Surgical management of spinal involvement in children and adolescents with Gaucher's disease

Gaucher's disease is an uncommon hereditary glycolipid storage disorder characterized by the accumulation of glucocerebroside in the lysosomes of macrophages of the reticuloendothelial system. Skeletal manifestations are variable in severity and typically involve the long bones. Vertebral involvement is less well characterized, particularly in children and adolescents. We report on the surgical management of spinal involvement in four children and adolescents with Gaucher's disease; two for kyphotic deformity and two for kyphotic deformity associated with neurologic compromise. We recommend anterior spinal release with fusion and posterior spinal fusion with segmental instrumentation in cases of kyphotic deformity. In cases of spinal cord compromise at the apex of the kyphotic deformity with retropulsion of involved bone, anterior decompression also should be performed. Routine surveillance for spinal deformity in patients with Gaucher's disease is necessary to allow early intervention before the development of severe deformity and neurologic compromise.     

Segmental wiring for spinal deformity. A morbidity report

Members of the British Scoliosis Society were circulated a questionnaire in order to establish the morbidity of surgery for spinal deformity in the years 1983 and 1984 in Great Britain. Surgeons were asked to report the complications relating to all types of surgery for spinal deformity in order to establish the relative morbidity of segmental spinal wiring. Of the 1,121 patients reported, 1.4% suffered neurologic complications. In straightforward surgery for adolescent idiopathic scoliosis, there were four serious neurologic complications: three with Harri-Luque and one with routine Harrington distraction instrumentation. A plea is made that morbidity studies of spinal deformity surgery should compare all types of instrumentation and should be sufficiently detailed to allow statistical comparison.     

Kyphosis – A risk factor for positioning brachial plexopathy during spinal surgeries

ObjectiveThe aim of this study was to evaluate the differences in transcranial electric motor-evoked potentials – TceMEP on upper limbs and the incidences of postoperative brachial plexopathy between patients with kyphotic and scoliotic trunk shapes.MethodsIn the period of January 2011–January 2017, 61 consecutive patients (mean age: 18.4 years ± 4.4 years (range: 10–32)) with pediatric spinal deformity underwent surgery in our Department. Eight of them had a kyphotic trunk deformity (Scheuermann kyphosis, neurofibromatosis, posterior thoracic hemivertebra), and the rest of the 53 patients had a scoliotic trunk deformity (mostly adolescent idiopathic scoliosis – AIS, lateral hemivertebra). The TceMEP recordings in all four limbs were analyzed every 30 min, or upon the surgeon's command. Upper limb TceMEP recordings were used as a control of systemic and anesthetic related changes, and as the indicator of positioning brachial plexopathy.ResultsFour out of 8 patients (50.0%) from the kyphotic group experienced noteworthy decreases in TceMEP amplitude (≥65%) in one or both arms, and only 2 out of 53 patients (3.8%) from the scoliotic group, confirming significant statistical difference (Chi-square 16.75, p < 0.05). Two out of 8 patients with decreases in TceMEP amplitude suffered from transitory postoperative brachial plexopathy, and both of them were from the kyphotic group.ConclusionIt seems that kyphotic trunks have a higher risk for positioning-related brachial plexopathy, probably due to distribution of trunk's weight onto only four points (two iliac bones and two shoulders), compared to the scoliotic trunks that have wider weight-bearing areas. We emphasize the importance of proper patient positioning and close intraoperative neuro-monitoring of all four limbs in more than one channel per limb.Level of EvidenceLevel IV Therapeutic Study.     

Late-onset spinal deformities in children treated by laminectomy and radiation therapy for malignant tumours

This is a retrospective study of 76 children who had had malignant tumours treated with laminectomy or laminoplasty and/or radiation therapy affecting the spine. Spinal tumours in children are extremely rare. However, their treatment can result in progressive spinal deformity. Radiation therapy affecting the growing spine can lead to asymmetric vertebral growth, causing kyphosis and/or scoliosis. These spinal deformities pose one of the most challenging problems for the spine surgeon. The aim of this article is to describe late-onset post-laminectomy/post-radiation spinal deformities and to evaluate the results of their treatment. Seventy-six children, with a mean age of 4 years and 7 months (range, 2 months to 16 years), underwent surgical removal of malignant tumours, between 1961 and 1995. Sixty-seven of them developed post-laminectomy/post-radiation spinal deformity. Conservative treatment consisted of bracing and corrective plaster casts. In 46 cases the deformity was treated surgically. A distraction plaster cast was used as preoperative preparation in the more severe and rigid curves, with or without neurological impairment. Surgery consisted of combined anterior and posterior fusion in 39 cases and posterior fusion in seven cases. Posterior instrumentation was used in 38 cases. The mean follow-up period was 6 years and 7 months (range, 9 months to 20 years and 2 months). Nine children did not develop deformity following the primary tumour treatment. One of them underwent laminectomy with posterolateral fusion and eight had laminoplasty combined with external immobilisation. Forty-six children developed iatrogenic kyphosis and underwent surgical correction from a mean of 75° pre-correction to a mean of 32°. The mean scoliotic angle correction was 66° preoperatively to 34° postoperatively. At follow-up, the mean correction loss was 7° in the sagittal plane and 5° in the coronal plane. Preoperative distraction plaster cast treatment resulted in a correction of 39% in kyphosis and of 58% in scoliosis, and in a partial or complete recovery of neurological deficits in all but one patient. In severe and rigid curves that develop following treatment of paediatric spinal tumours, preoperative application of a distraction plaster cast can reduce deformity and facilitate surgical correction. Furthermore, in the case of pure bony compression of the spinal cord due to the apical vertebra of the deformity, treatment with the distraction plaster can result in recovery from the neurological impairment. The prevention of post-laminectomy/post-radiation spine deformities is emphasised. Rigid external immobilisation for a period of 4 months in the cervical spine and of 6 months in the thoracic spine is recommended after both laminoplasty and laminectomy with posterolateral fusion.The research was carried out at Saint Vincent de Paul Hospital, Department of Pediatric Orthopaedic Surgery, Paris     

Bilateral transpedicular decompression and Harrington rod stabilization in the management of severe thoracolumbar burst fractures.

Fifty-eight patients with severe thoracolumbar burst fractures were treated with bilateral transpedicular decompression, Harrington rod instrumentation, and spine fusion. Spinal realignment and stabilization was achieved by contoured dual Harrington distraction rods supplemented by segmental sublaminal wiring. Posterior element fractures were noted in 25 patients, 9 of whom had associated dural tears. Computed tomography was performed to assess the cross-sectional area of the spinal canal before surgery and after decompression. Patients at initial evaluation averaged greater than 67% spinal canal compromise. After surgery, successful decompression was accomplished in 57 patients. One patient required staged, anterior thoracoabdominal decompression and fibula strut grafting. At follow-up (average, 43 months; range, 25-70 months), neurologic improvement was found in 77% of the patients who initially presented with neurologic deficits. Thirty-four of 40 patients with incomplete paraplegia improved one or more subgroups on the Frankel scale. A solid fusion was attained in all 58 patients. No patient had a significant residual kyphotic deformity. Single-stage bilateral transpedicular decompression and dual Harrington rod instrumentation reliably provides decompression of the spinal canal and restores spinal alignment. The procedure allows early mobilization and provides an environment for solid fusion and maximum neurologic return.     

先天性脊柱侧后凸畸形的手术治疗

目的 探讨半椎体切除后采用平移技术及悬梁臂技术治疗先天性脊柱侧后凸的有效性.方法 回顾性分析2005年1月~2010年1月于本院行手术治疗的先天性脊柱侧后凸患儿24例,男11例,女13例;年龄为6~14岁,平均10.3岁.均于全麻下接受半椎体切除联合内固定矫正脊柱侧后凸畸形,记录患者的手术时间、出血量,术后侧凸角和后凸角的矫正率及末次随访时丢失率.结果 所有患者均获随访,平均随访14个月,手术时间 （148±25） min,手术出血量 （760±85） mL.11例患者置入4对椎弓根螺钉,8例患者置入5对椎弓根螺钉,5例患者置入6对椎弓根螺钉,平均置入4.75对.侧凸Cobb角术前为52.7°±6.2°,术后为9.4°±2.3°,矫正率为82.2%,末次随访时为10.2°±1.2°,矫正丢失率为8.5%.后凸Cobb角术前为30.2°±5.3°,术后为7.2°±1.4°,矫正率为76.2%,末次随访时为7.7°±1.0°,矫正丢失率为6.9%.随访期间未发生内固定物相关并发症,无曲轴现象出现.结论 半椎体切除联合内固定矫形是治疗先天性脊柱侧凸的理想方法,应用平移及悬梁臂技术可显著矫正脊柱侧后凸畸形.     

Simultaneous anterior-posterior approach through a costotransversectomy for the treatment of congenital kyphosis and acquired kyphoscoliotic deformities

BACKGROUND: Congenital kyphosis and acquired kyphoscoliotic deformities are uncommon but are potentially serious because of the risk of progressive deformity and possible paraplegia with growth. Our current approach for the treatment of these deformities is to use a single posterior incision and costotransversectomy to provide access for simultaneous anterior and posterior resection of a hemivertebra or spinal osteotomy, followed by anterior and/or posterior instrumentation and arthrodesis. To our knowledge, this approach has not been reported previously. METHODS: The medical records and radiographs for sixteen patients who had been managed at our institution for the treatment of congenital kyphosis and acquired kyphoscoliosis between 1988 and 2002 were analyzed. The mean age at the time of surgery was twelve years. The diagnosis was congenital kyphosis for fourteen patients and acquired kyphoscoliotic deformities following failed previous surgery for two. The mean preoperative kyphotic deformity was 65 degrees (range, 25 degrees to 160 degrees ), and the mean scoliotic deformity was 47 degrees (range, 7 degrees to 160 degrees ). Fifteen patients were managed with vertebral resection or osteotomy through a single posterior approach and costotransversectomy, anterior and posterior arthrodesis, and posterior segmental spinal instrumentation. The other patient was too small for spinal instrumentation at the time of vertebral resection. A simplified outcome score was created to evaluate the results. RESULTS: The mean duration of follow-up was 60.1 months. The mean correction of the major kyphotic deformity was 31 degrees (range, 0 degrees to 82 degrees ), and the mean correction of the major scoliotic deformity was 25 degrees (range, 0 degrees to 68 degrees ). Complications occurred in four patients; the complications included failure of posterior fixation requiring revision (one patient), lower extremity dysesthesias (one patient), and late progressive pelvic obliquity caudad to the fusion (two patients). The outcome, which was determined with use of a simplified outcomes score on the basis of patient satisfaction, was rated as satisfactory for thirteen patients, fair for two patients, and poor for one patient. CONCLUSIONS: A simultaneous anterior and posterior approach through a costotransversectomy is a challenging but safe, versatile, and effective approach for the treatment of complex kyphotic deformities of the thoracic spine, and it minimizes the risk of neurologic injury. LEVEL OF EVIDENCE: Therapeutic Level IV.     

脊柱畸形手术失败原因与对策

目的探讨脊柱矫形手术失败原因、预防措施、处理方法及翻修手术适应证。方法31例患者,男18例,女13例;翻修手术时年龄4～35岁,平均14.7岁;既往平均手术史1.5次。初次手术距翻修手术时间平均47.9个月(13～114个月)。术前出现腰背部酸困疼痛、活动后加重16例,内植物并发症(断裂、松脱或外露等)5例,畸形进行性加重21例,下肢部分肌力和感觉障碍5例。翻修术前平均侧凸角75.3°,脊柱柔韧性9.8%;后凸角76°,柔韧性25.2%。分别采用脊椎截骨、椎弓根螺钉固定,原位固定和分期手术治疗。结果20例患者平均随访31.8个月,侧后凸平均矫正率分别为55.2%和67.5%。手术并发症:出现暂时性神经功能障碍4例(12.9%),经脱水、激素和电脉冲刺激等治疗,均在术后1～3周内得到完全恢复;内植物断裂2例,无其他严重并发症。结论正确掌握脊柱矫形手术治疗原则、良好的植骨融合、对先天性侧凸进行必要的内固定以及避免过早拆除内固定等,是防止矫形手术失败的有效手段。对有顽固性腰背痛、脊柱假关节和术后畸形进行性加重者,应根据患者年龄、畸形程度和脊柱柔韧性,采用不同的治疗方法。     

Kyphectomy for myelodysplasia

A progressive kyphotic deformity occurs in 15% of children with myelomingocele. The more common problems caused by the gibbus are recurrent or recalcitrant skin ulceration and seating difficulties. The only effective treatment is surgery. Excellent correction is possible by vertebrectomy of a portion of the cephalad limb of the deformity. Superior maintenance of the correction is accomplished by segmental spinal instrumentation. Alternative techniques that spare growth are currently being investigated. Vertebral body decancellation is one such method that is less extensive than vertebrectomy and, theoreticallty, allows continued spinal growth. It seems most appropriate for the younger patient with a less rigid and dramatic deformity.     

Idiopathic scoliosis: foundation for physiological treatment

The three-dimensional nature of the idiopathic spinal deformity has been investigated in cadaveric specimens and patients with both idiopathic scoliosis and idiopathic kyphosis (Scheuermann's disease). In both scoliotic and kyphotic deformities the essential lesion lies in the sagittal plane with apical vertebral wedging. In idiopathic scoliosis there is an apical lordosis which being biomechanically unstable rotates to the side to produce a scoliotic deformity as a secondary component. In contradistinction the kyphotic wedging process of Scheuermann's disease is mechanically stable and any associated idiopathic type scoliosis occurs above and below the region of kyphosis. When an asymmetric lordosis is created in the growing New Zealand white rabbit, a progressive lordoscoliosis is readily produced and when the thoracic kyphosis is restored the scoliotic deformity shows evidence of regression and this forms the basis of physiological treatment. In 25 patients with idiopathic thoracic scoliosis the thoracic kyphosis has been restored and this leads to enhanced correction of the deformity in all three planes.     

Atypical caudal regression syndrome with agenesis of lumbar spine and presence of sacrum – case report and literature review

Context: Caudal regression syndrome is a rare disorder, not well described in the literature.

Findings: Authors treated two patients with congenital absence of thoracolumbar vertebrae and lower limbs paraplegia. Patients had hypoplasia of the lower trunk and extremities with motion between upper and lower torso. Imaging showed caudal spine agenesis, but cleft sacrum was present. Due to severe kyphotic deformity and spinal instability, deformity correction and posterior fusion was performed at the age 6 and 8. Finally, fusion was achieved in one case and stable but non-fusion kyphotic posture was observed in second.

Conclusion: surgery in caudal regression syndrome is challenging and bears high risk of complications.     

Treatment of severe thoracolumbar kyphoscoliosis on the ground of ganglioneuroblastoma of the diaphragm: a case report

The treatment of an unusual case with severe kyphoscoliotic deformity of the thoracolumbar spine, in association with a ganglioneuroblastoma invading the adjacent diaphragm, is presented in this report. Severe scoliotic deformity of the spine, associated with ganglioneuroblastoma of the diaphragm is a rare combination and treatment can be very challenging, as both entities require extensive surgery. To the best of our knowledge, only two reports related to a similar condition have been published in the literature so far. A 9-year-old girl had been treated in another institution since the age of 5 years for thoracolumbar kyphoscoliosis with bracing. She was referred to our department with a stiff right kyphoscoliotic curve at the thoracolumbar region (T8-L2), with measurements of 105 and 90° for the scoliotic and kyphotic elements, respectively. There were no neurological signs detected at the preoperative work-up, which included clinical and radiological evaluation (plain X-rays and computed tomographic scan). The patient was treated with a two-stage spinal fusion. During the anterior approach through a right thoracotomy, a tumor was discovered infiltrating half of the diaphragm and extended to the frontal surface of the lower thoracic vertebral bodies. The vertebrae were cleared of the tumorous masses and anterior discectomies (T8-L2) were successfully carried out according to the preoperative plan. The histopathology report indicated ganglioneuroblastoma in both the prevertebral material and the speciments excised from the diaphragm. A week later a T3-L4 posterior fusion was carried out and the deformity was corrected and fixed with an SFS instrumentation system. Both the scoliotic and the kyphotic curves were reduced to 46°. Postoperatively the patient was referred to the oncology department of another hospital for the treatment of the diaphragm tumor. During the last follow-up (4 years), the scoliotic curve remains steady and the tumor shows no recurrence. Although there are cases of ganglioneuroblastoma associated with scoliotic deformity, the combination of this tumor infiltrating the diaphragm and a very severe deformity of the spine is extremely rare. Nevertheless, the treatment undertaken has proven to be successful so far.     

颈椎后路椎管扩大成形术后并发症的研究进展

目的总结颈椎后路椎管扩大成形术后并发症中发病机制尚未完全了解的轴性症状、后凸畸形、节段性运动麻痹的研究进展。方法查阅有关颈椎后路椎管扩大成形术后并发症文献,并综合分析。结果轴性症状、后凸畸形、节段性运动麻痹并发症的发病机制尚未完全了解,但已有了更多新的发现,如椎体后方肌肉韧带复合体的重要性和引起节段性运动麻痹的神经根麻痹、脊髓损伤两种学说。结论应对轴性症状、后凸畸形、节段性运动麻痹并发症的发病机制作进一步研究,以期能有效防治其发生。     

Transpedicular wedge resection osteotomy for the treatment of a kyphotic Andersson lesion-complicating ankylosing spondylitis

Two cases with a long-standing thoracolumbar kyphosis due to ankylosing spondylitis are presented with a symptomatic localized destructive kyphotic lesion of the spine. Clinical and radiographic findings demonstrated a progressive vertebral and discovertebral kyphotic pseudarthrosis, known as an Andersson lesion, at the L1 and L1-2 level, respectively. Surgical correction and stabilization was performed by an extending transpedicular wedge resection osteotomy to restore spinal stability, to facilitate fracture healing as well as to restore the sagittal balance of the ankylosed spine. To predict the effect of a surgical correction of the Andersson lesion on the sagittal balance, deformity planning was performed preoperatively. The indication for surgery, the surgical technique and the 2 years clinical results are described. In addition, the difficulties experienced with preoperative deformity planning are evaluated.     

Combination of luque instrumentation with polyaxial screws in the treatment of myelomeningocele kyphosis

Rigid congenital kyphosis in myelomeningocele is associated with an important morbidity with skin breakdown, recurrent infection, and decreased function. Kyphectomy is the classic treatment to restore spinal alignment; however, surgery is associated with morbidity and long-term complications. The purpose of this retrospective study was to examine the authors' experience using combination of Luque instrumentation with posterolaterally placed polyaxial screws in the treatment of myelomeningocele kyphosis. From June 1999 to June 2003, 7 patients were treated and followed up for an average of 68.6 months. The average age at the time of the operation was 7 year and 1 month. All patients underwent vertebral excision from just above the apex of vertebral deformity to realign the sagittal deformity. Posterolaterally placed polyaxial screws were used in combination with segmental Luque instrumentation. Kyphotic deformity averaged 104 degrees before surgery, 15.2 degrees after surgery, and 18.5 degrees at the latest follow-up. The average loss of correction was 3.3 degrees. The average blood loss was 611 mL. Complications occurred in 2 of 7 patients were superficial wound breakdown and deep wound infection that required rotational flap closure. Kyphectomy with posterior instrumentation by using Luque technique in the combination with polyaxial screws is reliable method for correcting rigid kyphotic deformity in patients with myelomeningocele. Rigidity of the construct, greater correction capacity, and low profile instrumentation by the help of posterolateral insertion of the polyaxial screws and wiring were the distinct advantages of this technique.     

Predictors of kyphotic deformity in osteoporotic vertebral compression fractures: a radiological study

Purpose

To report the radiological predictors of kyphotic deformity in osteoporotic vertebral compression fractures (OVCF).

Methods

This is a retrospective study of 64 consecutive patients with OVCF. We studied the radiographic features in the immediate post-injury image of patients, who developed significant (more than 30°) segmental kyphotic deformity at final follow-up and compared them with those patients who did not.

Results

Thirty-three (82.5 %) out of 40 patients with fracture at thoracolumbar (TL) junction, 5 (33.3 %) patients out of 15 with fracture at lumbar (L) spine and 7 (77.7 %) patients out of 9 with fracture at thoracic (T) spine developed significant segmental kyphotic deformity. Forty-one (75.9 %) [TL-33 (80.5 %), L-4 (33.33 %) and T-4 (80 %)] out of 54 [TL-37 (68.51 %), L-12 (22.23 %) and T-5 (9.26 %)] patients with superior endplate fracture developed significant segmental kyphotic deformity. Forty patients (86.9 %) [TL-28 (70 %), L-6 (15 %) and T-6 (15 %)] out of 46 [TL-32 (69.56 %), L-8 (17.4 %) and T-6 (13.04 %)] with anterior cortical wall fracture developed significant segmental kyphotic deformity. Five patients (71.42 %) [TL-2 (40 %) and T-3 (60 %)] out of 7 [TL-02 (28.58 %), L-01 (14.28 %), T-04 (57.14 %)] with adjacent level fracture developed significant segmental kyphotic deformity. The average immediate post-injury kyphosis of 11° (5°–25°) increased to 29° (15°–50°) at final follow-up.

Conclusion

Progressive segmental kyphotic collapse following an OVCF seems unavoidable. Patients with TL junction and superior endplate fracture are probably at the highest risk for significant segmental kyphotic deformity.     

Spinal deformity changes in children with long-term vertical expandable prosthetic titanium rib treatment

Background Context

In several studies, vertical expandable prosthetic titanium rib (VEPTR) implants have shown good scoliosis control in children with the longest reported follow-up of 3.6 years. For growing rods, recent studies suggest a decreased efficiency of correction starting just after that time. To our knowledge, no long-term results of children with VEPTR treatment are available.

Spinal loop rectangle and sub laminar wiring as a technique for scoliosis correction

Background:

Most literature popularizes the efficacy of third generation instrumentation in the surgical correction of spinal deformities. A cheap and effective scoliotic deformity correction method is reviewed in this article. The aim of this study is to evaluate the efficacy of spinal loop rectangle and sub laminar wires as a modality for spinal deformity correction and its co-relation with patients'' satisfaction and clinical outcome.

Material and Methods:

Thirty six patients of scoliotic spinal deformities with various etiologies (congenital-4, idiopathic- 25, neurofibromatosis-3, neuromuscular-2 and ‘syndromic’-3) with ages ranging from 8 to 23 years underwent corrective posterior spinal arthrodesis with stainless steel Hartshill loop rectangle and sublaminar wires. Clinicoradiological evaluation was done at an average follow-up of 6 ½ years (min-2 ½, years). Along with clinicoradiological outcome, patient satisfaction (as per the SRS 24), was accounted.

Results:

Average preoperative Cobb''s angle were 73.25° in the entire group and 66.48° in the idiopathic group. Average percentage correction was 64.34% in the entire group and the (average degree of correction was 47.13). In the idiopathic group, the respective values were 69.19% and 46°. Loss of correction in the whole group was 2.2° at two year follow up. Sagittal profiles, truncal balance were well corrected too; minimal complications were seen. Patient satisfaction results were encouraging in 36 patients as per – SRS24). About 80.2% patients were ready to undergo the same surgery if required. (SRS24).

Conclusion:

Segmental spinal fixation with locally made spinal loop rectangle and sublaminar wires is comparable as a modality to correct scoliotic spinal deformities.     

Fracture dislocations of the cervical spine: a review of 106 conservatively and operatively treated patients

We compared clinical outcomes following conservative treatment of subaxial fracture dislocations of the cervical spine and posterior fusion using bone grafts and interspinous Rogers wiring (Bohlman modification). We reviewed 106 patients: 51 were treated primarily surgically, and 55 treated conservatively served as historical controls. Those patients who neurologically recovered at least one Frankel grade had on average less displacement on discharge (1.3 mm vs 3.1 mm, p=0.04). Although anatomical outcomes were better in the operatively treated group (1.6 mm vs 2.9 mm displacement at end of follow-up, p=0.001), there was no difference in neurological recovery. Late neck pain correlated with residual displacement (p=0.04) and was more common in the conservatively treated patients (p=0.01). Time in hospital was shorter in the group with posterior fusions, and complication rates were similar to those found after conservative treatment. A significant number of the conservatively treated patients developed kyphotic deformity, and 29% needed later surgery because of chronic instability or unacceptable anatomical results.     

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.