Freeman Miller

To determine the degree to which a high-frequency, low-magnitude vibration (HLV) signal emitted by a floor-based platform transmits to the distal tibia and distal femur of children with spastic cerebral palsy (CP) during standing. Cross-sectional study SETTING: University research laboratory PARTICIPANTS: 4 to 12 year-old children with spastic CP who could stand independently (n=18) and typically developing children (n=10) participated in the study. Not applicable MAIN OUTCOME MEASURES: The vibration signal at the HLV platform (∼33 Hz and 0.3 g), distal tibia and distal femur was measured using accelerometers. Degree of plantar flexor spasticity was assessed using the Modified Ashworth Scale. The HLV signal was greater (p<0.001) at the distal tibia than at the platform in children with CP (0.36±0.06 vs. 0.29±0.05 g) and controls (0.40 ± 0.09 vs. 0.24 ± 0.07 g). Although the HLV signal was also higher at the distal femur (0.35±0.09 g, p<0.001) than at the platform in controls, ...

... In a variation on the turns counting method, Jeleń & Sławińska (1996) compared the activation of two muscles using a spike counting method. ... The method begins with a full wave rectification of surface EMGs recorded as gait data. ...

Using high resolution magnetic resonance imaging, we detected severely underdeveloped trabecular bone microarchitecture in the distal femur of children with cerebral palsy who can not ambulate independently vs. typically developing controls. Furthermore, very good short-term reliability of trabecular bone microarchitecture measurements was observed in both groups of children. Severe forms of cerebral palsy (CP) are associated with very low areal bone mineral density and a very high incidence of fracture in the distal femur; however, the state of trabecular bone microarchitecture has not been evaluated. Furthermore, the short-term reliability of trabecular bone microarchitecture assessment in children using high-resolution magnetic resonance imaging (MRI) has not been determined. Apparent bone volume to total volume (appBV/TV), trabecular number, (appTb.N), trabecular thickness (appTb.Th) and trabecular separation (appTb.Sp) were determined in the distal femur of non-ambulatory child...

Cerebral palsy is a term applied to a variety of clinical syndromes that have as a common feature the abnormal control of motor function by the brain, resulting in disorders of movement and posturing. Sensory function is often affected as well. Cerebral palsy afflicts the immature brain (under 2 years of age) and usually produces neuropathologic changes that do not progress with increasing age. Because myelination of the brain is a progressive process that begins late in the third trimester and continues into adolescence in a well defined pattern, only after the neuronal pathways from brain lesions have become myelinated and the pattern of abnormality determined, can the brain lesions forming the basis of cerebral palsy be detected.

Hip disorders are common in patients with cerebral palsy and cover a wide clinical spectrum, from the hip at risk to subluxation, dislocation, and dislocation with degeneration and pain. Although the hip is normal at birth, a combination of muscle imbalance and bony deformity leads to progressive dysplasia. The spasticity or contracture usually involves the adductor and iliopsoas muscles; thus, the majority of hips subluxate in a posterosuperior direction. Many patients with untreated dislocations develop pain by early adulthood. Because physical examination alone is unreliable, an anteroposterior radiograph of the pelvis is required for diagnosis. Soft-tissue lengthening is recommended for children as soon as discernable hip subluxation (hip abduction <30 degrees, migration index >25%) is recognized. One-stage comprehensive hip reconstruction is effective treatment for children 4 years of age or older who have a migration index >60% but who have not yet developed advanced ...

Anatomical study of 72 pelves revealed that the most substantial area of bone that could best support a rod for surgical fixation is the body of the ilium, with the best part of the bone being the area above the sciatic notch. Placement of the rods into the body of the ilium provides good fixation but requires complex bending of the rods and a good three-dimensional perception of the osseous anatomy of the pelvis. This study was undertaken to define the intraosseous anatomy and the range of anatomical variation of the ilium. The results of this study are useful in developing a prebent spinal fixation rod, providing a better understanding of this anatomical area from a three-dimensional perspective and providing better lumbosacral spinal instrumentation.

A retrospective matched cohort study with control group. To compare the infection rate after posterior spinal fusion with unit rod instrumentation with or without gentamicin-impregnated allograft bone in children with cerebral palsy (CP). Previous studies evaluating wound infection rates after spinal fusion surgery in children with CP report an 8.7% to 10% wound infection rate. The concept of using antibiotic-loaded bone graft (AbBGF) to provide local antibiotics has been explored in high risk patients, such as those with osteomyelitis or infected joint arthroplasty. There have been no reports of using AbBGF prophylactically in spine surgery. After IRB approval, the medical records of 220 children with CP who underwent spinal fusion with unit rod instrumentation for a primary spinal deformity between January 2000 through December 2006 at a single institution were retrospectively reviewed. We evaluated the incidence of postoperative wound infection in patients with AbBGF and those without bone graft (BGF). One hundred fifty-four patients received AbBGF during spinal fusion surgery and 6 patients (3.9%) developed a deep wound infection. Ten (15.2%) of the 66 patients without AbBGF developed a deep wound infection. The difference between groups was statistically different (P = 0.003). The mean age at surgery, preoperative Cobb angle, correction rate, operative time, and estimated blood loss were not statistically different between the 2 groups (P > 0.05). The length of hospital stay was decreased in the AbBGF group (P < 0.05). The incidence of deep wound infection after spinal fusion in 220 children with CP scoliosis decreased from 15% to 4% with the use of prophylactic antibiotics in the corticocancellous allograft bone.

Retrospective clinical and radiographic review of complications related to intrathecal baclofen therapy (ITB) and posterior spine fusion (PSF) in patients with cerebral palsy. To report the technical considerations and complications associated with ITB in patients undergoing PSF. A common treatment for spasticity in children with cerebral palsy is ITB. This population also has a high incidence of severe spinal deformities requiring PSF. There were 4 groups: A, 26 patients with PSF before ITB; B, 11 patients who underwent PSF and ITB concurrently; C, 25 patients with PSF after ITB; and D, the control group: 103 patients with ITB only. Complications and infections were tabulated from a retrospective chart review and ongoing surveillance data. Multiple chi analyses were used to compare the number of patients who experienced complications and infections among the groups. The operative sequence and catheter management techniques for the various scenarios are described in detail in the text. The outcome by group was as follows: group A had 5 catheter malfunctions and 2 infections at the pump site, group B had 2 catheter malfunctions, 1 hypermobile pump and 1 infection at the spinal site, group C had 3 catheter malfunctions, 1 infection at the pump site and 1 infection at the spinal site. The control group had 23 catheter malfunctions, 5 pump failures, 8 infections at the pump site, and 1 infection at the spinal site. Multiple chi analyses showed no difference in the number of infection or device/catheter complications among any of the groups. The rate of ITB therapy complications is not increased despite PSF in any order of the procedures. There are technical details in each situation that require attention. With understanding of the appropriate techniques of catheter management, ITB pumps can be implanted and managed without an increased complication rate before, during or after spinal fusion surgery.

Retrospective radiographic and medical chart review with matched control group. To identify the effect of intrathecal baclofen on the incidence of scoliosis, rate of curve progression, and pelvic obliquity compared with a matched cohort. Although intrathecal baclofen therapy (ITB) has been shown to be effective in decreasing spasticity, case reports have described some children receiving ITB in whom progressive scoliosis was noted; other authors have described no effect on the spinal column. A controlled study has not been performed. All patients with spastic CP treated with ITB between 1997 and 2003 at a single institution were reviewed. A total of 107 patients undergoing ITB for a minimum of 2 years were identified, of which 26 patients subsequently developed or had progression of scoliosis. Twenty-five age, gender, and gross motor function classification system (GMFCS) score-matched quadriplegic CP patients with scoliosis who did not receive ITB constituted the control group used to compare the rate of curve progression and pelvic obliquity. The average curve progression for the baclofen group after pump implantation was 16.3 degrees per year; and for the control group was 16.1 degrees per year. Both groups' curves progressed over time during growth (P = 0.001), but baclofen did not have an independent effect on curve progression (P = 0.181). Average pelvic obliquity for the 2 groups increased over time (P = 0.001), but there was no difference between the groups (P = 0.536). Twelve of 57 patients (21%) developed scoliosis after pump implantation during a mean of 3.6 years of follow-up. Thirty of 92 matched control patients (32%) not treated with ITB within the same time interval had scoliosis by maturity. This study demonstrates that ITB has no significant effect on curve progression, pelvic obliquity, or the incidence of scoliosis when compared with an age, gender, and GMFCS score-matched control group of patients with spastic CP without ITB.

Objective documentation of dynamic varus and valgus deformities of the hindfoot is still a clinical dilemma. In a review of spastic foot deformities, clinical, radiographic, and foot pressure data were collected in 108 children with cerebral palsy. According to the clinical assessment, five categories of foot deformities were defined: severe varus, varus, neutral, valgus, and severe valgus. A coronal index of the pedobarograph was determined by comparing the pressure/time integral under the medial column to that under the lateral column of the foot. Coronal index is highly correlated with clinical assessment and offers better information than radiographic measurements in differentiating the clinical categories. The authors recommend the pedobarograph as the primary evaluation tool to measure the severity of deformity for patients with varus and valgus foot dysfunction. The severity of the deformity can be monitored with a single measurement, which has the best correlation with the clinical assessment. The clinical assessment is still the primary tool to determine general patterns, but it is difficult to apply an objective measurement. Radiographic study is most useful for the preoperative assessment when surgery is indicated.

The aim of our study was to evaluate the surgical results after pectoralis major muscle release in children with cerebral palsy and to assess a caregiver's satisfaction in patient's care after this procedure.Twenty-one shoulders in 13 patients were analyzed at least 12 months after surgery. All but 1 patient involved in our study had quadriplegic type of cerebral palsy involvement. In 17 limbs, only pectoralis release was performed; in 4 limbs, latissimus dorsi release was added. After clinical examination, a questionnaire was used to determine the caregiver's assessment. The passive range of abduction and flexion was maintained at an angle greater than 90 degrees in 10 patients. External rotation was restricted in 6 patients. All caregivers would recommend this surgery to another patient. All but one reported improvement in care of the patients after surgery. The indication for this surgical procedure is limited only to the selected cerebral palsy cases in which custodial care is impaired by shoulder adduction contracture. The 2 main indications for the procedure are the limitations in the ability to bathe the axilla and the difficulty in dressing.

Twenty percutaneous rotational osteotomies, stabilized with interlock nails, were performed in the lower limbs of 15 skeletally mature adolescents with cerebral palsy to correct rotational deformities. The medical records and radiographs of those patients were retrospectively reviewed. Nineteen osteotomies (95%) in 15 patients healed without major complications. One patient had one tibia (5%) pseudarthrosis, which was successfully treated with additional fibular osteotomy and exchanging the nail. Excluding this case, the average healing time for the femoral and tibial osteotomies was 8 weeks, ranging from 7 to 9 and from 6 to 10 weeks, respectively. Casting was not required to add stability. Percutaneous rotational osteotomy with intramedullary nail fixation is a reliable and effective treatment option to correct rotational malalignment of the lower limb in skeletally mature patients with cerebral palsy.

We used the dynamic pedobarographs to study pressure distribution patterns in the foot after surgical correction of cavovarus feet. We also assessed the influence of ankle power generation on pressure distribution in these feet. Nine children (14 feet) diagnosed with Charcot-Marie-Tooth disease who had undergone operative treatment with a combination of osteotomies and muscle transfers were the subjects of this study. Preoperative and postoperative pedobarographic measurements recorded included pressure over the medial forefoot, lateral forefoot, medial midfoot (MMF), lateral midfoot (LMF), and heel segments. In 6 patients (9 feet) who had a complete gait analysis, the power generation of the ankle was also obtained both preoperatively and postoperatively. Lateral radiographic measurements included the (1) talus-first metatarsal angle, (2) calcaneus-first metatarsal angle, and (3) calcaneal pitch. The radiographs showed significant improvements in all 3 angles. Increased LMF and decreased forefoot pressures were seen on preoperative pedobarographic measures. Postoperatively, improvement in pressure at the LMF was seen. When postoperative measurements were compared with the normal values, only the LMF was similar; the other 4 segments showed decreased forefoot and MMF pressures and increased heel pressures (P = 0.000 for the lateral forefoot and MMF; 0.040 for the heel and medial forefoot). The heel pressures displayed an inverse relationship to ankle power generation. The amount of correction achieved radiographically did not correlate with pedobarographic measurements. The increased heel pressure that was noted was not addressed by treatment. Normalization of pressure patterns should be the goal in treating children with symptomatic cavovarus feet. Although the foot deformity is corrected completely in neuromuscular disorders, pressure distribution was not normalized, and therefore, symptoms might persist. Both patients and parents should be informed about this possible problem before surgical intervention.

Pelvic radiographs of 25 children aged 6 months to 2 years had the acetabular index measured 3 times by each of five pediatric orthopaedists. Interobserver measurements were found to vary +/-3.0 degrees, whereas the intraobserver variation was +/-3.6 degrees. This error reflects only measurement error and does not consider error introduced with different positioning of the pelvis.

Sixty-eight patients from a clinical population of 183 patients with Duchenne muscular dystrophy underwent posterior spinal fusion with instrumentation for progressive spinal deformity. Pulmonary complications were the most common postoperative problem, occurring in 17 per cent of the patients. A 35 per cent normal forced vital capacity was a reliable indicator of pulmonary complication risk. The patients with surgically stabilized spines were more comfortable in the later years of life and easier to care for, but deteriorating pulmonary function was not affected by the spinal fusion. The average age at death for the 29 boys who underwent spinal fusion was 18.3 years, which was similar to that of the 58 boys with scoliosis. Factors that improved the patients' quality of life included segmental instrumentation, fusion from T2 to the pelvis, correcting or balancing scoliosis, creating normal sagittal plane alignment and correcting pelvic obliquity.

One female and two male patients with multiple lateral meningoceles are presented. They do not have neurofibromatosis or Marfan syndrome and share findings with the two previously described patients with multiple lateral meningoceles. The original report by Lehman et al. [1977: J Pediatr 90:49-54] was titled "familial osteosclerosis," because osteosclerosis was present in the proposita and her mother; the patient described by Philip et al. [1995: Clin Dysmorphol 4:347-351] also had increased bone density of the skull base and the sutures. Thickened calvaria were present in one of our patients; two had a prominent metopic suture. Other shared findings include multiple lateral meningoceles, Wormian bones, malar hypoplasia, downslanted palpebral fissures, a high narrow palate, and cryptorchidism in males. In addition, our patients showed ligamentous laxity, keloid formation, hypotonia, and developmental delay. A short umbilical cord was noted in two patients. One had a hypoplastic posterior arch of the atlas and an enlarged sella, as reported by Lehman et al. [1977]. Our patients appear to have the same syndrome as previously reported. We suggest it be called "lateral meningocele syndrome," because of this unique finding.

To describe the computed tomography (CT) findings encountered when catheter patency is questionable. The role of CT in directing treatment is evaluated. Records of children with intrathecal baclofen pump management were reviewed. Only patients with CT evaluation who had revision pump/catheter surgery were included. From 295 patients, 27 had CT contrast study; in three of them, baclofen could not be aspirated and the procedure was stopped, eight had normal scan and did not need surgery and 16 patients were reported. Four patients had normal CT (free contrast formed a perfect crescent shape), and had surgery because the pump battery was close to expiration. Five patients had inadequate fluid pooling (fluid was seen without a crescent shape). Five patients had fluid leak (fluid was seen around the pump or in the lumbar canal below catheter entrance level or outside the canal in the lumbar region). Two patients had catheter occlusion (fluid loculation around the catheter tip with no fre...

Muscle is strongly related to cortical bone architecture in children; however, the relationship between muscle volume and trabecular bone architecture is poorly studied. The aim of this study was to determine if muscle volume is related to trabecular bone architecture in children and if the relationship is different than the relationship between muscle volume and cortical bone architecture. Forty typically developing children (20 boys and 20 girls; 6 to 12y) were included in the study. Measures of trabecular bone architecture [apparent trabecular bone volume to total volume (appBV/TV), trabecular number (appTb.N), trabecular thickness (appTb.Th), and trabecular separation (appTb.Sp)] in the distal femur, cortical bone architecture [cortical volume, medullary volume, total volume, polar moment of inertia (J) and section modulus (Z)] in the midfemur, muscle volume in the midthigh and femur length were assessed using magnetic resonance imaging. Total and moderate-to-vigorous physical activities were assessed using an accelerometer-based activity monitor worn around the waist for four days. Calcium intake was assessed using diet records. Relationships among the measures were tested using multiple linear regression analysis. Muscle volume was moderately-to-strongly related to measures of trabecular bone architecture [appBV/TV (r=0.81), appTb.N (r=0.53), appTb.Th (r=0.67), appTb.Sp (r=-0.71); all p<0.001] but more strongly related to measures of cortical bone architecture [cortical volume (r=0.96), total volume (r=0.94), Z (r=0.94) and J (r=0.92; all p<0.001)]. Similar relationships were observed between femur length and measures of trabecular (p<0.01) and cortical (p<0.001) bone architecture. Sex, physical activity and calcium intake were not related to any measure of bone architecture (p>0.05). Because muscle volume and femur length were strongly related (r=0.91, p<0.001), muscle volume was scaled for femur length (muscle volume/femur length(2.77)). When muscle volume/femur length(2.77) was included in a regression model with femur length, sex, physical activity and calcium intake, muscle volume/femur length(2.77) was a significant predictor of appBV/TV, appTb.Th and appTb.Sp (partial r=0.44 to 049, p<0.05) and all measures of cortical bone architecture (partial r=0.47 to 054; p<0.01). The findings suggest that muscle volume in the midthigh is related to trabecular bone architecture in the distal femur of children. The relationship is weaker than the relationship between muscle volume in the midthigh and cortical bone architecture in the midfemur, but the discrepancy is driven, in large part, by the greater dependence of cortical bone architecture measures on femur length.

When hip displacement in children with cerebral palsy (CP) is identified early, treatment is more successful. The standard test is a radiograph of the pelvis measuring the migration index (MI). Our study aims to review published literature of the natural history of hip dislocation among children with CP and to define related risk factors to develop screening criteria for early recognition. The review included 10 studies with sample sizes greater than 20 children with CP below18 years who had hips with no surgical intervention or dislocation at initial presentation, minimum 2-year follow-up, and recorded MI, pattern, and Gross Motor Function Classification System (GMFCS) level. On the basis of this review, we suggest screening with 1 radiograph for GMFCS I and II, or, if MI>30%, an annual radiograph between ages 2 and 8 years, followed by a radiograph every 2 years until the age of 18 years. For GMFCS III, IV, and V, we recommend an annual radiograph if MI<30% or 1 every 6 months if MI>30% between ages 2 and 8 years, followed by radiograph every 2 years until the age of 18 years. Applying a practical surveillance program for children with CP can prevent hip dislocation, provide early treatment, and ultimately lead to consistently better outcomes than those of neglected hip dislocations. The GMFCS level has a strong impact on subluxation risk and that the risk continues to the end of growth. Level III-systematic review.

We found that the underdeveloped trabecular bone microarchitecture in the distal femur of children with cerebral palsy (CP) who are unable to ambulate independently becomes more pronounced with increased distance from the growth plate. This suggests that the degree of underdevelopment in trabecular bone in children with CP is greater than previously understood. Children with CP who are unable to ambulate independently have severely underdeveloped trabecular bone microarchitecture in the distal femur. The aim of the study was to determine if the level of underdevelopment in trabecular bone microarchitecture is consistent across the distal femur in children with CP. Children with quadriplegic CP and typically developing children were studied (n=12/group, 5-14 years). Apparent bone volume to total volume (appBV/TV), trabecular number (appTb.N), trabecular thickness (appTb.Th), and trabecular separation (appTb.Sp) were estimated in each of 20 magnetic resonance images collected above th...

It is not uncommon for children with cerebral palsy (CP) to be discovered dead during sleep (DDDS); however, the factors associated with this pattern of mortality remain unknown. The current study aims to describe the mortality associated with children with CP from a single, tertiary care center who were DDDS. A retrospective (case-only) design to examine proportionate mortality and patient characteristics and co-morbidities that may be related to children DDDS between 1993 and 2011. There were 177 patients with CP whose deaths were reported to our institution during the study period, of which 19 were DDDS at home. The period proportionate mortality (PPM) was 114.5 per 1000. The average age at time of death was 17 years and 6 months (minimum, 6 years; maximum, 25 years). All but one of the DDDS patients had gastrointestinal feeding tubes, seizure disorders, respiratory disorders, and were non-ambulatory. Very importantly, our DDDS patients manifested clusters of respiratory disorder...

To our knowledge, there have been no previous studies addressing the indications for and the results of treatment of patients with cerebral palsy and concomitant kyphosis or lordosis without scoliosis. The purpose of the present study was to identify the indications for and the results of treatment of patients with cerebral palsy who have a spinal curve deformity solely in the sagittal plane. We conducted a retrospective review of the data on all patients with cerebral palsy who had a sagittal plane spinal deformity but no coronal plane deformity, had undergone posterior spinal fusion with unit rod instrumentation at our institution, and had been followed for at least two years. Medical records and radiographs were reviewed for symptoms, type and magnitude of deformity, age at surgery, duration of surgery, nutritional status, complications, and concomitant medical problems. Twenty-four patients-ten boys and fourteen girls-were identified. Eight patients had a hyperlordotic deformity...

An unblinded comparison of laboratory values between patients with idiopathic and neuromuscular scoliosis. To compare standard tests of coagulation and thromboelastography (TEG) parameters between two groups of patients undergoing posterior spinal fusion (PSF). Children with neuromuscular scoliosis such as cerebral palsy have more intraoperative blood loss than children with idiopathic scoliosis during PSF. Various reasons suggested for this include nutritional deficiencies, altered tissue integrity, hepatic dysfunction, and use of antiepileptic medications that can cause poor hemostasis and altered coagulation. We have observed alterations in coagulation factor levels in patients with cerebral palsy due to spastic quadriplegia with moderate blood volume loss (25% estimated blood volume). In a prospective analysis, we compared standard tests of coagulation (prothrombin time [PT], partial thromboplastin time [PTT], platelet count, fibrinogen levels) and TEG at baseline and at a blood loss of 15% estimated blood volume in patients with idiopathic scoliosis and cerebral palsy undergoing PSF. There were no differences between the groups in terms of gender distribution and age. There was a significant difference between the baseline PT and PTT values, although both groups were within laboratory norms. After 15% blood volume loss, there were differences seen in the PT, PTT, maximum amplitude on the TEG, ionized calcium, and serum magnesium levels (P < 0.05). Children with cerebral palsy undergoing PSF have increased bleeding that starts earlier in the procedure than it does for patients with idiopathic scoliosis undergoing PSF. We found that, even though children with spastic quadriplegia had baseline PT and PTT values within normal limits, they were significantly different when compared with normal patients.

ABSTRACT Study Design Matched cohort. Objective To compare the unit rod instrumentation (UR) technique with all–pedicle screw (PS) constructs in the surgical care of scoliosis in Gross Motor Function Classification System IV/V non-ambulatory spastic quadriplegic cerebral palsy patients. Summary of Background Data Over the past 20 years, there has been a transition from the UR technique to the use of pedicle screws and iliac screws in neuromuscular scoliosis. To date, no head-to-head comparative analysis has been reported between the UR technique and PS constructs for posterior segmental spinal instrumentation and fusion in cerebral palsy patients. Methods A matched cohort study was performed between 2 tertiary-care pediatric centers: 1 using UR technique and the other PS constructs. Minimum follow-up was 2 years postoperatively (PS 2.5 years, UR 4.6 years, not significant). Fourteen patients were matched from each center based on age (mean age: PS 15.4 years, UR 15.5 years), preoperative pelvic obliquity (mean: PS 33.8°, UR 29.1°) and major coronal Cobb angle (mean: PS 100.9°, UR 100.1°). Results There was posterior-only surgery in 14 of 14 PS and 11 of 14 UR surgeries. The final follow-up Cobb angle was lower in the PS group (13.5° vs. 34.3°, p < .05), with 86.5% correction in the PS group and 65.7% in the UR group. Final follow-up pelvic obliquity was similar (PS 8.5° vs. UR 3.3°; not significant). There were no major complications in the PS group. In the UR group, there was 1 deep infection and 1 reoperation for removal of a prominent sublaminar wire. Conclusions This is the first study to directly compare UR with PS constructs using matched patient cohorts in this patient population. All–pedicle screw constructs had better correction of coronal Cobb angle, lower blood loss, and shorter hospital stays. There was no difference in the correction of pelvic obliquity, complications, or reoperations.

Retrospective review of radiographs and charts (case-only). The aim of this study was to describe the long-term outcomes of spine fusion for neuromuscular scoliosis in children <10 years of age with cerebral palsy (CP). Severely involved children with CP may develop early onset scoliosis. The outcome of spine fusion is not clear and there are no studies focused on spine fusion in this young patient population. This is a retrospective review of 33 children who underwent spine fusion with unit rod instrumentation between 1989 and 2006 for CP neuromuscular scoliosis, aged <10 years at spine fusion, and with follow-up> 5 years. Demographic, medical, and radiographic data were retrospectively assessed. RANOVA and Kaplan-Meier survival estimates were used for data assessment. 33 of 42 patients who underwent spine fusion in this period, 19 boys and 14 girls, met the inclusion criteria. Out of 9 patients who were excluded, 3 were lost to follow up and remaining 6 died within 5 years of surgery. Mean age at surgery was 8.3 years (range, 4.4-9.9). Mean follow-up was 9.8 years (range, 5.5-15.8). Gross motor function classification system level was V in 31 patients and IV in 2 patients. Thirty-one patients (94%) had seizure disorder, 29 patients (88%) had gastric feeding tubes, and 9 patients (27%) had tracheostomy tubes. 85% of the patients had posterior only surgery. Mean Cobb angles preoperative, immediately postoperative, and at final follow-up were 85°, 21°, and 24°, respectively. Mean postoperative pelvic obliquity correction was 15°±9°, P<0.001. At final follow-up, there was no significant change from the postoperative measurements. Complications included one deep wound infection and 10 other problems. Eleven patients (28.2%) died after a mean follow-up of 5.6±3.8 years. In our cohort with early onset neuromuscular scoliosis, spine fusion was associated with minimal short and long-term morbidity, but there 28% mortality at ten years follow-up and 50% predicted mortality at 15 years.