Steven E. Koop

Rectus femoris transfer to improve knee function of children with cerebral palsy.

Stance phase stability and swing phase clearance, prerequisites for normal ambulation, often are lost in the gait of children with cerebral palsy. Lengthening of the hamstrings usually will improve stance‐phase knee extension but will not greatly alter swing‐phase knee flexion. This paper presents the outcome of transfer of the distal end of the rectus femoris in conjunction with hamstrings lengthening in 37 knees, and compares it with a control group of 24 knees in which only hamstrings lengthening was done. In the first group swing‐phase knee flexion was improved by 16‐0± 14‐4o, compared to 9‐5±7‐5o in the control group, and residual knee flexion in stance was reduced to 8–9±8‐1o, compared to 15. 1 ± 13‐8o in the controls. Poor outcome in the transfer‐plus‐lengthening group was associated mainly with foot rotation in excess of 8o internally or externally, or postoperative knee flexion in stance. Criteria for selection of cases and methods of improving surgical outcome are discussed.

Spinal instrumentation without fusion for progressive scoliosis in young children

Between 1973 and 1993, a heterogeneous group of 67 children with progressive scoliosis entered a program of incremental-distraction spinal instrumentation without fusion supplemented by full-time external orthotic support. Over the course of treatment, curve magnitude improved from an average of 67 degrees at initial instrumentation to 47 degrees at definitive fusion. For all patients, curve response tended to decline with consecutive procedures. The measured growth of the instrumented but unfused spinal segments averaged 3.1 cm over a mean treatment period of 3.1 years. The results of our study suggest that spinal instrumentation without fusion can control progressive scoliosis in a majority of children while allowing normalized growth of instrumented spinal segments. The mean duration of treatment and ultimate gain in spinal length are constrained by progressive structural changes that alter curve response to incremental distraction. Despite these limitations, spinal instrumentation without fusion may provide a reasonable management alternative when individualized among these difficult patients.

The surgical treatment of instability of the upper part of the cervical spine in children and adolescents.

In a retrospective review of the cases of thirteen skeletally immature children and adolescents (four to eighteen years old) with instability of the upper part of the cervical spine (occiput to fifth cervical vertebra), we determined the efficacy of posterior arthrodesis and halo-cast immobilization in the management of this condition. The patients were divided into two groups: those with congenital vertebral anomalies alone (fusion or structural defects, or both) and those with cervical anomalies and systemic disorders (dwarfism, juvenile rheumatoid arthritis, Down syndrome, and cerebral palsy). Two patterns of instability were found: instabilities at intervertebral joints adjacent to vertebral fusions, and instabilities located in vertebral defects. For all patients treatment included a posterior arthrodesis with external immobilization by a halo cast, and in two patients internal fixation with wire was also used. Solid arthrodesis was obtained in the twelve patients who were treated with autogenous grafts (iliac cancellous bone in eleven and rib bone in one), and a non-union developed in a child who was treated with bank-bone rib segments. Posterior cervical arthrodesis with wire fixation carries some risk of neural injury and often is not applicable in children with anomalous vertebrae. Spine fusion using delicate exposure, decortication using an air-drill, and placement of autogenous cancellous iliac grafts with external immobilization by a halo cast minimizes the risk of neural damage and is a reliable way to obtain a solid arthrodesis.

Syringomyelia and scoliosis: a review of twenty-five pediatric patients.

A retrospective review of 25 patients with scoliosis secondary to syringomyelia was conducted. All patients had large syrinxes, but only 10 patients had abnormal neurologic findings, suggesting that subtle neurologic findings in patients with scoliosis should not be underestimated and supporting liberal use of magnetic resonance imaging (MRI). The results of three approaches to the care of these patients were reviewed: passive observation, bracing, and spinal fusion. Curves progressed > or = 5 degrees in patients aged < 10 years who were observed, but in children aged > 10 years, curves progressed > or = 10 degrees. Curves responded well to bracing, but correction was not maintained out of brace for patients with progressive and/or severe curves. Good corrective was achieved safely during operation.

Determination of "normal" thoracic kyphosis: a roentgenographic study of 121 "normal" children.

The purpose of this prospective study was to determine the range of “normalcy” in the radiologic measurement of thoracic kyphosis in children by using a standardized position and full-length radiograph in 121 normal children. Using ± two standard deviations from the mean as the definition of “normal,” a range of 20°–50° was determined. There was no difference between the various age subgroups or between genders.

Measurement Accuracy in Congenital Scoliosis

Background: The accuracy of measurement of curves in idiopathic scoliosis has been extensively studied; however, we know of only one article in the literature concerning the accuracy of measurement of curves in congenital scoliosis. That article stated that intraobserver variability was ± 9.6° and interobserver variability was ± 11.8°. Methods: Sixty-nine curves in fifty patients with congenital scoliosis were measured on two separate occasions by seven different observers with varying experience in curve measurement. Results: Mean intraobserver variance ranged from 1.9° to 5.0°, with an average of 2.8° (95% confidence limit, ± 3°) for the seven observers. The interobserver variance was 3.35° (95% confidence limit, 7.86°). Conclusions: It is possible to measure curves in congenital scoliosis with much greater accuracy than previously reported. In the clinical situation in which a skilled observer can measure two radiographs at the same time, an accuracy of ± 3° can be expected 95% of the time.

Results and complications after spinal fusion for neuromuscular scoliosis in cerebral palsy and static encephalopathy using luque galveston instrumentation: Experience in 93 patients

Study Design. Retrospective cohort analysis. Objective. To evaluate the results of spine fusion for neuromuscular scoliosis in cerebral palsy and static encephalopathy, using Luque-Galveston technique, with emphasis on the early and late complications, especially those increasing the hospital stay or requiring additional surgery. Summary of Background Data. There are numerous studies in the literature on the treatment of neuromuscular scoliosis using Luque-Galveston instrumentation analyzing the results and complications. Most series are small and evaluate some of the complications, with none evaluating all the early and late complications and none assessing the impact of the complication on length of hospitalization or the need for additional surgical intervention. Methods. This was a retrospective review of a consecutive series of patients operated on for neuromuscular scoliosis in cerebral palsy and static encephalopathy with Luque-Galveston instrumentation at 1 institution from January 1997 to December 2003. Ninety-three patients were identified. Results. The average age at surgery was 14.3 years, with an average age at follow-up of 18.2 years, and an average follow-up of 3.8 years. The mean preoperative scoliosis was 72° with correction postoperatively to a mean of 33°, maintained at 36° at final follow-up. There were 83 early complications in 54 patients, for a complication rate of 58% of patients for the entire study. There were no perioperative deaths or neurological complications. There was only 1 deep wound infection, for an infection rate of 1.1%. Patients with 1 complication had a longer length of stay, 9 versus 7 days, the difference being statistically significant (Mann-Whitney U test, P < 0.001). Two patients required reoperation during the initial hospitalization (1.1%): 1, one for infection and 1 for proximal hook cutout and proximal junction kyphosis. There were a total of 81 late complications in 44 patients, for a late complication rate of 47% of patients for the entire study. The majority of the complications were minor, not requiring additional care or surgery. Seven patients had a pseudarthrosis (7.5%), presenting at an average of 30 months postoperatively. Eight patients underwent 9 procedures for late complications: 5 for repair of a pseudarthrosis, 3 for removal of a prominent iliac screw, and 1 for superior junctional kyphosis. All the pseudarthrosis repairs were solid at follow-up. Conclusion. Spinal fusion in neuromuscular scoliosis with Luque-Galveston technique is a safe and effective procedure. Any early complication increased the length of stay, with a low rate of reoperation during the hospitalization. The majority of late complications were minor, not requiring additional care. Pseudarthroses were detected late and were the main reason for additional surgery.

Scoliosis in cerebral palsy

Spinal deformity is a common musculoskeletal problem for individuals with cerebral palsy. Severe scoliosis may impair physical function and may be a source of pain. Spine braces and carefully constructed seating arrangements may moderate the behavior of these deformities but do not seem capable of stopping progression, which often continues in adulthood. Spine fusion surgery can produce a stable, durable trunk shape that improves sitting and positioning but the process of surgery is arduous and outcomes can be compromised by numerous serious complications. Despite complications, many families and caregivers express satisfaction with the results of surgery. Careful patient evaluation, studious attention to surgical planning and performance, and a good relationship with patients, family members, and other providers is essential.

Scoliosis and Rett syndrome

dren with spina bifida have long expressed concern that once their children are ineligible for services in the pediatric system, they are very much left to their own devices in terms of securing, for example, health care, independent living support, and employment. Successful transition from life as a child to that of an adult requires a life course approach and plan which can be difficult in light of the fact that some individuals with spina bifida have a cognitive profile that may challenge such planning. Much work remains to be done on realizing the concept of transition for those with spina bifida; moving from life as a child with significant care needs to that of an adult who can meet those needs, as well as meeting other psychosocial needs, as independently as possible. Continued systematic exploration of this area is greatly needed so that recommendations and policies can be based on empirical evidence. By empirically identifying modifiable facilitators and barriers to successful transitioning for individuals with spina bifida and other complex conditions, we will be able to help in delineating, at an early stage, who may require specific assistance, what type of assistance, and when to provide the assistance. Bellin et al.’s article is encouraging in that it not only addresses relevant topics to transition, such as self-management and employment, but it also takes into consideration other variables that may greatly impact on the trajectory of young adults with spina bifida, such as sex and level of lesion. Such information will prove very useful once we more actively start to implement research findings into practice.

Factors influencing recurrent admissions among children with disabilities in a specialty children's hospital

PURPOSE To describe recurrent admissions in a cohort of complex chronic patients at a specialty childrens hospital, identify factors that contribute to multiple admissions, and test the hypothesis that risk factors predict patterns of readmissions within specified time intervals. METHODS Retrospective cohort analysis of patients admitted to a specialty childrens hospital during calendar year 2006 followed through 2011. Administrative and medical record abstracted data were analyzed by the total number of recurrent admissions and by readmissions with 7, 30 and 90 days at any point during the five year study period. RESULTS One thousand two hundred and twenty-nine patients with 2295 inpatient admissions were examined. Four hundred and sixty-seven patients (38%) experienced at least one additional inpatient admission at any time during the study period. Eight variables were significant risk factors for subsequent admission at any time during the study period: indwelling technology, mobility support, critical care consultation, medical (vs. surgical) admission, mean LOS across all admissions, number of scheduled medications at discharge, insurance on index admission, and gross charges on index admission. Presence of indwelling technology, increasing numbers of scheduled medications at discharge and Nervous System APR-DRG diagnoses were significant factors predicting readmission within 7, 30, and 90 day intervals. CONCLUSIONS Within this population of complex chronic patients risk factors were identified that predict vulnerability to recurrent admissions suggesting that further research is needed to address a unique subset of complex chronic patients and the complement of systems organized to provide health care delivery services for them.