Amount of change was calculated over the respective periods. Forty-three patients had at least 2 years of clinical follow-up. Twenty-nine patients had more than 2 years of both radiographic (mean, 4.6 years) and clinical follow-up (mean, 4.8 years) with a closed triradiate cartilage on their most recent films. In this group, the mean absolute curve change over the length of radiographic follow-up was 0.6 degrees (range, -9-14). None of the 43 patients with at least 2 years of clinical follow-up (mean, 4.5 years) had any radiographic change that was clinically significant on chart review. Therefore posterior spinal fusion alone with unit rod instrumentation is adequate treatment to control crankshaft deformity in skeletally immature children with neuromuscular scoliosis due to cerebral palsy.     

腰椎椎管狭窄症的外科治疗

目的探讨腰椎椎管狭窄症外科治疗的临床疗效。方法回顾性研究手术治疗退变性腰椎椎管狭窄患者共172例,其中10例有脊柱侧凸改变,22例Ⅰ～Ⅱ度滑脱。单节段狭窄者90例,双节段狭窄者51例,3个节段狭窄者31例。采用单纯开窗减压者5例,后路融合固定者100例,后路椎间融合固定62例,Dynesys弹性内固定5例。临床疗效评定采用疼痛视觉模拟量表（visual analogue scale,VAS）评分,功能评分采用北美脊柱外科学会改良腰椎功能障碍指数（modified Oswestry low back pain disability questionaire index,MODI）,从腰腿痛、生活自理、行走和举重物等10个方面评估患者腰椎的ODI分数。结果所有病例随访6～72个月,平均50个月。术前腰腿痛平均评分为7.5分,术后3个月为3.2分,末次随访时为3.6分。术后3个月随访,MODI改善的优良率为94.3%;末次随访时优良率为87.6%。结论对退行性腰椎椎管狭窄根据术前临床及影像学检查,有针对性地进行椎管减压或联合椎弓根螺钉内固定治疗均可取得较好疗效。