Long-term outcome of reconstruction of the hip in young children with cerebral palsy.

We reviewed the long-term radiological outcome, complications and revision operations in 19 children with quadriplegic cerebral palsy and hip dysplasia who underwent combined peri-iliac osteotomy and femoral varus derotation osteotomy. They had a mean age of 7.5 years (1.6 to 10.9) and comprised 22 hip dislocations and subluxations. We also studied the outcome for the contralateral hip. At a mean follow-up of 11.7 years (10 to 15.1) the Melbourne cerebral palsy (CP) hip classification was grade 2 in 16 hips, grade 3 in five, and grade 5 in one. There were five complications seen in four hips (21%, four patients), including one dislocation, one subluxation, one coxa vara with adduction deformity, one subtrochanteric fracture and one infection. A recurrent soft-tissue contracture occurred in five hips and ten required revision surgery. In pre-adolescent children with quadriplegic cerebral palsy good long-term outcomes can be achieved after reconstruction of the hip; regular follow-up is required.

Comparison of one-stage versus two-stage anteroposterior spinal fusion in pediatric patients with cerebral palsy and neuromuscular scoliosis.

STUDY DESIGN: A retrospective study was performed including 45 pediatric patients with spastic quadriplegic cerebral palsy and neuromuscular scoliosis who underwent anteroposterior spinal fusion. OBJECTIVES: To evaluate the outcomes and complications of one-stage and two-stage combined anteroposterior spine fusion and to document which procedure is more efficacious and provides better results. SUMMARY OF BACKGROUND DATA: Circumferential spinal arthrodesis has been proven to achieve better scoliotic curve correction, decreasing significantly the risk of pseudarthrosis and progression of the deformity. There have been a few studies comparing same-day versus staged anteroposterior spinal surgery in mixed populations with neuromuscular scoliosis, but not in an isolated group of pediatric patients with spastic cerebral palsy. METHODS: The medical records and radiographs of all patients were reviewed, and the results were statistically analyzed. The complications were divided into medical, subcategorized into major and minor, and technical. RESULTS: There was no statistically significant difference (P > 0.05) between one-stage (Group 1) and two-stage (Group 2) patients, considering age at surgery, preoperative scoliosis angle, pelvic obliquity, kyphosis angle, lordosis angle, levels of anterior release, percentage of scoliosis correction, radiographic follow-up, hospitalization time, and intensive care unit stay. Sequentially performed spinal procedures (Group 1) were associated with increased intraoperative blood loss, prolonged operative time, and a considerably higher incidence of medical and technical complications, including two perioperative deaths. CONCLUSION: Two-stage anteroposterior spinal fusion provides safer and more consistent results with several advantages over the single-stage procedure in the management of patients with cerebral palsy and neuromuscular scoliosis.

Preserving ambulatory potential in pediatric patients with cerebral palsy who undergo spinal fusion using unit rod instrumentation.

STUDY DESIGN: A retrospective study investigated 24 ambulatory pediatric patients with spastic cerebral palsy and neuromuscular scoliosis. OBJECTIVE: To evaluate the effect of spinal fusion from T1-T2 to the sacrum with pelvic fixation using unit rod instrumentation on the ambulatory potential of these patients. SUMMARY OF BACKGROUND DATA: Spinal deformities in patients with cerebral palsy and good ambulatory capacity are infrequently associated with pelvic obliquity, so instrumented spinal fusions traditionally do not extend to the pelvis. METHODS: The medical charts and radiographs were reviewed, and the patients' ambulatory ability was assessed clinically with videotape or complete gait analysis. A questionnaire assessing patients' functional improvement was given to the caretakers. RESULTS: The study group included 17 female and 7 male patients, among whom were 19 quadriplegics and 5 diplegics. The mean age at surgery was 15.4 years. Of the 24 patients, 20 underwent posterior spinal fusion and 4 had combined anteroposterior procedures. The patients were evaluated clinically before surgery and after surgery. Follow-up evaluations of ambulatory function occurred at a mean of 2.86 years after surgery. No alteration in the ambulatory status of the patients was found, except in one patient who experienced bilateral hip heterotopic ossification and gradually lost her ability to ambulate. Preoperative and postoperative gait analysis was performed for 12 patients, showing no change in their ambulatory function. The surgical outcome survey demonstrated significant improvement in the patients' physical appearance, head and trunk balance, sitting ability, and respiration, with no change in ambulatory capacity. CONCLUSIONS: Spine surgery with fusion extending to the pelvis in ambulatory patients with cerebral palsy provided excellent deformity correction and preserved their ambulatory function.

Factors predicting postoperative complications following spinal fusions in children with cerebral palsy.

A retrospective review of 107 patients with cerebral palsy who had undergone a posterior spinal fusion with unit rod instrumentation by the same two surgeons was done to determine what factors cause complications that lead to delayed recovery time and a longer than average hospital stay. The operative risk score was developed with scores for the child's ability to walk and talk, oral feeding ability, cognitive ability, and medical problems within the year prior to surgery. Operative risk score is primarily a measure of degree of neurologic involvement. The postoperative complication score (POCS) is a combined measure of all postoperative complications including factors for prolonged intubation, intensive care unit stay, hospital stay, and delayed feeding. The mean age at surgery was 14.3 years. The mean weight was 29.5 kg, with 89 of 107 patients below the fifth percentile for weight compared with age. The mean degree of spinal deformity was 75.2 degrees (range 43-120 degrees ). The mean weight for age was -1.96 SD below the normal. The mean operative time was 4.3 h, with estimated blood loss of 1.2 blood volumes. The mean length of hospitalization was 23 days 2 h, with 5 days 2 h in the intensive care unit. The operative risk score and weight for chronological age below the fifth percentile showed statistical significance (p = 0.05) in regard to increased POCS. The weight for height-age and deficient total lymphocyte count, both factors that measure nutritional status, showed no statistical significance (p > 0.05) compared with POCS. Curves with deformity of >70 degrees had statistically significant high POCS (p = 0.03). Complications for patients having a posterior and an anterior surgery versus those who had a posterior fusion alone were not statistically different (p > 0.05). The factors that led to a greater rate of complications were the severity of neurologic involvement, severity of recent history of significant medical problems, and severity of scoliosis.

Anterior hip dislocation in children with cerebral palsy.

This is a report of 27 hips with anterior dislocation in 17 children with cerebral palsy. Type 1 consists of patients with extension-external rotation and adduction contracture of the hip and extension contracture of the knee; type 2 consists of patients with extension-external rotation and abduction contracture of the hip and flexion contracture of the knee; and type 3 consists of patients without contractures. All children with types 1 and 2 were unable to sit and were forced into an almost full-time lying position. No child with type 3 pattern had sitting troubles. Hip pain was present in 50% of patients. All children with type 1 pattern and half of children with types 2 and 3 developed a thoracolumbar kyphosis. Standard radiographic hip measurements were inconsistent, and only three-dimensional computed tomography scans were useful in fully assessing the deformity. Indications for hip surgery were inability to sit or hip pain or both. Reconstruction was performed by anterior superior acetabular reconstruction and varus-shortening femoral osteotomy or proximal femur resection as a salvage procedure. Although three of the 13 children required two procedures, 11 of 13 children who underwent 16 hip procedures had stable and painless hips at the time of follow-up.

Cerebral palsy.

Cerebral palsy is caused by a static lesion to the cerebral motor cortex that is acquired before, at, or within 5 years of birth. Multiple causes for the condition exist and include cerebral anoxia, cerebral hemorrhage, infection, and genetic syndromes. Cerebral palsy is commonly classified according to the type of movement problem that is present (spastic or athetoid) or according to the body parts involved (hemiplegia, diplegia, or quadriplegia). To care for children with cerebral palsy, a team approach is most effective; the team should include the pediatrician and orthopedist, among others. In the nonambulatory patient, good sitting posture, the prevention of hip dislocation (spastic hip disease), and the maintenance of proper custodial care are prime concerns. Careful monitoring and treatment of spastic hip disease and the correction of scoliotic spinal deformity are also important. In the ambulatory patient, the main goal is to maximize function. Computerized gait analysis in patients with complex gait patterns helps to show whether orthotic or surgical treatment is indicated. In this paper, we also review both the proper indications for orthopedic intervention in patients with upper extremity involvement and recent methods to control spasticity, such as selective dorsal rhizotomy and administration of botulinum toxin or intrathecal baclofen.

Soft-tissue release for spastic hip subluxation in cerebral palsy.

Children with spastic hip subluxation secondary to cerebral palsy were treated with a standard protocol that focused on early detection of the subluxation using physical examination and anteroposterior pelvis radiographs. Using limited hip abduction of < or =30 degrees and subluxation of > or =25% migration percentage as indications, patients had open adductor and iliopsoas lengthenings with immediate postoperative mobilization and no abduction bracing. The protocol was applied to 74 children with a mean age of 4.5 years and had 147 hips surgically addressed. Of these hips initially, 20% were normal (migration percentage <25%), 52% were mildly subluxated (migration percentage 25-39%), 22% were moderately subluxated (migration percentage 40-59%), and 6% were severely subluxated (migration percentage > or =60%). At a final postoperative follow-up of 39 months, 54% of these hips were classified as good (migration percentage <25%), 34% were fair (migration percentage 25-39%), and 12% were poor (migration percentage > or =40%). Of this patient population, 69% were nonambulators and their outcomes were not statistically different from children who could walk. No child developed an abduction contracture or wide-based gait that required treatment. With early detection and applying this treatment algorithm, 80% of children with spastic hip disease should have good or fair outcomes. Longer follow-up will be required to determine how many children will need bony reconstruction to maintain stable and located hips at the conclusion of growth.

The effect of rectus EMG patterns on the outcome of rectus femoris transfers.

Rectus femoris transfer to the sartorius is performed in children with cerebral palsy to treat stiff-knee gait. To determine whether preoperative electromyographic (EMG) activity of the rectus femoris is predictive of outcome, we studied 25 children with stiff-knee gait who had preoperative EMG gait analysis before rectus femoris transfer. Fifteen patients had bilateral surgery, and 10 patients had unilateral surgery. The mean age at surgery was 9.6 years for the retrospective review. Patients were divided into three groups based on the recorded EMG patterns of the rectus femoris during the gait cycle. Group I patients had predominant swing-phase activity only. Group II patients had constant rectus activity through the entire gait cycle. Group III patients had normal rectus, defined as minimal EMG activity in the last 75% of swing phase. A repeated gait analysis at a mean of 1.5 years after surgery was available for comparison. In group I, mean peak knee flexion increased 26 degrees after surgery from 44 to 70 degrees. In group II, mean peak knee flexion increased 18 degrees after surgery from 51 to 69 degrees. In group III, mean peak knee flexion increased 12 degrees from 54 to 66 degrees. Results of this study show the greatest improvement in outcome, as measured by knee flexion, occurred in group I in which the rectus fired predominantly in swing phase. Preoperative EMG patterns are therefore useful in determining the outcome after rectus femoris transfer to the sartorius.

Reconstruction of the dysplastic spastic hip with peri-ilial pelvic and femoral osteotomy followed by immediate mobilization.

All children with cerebral palsy who had a pelvic osteotomy performed by the senior author (F.M.) from 1989 through 1991 were reviewed. Indications for operative reconstruction were failed muscle lengthening in a child younger than 8 years or a painful hip. The operative procedure included adductor muscle lengthening, varus shortening femoral osteotomy, and peri-ilial pelvic osteotomy. Patients were immediately mobilized after surgery by physical therapy. Fifty-one children had reconstruction of 49 subluxated and 21 dislocated hips. Femoral and pelvic osteotomies were performed on 59 hips, and 11 hips had only a femoral osteotomy. Forty-nine hips had adductor muscle lengthening, and 27 hips had femoral osteotomy to provide for relief of contractures. At mean follow-up of 34 months, two hips in two patients had redislocated, requiring repeated surgery. Two hips remained subluxated and asymptomatic. Twenty-three hips in 18 patients were painful before surgery. One hip continued with severe pain after surgery, requiring further surgery. Three hips continued with mild pain not requiring surgery, and 14 (82%) hips had complete pain relief. Of 37 caretakers interviewed, 80% felt the procedure was beneficial and would recommend it to others. Eight percent were uncertain, and 6% (two caretakers) thought it was not helpful.

Evaluation of limp in children.

The limping child is a common and often challenging problem for the pediatrician. A practical guide is given to the evaluation of normal and abnormal gait, important historical and physical findings, and laboratory and radiographic studies. The differential diagnosis is quite variable, ranging from benign to very serious life- or limb-threatening possibilities. Some of the more common and important conditions are summarized.

Serotonergic response to spinal distraction trauma in experimental scoliosis.

The effects of distraction injury to the spinal cord on serotonin (5HT) content and metabolism in a rat model of scoliosis were studied. Previous studies in this laboratory (Salzman et al., 1987a) have identified the 5HT response as a major component of the posttraumatic progression of spinal injury after impact trauma in the rabbit. The present study was designed to determine the universality of this response by examining a different model of injury in a different species. The results demonstrate that distraction trauma in the rat, like impact injury in the rabbit, is associated with a rapid and robust increase in the local spinal cord content and metabolism of 5HT and a long-term depletion of 5HT below the site of injury. The roles of the blood platelet and the raphe-spinal tract in the acute response and the disruption of axoplasmic transport during the chronic phase of injury are discussed.

Experimental scoliosis in the rat. II. Biomechanical analysis of the forces during Harrington distraction.

The purpose of this study was to characterize the forces resulting from Harrington distraction of the spine in an experimental model of scoliosis in the rat, in order to establish both the similarity of this model to human scoliosis and identify potential force parameters that may be useful for clinical decision-making. Harrington distraction was performed in 36 rats that had been made scoliotic 9-12 weeks earlier by the method described in the previous paper. Distractions were carried out in discrete and timed steps until separation of the vertebral laminae (mechanical failure) occurred at the upper hook site. Distractive forces were monitored continuously by a strain gauge mounted on the tension side of the upper arm of the outrigger. The resulting data were compared among the various curvature groups. The relationship between the length of distraction and the maximum force produced was similar for all animals regardless of curvature. This relationship was quadratic and was characterized by an inflection point where forces increased rapidly with each distraction. The amount of distraction necessary to reach both the inflection and failure points differed only for curves above 100 degrees. The amount of force required to reach failure was lower for curves above 75 degrees. Curves above 50 degrees had a lower percent correction at the inflection point. Bending and tensile forces were calculated by vector analysis. Axial load efficiencies were greater for curves above 50 degrees, as evidenced by increased bending forces in these animals. The viscoelasticity of the spine decreased after inflection in all animals.(ABSTRACT TRUNCATED AT 250 WORDS)

Experimental scoliosis in the rat. I. Methodology, anatomic features and neurologic characterization.

The purpose of this study was to develop a simple and reproducible model of scoliosis in the rat without producing direct trauma to the spine, in order to both provide a system for the study of human scoliosis and to ultimately study the conditions under which distraction-induced trauma might occur. Right lateral curvatures were produced in three groups of rats (N = 36, 59, 25) at 18-21 days of age by suturing the inferior angle of the scapula to the ipsilateral bony pelvis. In the first group of animals, it was found that a minimum period of 6 weeks of tethering was required in order to produce permanent structural curvatures after release of the tethering sutures. In the second group of rats, a number of morphologic and histologic changes characteristic of human scoliosis were noted, including apical wedging, deviation of the apical spinous process, pelvic asymmetry, rib hump deformity, vertebral rotation, displacement of the nucleus pulposus, disorganized columnization of disc cartilage cells, and an increased number of Type I muscle fibers in paravertebral muscle on the convex side of the curve. Finally, in the third group of rats, a mild spasticity was seen in the ipsilateral hindlimb in approximately one-third of the animals with curvatures greater than 40 degrees. This spasticity was not associated with a change of latency or amplitude of the somatosensory-evoked potential (SSEP). Each curvature group displayed SSEP characteristics that were not statistically different from unoperated control animals. These results indicate the validity of this model system for the study of scoliosis, and, in particular, its usefulness for the study of operative-induced trauma.(ABSTRACT TRUNCATED AT 250 WORDS)

The somatosensory evoked potential predicts neurologic deficits and serotonergic pathochemistry after spinal distraction injury in experimental scoliosis.

The validity of the somatosensory evoked potential as an intraoperative spinal cord monitor was evaluated in an experimental model of scoliosis in the rat and a Harrington distraction model of injury. Under these conditions, it was found that any change in latency or amplitude of the major negative wave above a certain level was a significant predictor of an adverse neurologic outcome. Changes in latency of 4% or greater and changes in amplitude of 50% or greater were unequivocal indicators of spinal cord injury. Postmortem analyses of the spinal neurotransmitter serotonin revealed that apparent false-positive results of the SEP were, in fact, true-positive results.

Recurring digital fibrous tumor of childhood: case report with long-term follow-up and review of the literature.

A case report and literature review of recurring digital fibrous tumors of childhood are presented. Of 92 reported cases, 78 patients were treated surgically. Tumor excision ultimately was curative in 85%, with 64% having recurrences and 10% needing amputation. Our patient, the first with follow-up of greater than 10 years, had extensive tumors of the hands and feet and eventually required partial amputation of many digits. At 19 1/2 years of age, no recurrences were noted. Recurring digital fibrous tumors of childhood should be treated with early surgical excision, with amputation reserved for cases of severe and progressive lesions.