James F. Mooney

Management of cerebral palsy with botulinum-A toxin: preliminary investigation.

Summary: Use of intramuscular botulinum-A toxin (Botox) to produce neuromuscular blockade has been effective in treating certain ocular and facial muscular imbalances as well as spasmodic torticollis. In this preliminary open study, the effectiveness of intramuscularly injected Botox on the muscular imbalances of cerebral palsy was assessed in 27 pediatric patients. Each patient had “dynamic deformities” unresponsive to other treatment, and operation was the only other realistic alternative. The dose of Botox was calculated on a unit/body weight basis. In ambulatory patients, clinical changes in gait were assessed by a physicians rating scale. Reduction in spasticity became apparent in 12–72 h after injection; the effect of Botox after target threshold was reached lasted 3–6 months. No major side effects occurred. Botox may prove a useful adjuvant in conservative management of the spasticity of cerebral palsy. Successful management with these injections may allow delay of surgical intervention until the child is older and at less risk of possible complications, including the need for repeated surgical procedures.

Botulinum Toxin Type A Neuromuscular Blockade in the Treatment of Lower Extremity Spasticity in Cerebral Palsy: A Randomized, Double-Blind, Placebo-Controlled Trial

Increased gastrocnemius/soleus muscle tone in children with cerebral palsy may cause an equinus of the ankle. Botulinum toxin type A (BTX), a neuromuscular blocking agent, reduces muscle tone in various neuromuscular disorders. The safety and short-term efficacy of BTX injections were evaluated in a prospective, 3-month, double-blind, randomized clinical trial involving 114 children with cerebral palsy and dynamic equinus foot deformity. Outcome was determined by observational gait analysis, ankle range-of-motion measurements, and quantification of muscle denervation by nerve conduction. Patients in the BTX group demonstrated improved gait function and partial denervation of the injected muscle. No serious adverse events were reported.

Treatment of femoral fractures in children by pediatric orthopedists: results of a 1998 survey.

This study aimed to determine treatment preference of various femoral fracture patterns in children by pediatric orthopedists and whether it is practice dependent. In September 1998, members of the Pediatric Orthopedic Society of North America were surveyed to determine their current preferences in treating each of four middle one-third femoral fracture patterns in four age groups. Forty-four percent (286/656) of those surveyed responded. For each fracture pattern, operative treatment was increasingly preferred over nonoperative as patient age increased, and the preferred treatments within the operative and nonoperative categories changed significantly as patient age increased. Fourteen specific cases of femoral head avascular necrosis were noted after rigid reamed and unreamed rodding. There is a statistically significant trend by pediatric orthopedists to treat older childrens femur fractures operatively and younger childrens nonoperatively. The consensus treatment is age dependent. The numerous cases of avascular necrosis after rigid rodding are a concern.

Predicting Scoliosis Progression from Skeletal Maturity: A Simplified Classification During Adolescence

BACKGROUND Both the Tanner-Whitehouse-III RUS score, which is based on the radiographic appearance of the epiphyses of the distal part of the radius, the distal part of the ulna, and small bones of the hand, and the digital skeletal age skeletal maturity scoring system, which is based on just the metacarpals and phalanges, correlate highly with the curve acceleration phase in girls with idiopathic scoliosis. However, these systems require an atlas and access to the scoring system, making their use impractical in a busy clinical setting. We sought to develop a simplified system that would correlate highly with scoliosis behavior but that would also be rapid and reliable for clinical practice. METHODS A simplified staging system involving the use of the Tanner-Whitehouse-III descriptors was developed. It was tested for intraobserver and interobserver reliability by six individuals on thirty skeletal age radiographs. The system was compared with the timing of the curve acceleration phase in a cohort of twenty-two girls with idiopathic scoliosis. RESULTS The average intraobserver unweighted kappa value was 0.88, and the average weighted kappa value was 0.96. The percentage of exact matches between readings for each rater was 89%, and 100% of the differences were within one unit. The average interobserver unweighted kappa value was 0.71, and the average weighted kappa value was 0.89. The percentage of exact matches between two reviewers was 71%, and 97% of the interobserver differences were within one stage or matched. The agreement was highest between the most experienced raters. Interobserver reliability was not improved by the use of a classification-specific atlas. The correlation of the staging system with the curve acceleration phase was 0.91. CONCLUSIONS The simplified skeletal maturity scoring system is reliable and correlates more strongly with the behavior of idiopathic scoliosis than the Risser sign or Greulich and Pyle skeletal ages do. The system has a modest learning curve but is easily used in a clinical setting and, in conjunction with curve type and magnitude, appears to be strongly prognostic of future scoliosis curve behavior.

Management of Unstable/Acute Slipped Capital Femoral Epiphysis Results of a Survey of the POSNA Membership

There is limited literature regarding the treatment of unstable slipped capital femoral epiphysis (SCFE). The Evidence Analysis Work Group (EAWG) initiated a questionnaire assessing management of unstable SCFE by the members of the Pediatric Orthopaedic Society of North America (POSNA). All members were surveyed by e-mail and fax. The survey was accessible through the POSNA web site. One third of the members responded. Seventy-three percent of members used the “stable/unstable” classification, while 27% used the “acute/chronic” terminology. Thirty-one percent of respondents felt that an unstable SCFE is an emergency, while 57% felt that treatment could be on an urgent (<8 hours) basis. Fifty-seven percent reported use of a single threaded screw for fixation for an unstable SCFE, and 40.3% recommended two threaded screws. There seems to be agreement on methods of patient evaluation, but discrepancies remain in classification and fixation methods. The EAWG recommends development of multicenter studies to evaluate the treatment of unstable SCFE.

The effect of botulinum toxin type A injections on the natural history of equinus foot deformity in paediatric cerebral palsy patients

Neuromuscular blockade with botulinum toxin type A (BTX‐A) injections was employed to manage equinus foot deformity in the lower extremities of paediatric cerebral palsy patients. The patients were followed to evaluate the effect of the blockade on lower extremity function and to determine the effect of treatment on the need for tendo‐Achilles lengthening (TAL) surgery. The average length of follow‐up was 3.4 years. Of the 48 patients, 17 (35%) responded to treatment witbin 6 months of initiation of therapy, as determined by a Physician Rating Scale of gait. Overall, 25 (52%) of the 48 patients underwent TAL surgery during the follow‐up period, including eight patients who responded to the BTX‐A treatment. The average age of the patients at the time of surgery was 7 years. This age compares favourably with the age at surgery reported in the recent literature. There were no significant differences by responder status, age, or gender in those patients who did and did not require TAL surgery. No adverse events relating to BTX‐A were reported. These results indicate that patients who respond to BTX‐A treatment have improved physical functioning and gait, and are able to sustain these results long term.

Use of cross-extremity flaps stabilized with external fixation in severe pediatric foot and ankle trauma: an alternative to free tissue transfer.

Pedicled cross-extremity flaps for wound coverage have been replaced, in most cases, by free tissue transfer. Classically, cross-leg flaps have been problematic because of difficulties with immobilization and positioning of the extremities from the time of initial coverage to detachment. Three children with severe foot and ankle trauma had cross-extremity flaps using linkage of bilateral lower-extremity external fixators in place of traditional casting. Cross-leg flaps were used in two patients, and a cross-foot flap was applied in one. Each flap survived completely, and the linking fixators were disassembled at the time of flap detachment. No complications were related to the donor site or the flap itself or were caused by the fixation. Lower-extremity range of motion was regained rapidly, and each patient resumed essentially normal gait and activity. Addition of external-fixator stabilization aids greatly in wound care, as well as general ease of patient mobility and positioning. External fixation facilitates the use of cross-extremity flaps in pediatric patients in whom free tissue transfer may not be optimal.

Previously unreported structure associated with Sprengel deformity.

Congenital elevation of the scapula (Sprengel deformity) is the most common congenital abnormality involving the scapula. In up to 50% of cases, there is an associated connection, which may be osseous, cartilaginous, or fibrous, between the scapula and the lower cervical spine. To date, there have been no reports of an osseous structure extending from the medial scapula to the clavicle and occipital region of the skull associated with Sprengel deformity.

Pharmacologic management of spasticity in cerebral palsy.

Cerebral palsy (CP) results from a nonprogressive injury to the developing central nervous system (CNS) and produces motor dysfunction, movement disorders, mentation deficits, and impaired function. Approximately 500,000 to 700,000 children and adults have CP, with an annual incidence of 1.5 to 2.5 cases per thousand live births. Motor dysfunction associated with CP may include spasticity, rigidity, and weakness. Spasticity is “a motor disorder characterized by velocitydependent increase in tonic stretch reflexes that exaggerate tendon jerks, resulting in hyperexcitability of the stretch reflex” (Fig. 1). Excessive spasticity interferes with function, contributes to discomfort and pain, has a negative impact on health-related quality of life, and may produce, over time, deformities of soft tissue, joints, and bone. The most common etiology of the nonprogressive CNS lesion is an insult within the perinatal period. Insults include infection, anoxia, and hemorrhage, each of which occurs more frequently in association with prematurity, very low birthweight, and twin gestations. Although the CNS lesion occurs once and remains constant, expression of this lesion and resultant spasticity and muscle imbalance are affected by the interactions of growth, development, maturation, and disease processes that may confound the clinical picture. Traditionally, nonoperative management of orthopedic issues for patients with CP has included family-based and professional physical therapy, adaptive modalities, orthotics, and casting. The goal of these efforts is to potentiate function. Operative treatment has been used for patients with significant functional impairment, progressive deformity, or pain secondary to fixed contracture or deformity who do not respond to nonoperative treatment. In a severely involved patient, surgery may be indicated to improve positioning and/or hygiene. Recently, there has been an emphasis on management and modulation of spasticity rather than merely addressing the effects of spasticity on the trunk and extremities. Pharmacologic intervention is employed to improve function, maximize health-related quality of life, facilitate other modalities, and/or delay invasive surgical procedures. Some pharmacologic interventions, however, are invasive, and all have inherent morbidity. Pediatric orthopedic surgeons should be cognizant of pharmacologic modulation of spasticity to provide coordinated interventions, since it is often used in conjunction with other nonsurgical methods and may be a valuable adjunct to surgical procedures. There are no spasticity modulation methods that directly effect a fixed contracture, since these require orthopedic surgical intervention. There is evidence that pain associated with fixed deformity may be modulated by pharmacologic agents.

Sudden Appearance of New Upper Extremity Motor Function While Performing Neurophysiologic Intraoperative Monitoring During Tethered Cord Release: A Case Report

&NA; Tethered cord syndrome occurs when the distal spinal cord or filum adheres to adjacent structures resulting in progressive sensorimotor deficits in the lower extremities, fecal and/or urinary incontinence, and musculoskeletal deformities. Tethering of the distal cord may be idiopathic, may be associated with an intraspinal abnormality such as a lipoma, but most commonly the distal spinal cord remnant is adherent to the area of the original dysraphism repair in patients with myelodysplasia. Surgery to untether the cord is indicated in patients with worsening pain symptoms, progressive limb deformity or spasticity, or before any acute correction of an associated spinal deformity. Neurophysiologic intraoperative monitoring is used to minimize the risk of inadvertent nerve root or spinal cord injury during the untethering procedure and to assess any changes in cord function at the time of an associated spinal deformity correction. We present a patient with a lumbar level myelodysplasia, Chiari II malformation, severe scoliosis, and tethered cord that underwent concurrent scoliosis correction and tethered cord syndrome surgery, who demonstrated immediate intraoperative improvement in neurophysiologic responses in a previously flaccid upper extremity after untethering. These monitoring changes correlated with clinical improvements noted by physicians and family postoperatively. Level of Evidence Level IV.