Cerebral Palsy

BACKGROUND: In a previous randomized trial, volume controlled ventilation (VCV) was noted to be efficacious in ventilating very preterm and extremely low birth babies. OBJECTIVE: To compare long term survival, pulmonary morbidities and gross neurodevelopmental outcomes of babies randomized to either VCV or Pressure Limited Ventilation (PLV) for treatment of RDS. Design/methods: All surviving children were prospectively followed. Masked evaluation of health status, including frequency of respiratory illness such as cough and wheeze, use of medications, hospital admissions, and gross neurodevelopmental status was obtained using a structured parental questionnaire and verification from medical records. RESULTS: 94 of the 109 children (86%) survived to discharge. Three children died after discharge (VCV=2, PLV=1). Modality of ventilation did not affect overall mortality;7 in VCV group(12%) vs 11(21%) in the PLV group [OR 0.5 (0.1-1.4), p= 0.13]. Respiratory abnormalities were present in 32 (37%), and 26 (30%) required hospital readmission. There was no significant difference in readmission rates between the two groups; VC 13/45 (29%) and PLV 19/40 (47%) [OR 0.4 (0.1-1.1), p=0.07]. Modality of ventilation did not affect frequency of respiratory illness; VC 12(27%) PLV 14(35%) ( [OR 0.46 (0.1-1.1), p=0.09]. However, significantly fewer children in the VCV group (6 =13%) compared to PLV ( 13=32%) required treatment with inhaled steroids/bronchodilators [OR 0.3 (0.1-0.9, p=0.04]. Severe neurodevelopmental disability (cerebral palsy, blindness, or deafness) was present in 9 (9.8 %) (VCV= 3; PLV= 6) [OR 0.4 (0.09-1.7)]. CONCLUSIONS: The efficacy of VCV in very preterm and low birth babies appears to be maintained on longer term evaluation.

Children with cerebral palsy have various risk factors for compromised bone health. Evidence concerning their bone fragility is gathering; however, there is no consensus regarding risk factors, indications for evaluation, follow-up, or treatment. We performed an evidence-based review targeted to address the following questions concerning children with cerebral palsy: Is bone strength impaired and what are the risk factors? Are these children at increased risk for bone fractures? What are the relations between bone mineral density and fracture risk? What methods can be used for bone health assessment? How can bone strength be improved? Currently, the most acceptable method for evaluating bone status in children is dual-energy x-ray absorptiometry. Evidence demonstrates reduced bone mass in children with cerebral palsy; yet, no clear association with fractures. Preventive methods are suggested.

BACKGROUND: Hallux valgus deformity is a common sequel of spastic cerebral palsy. METHODS: Twenty ambulatory patients (24 feet) suffering hallux valgus deformity, with painful forefoot and restricted footwear, secondary to spastic cerebral palsy acquired perinatally, were treated with great toe metatarsophalangeal (MTP) arthrodesis using percutaneous K-wires for fixation. The mean age at the time of surgery was 16.2 years (range 14-18 years). They were retrospectively evaluated for the results after arthrodesis at a mean interval of 3 years and 4 months (range 3-4 years) by physical examination and radiographs. RESULTS: All patients had a stable painless aligned great toe, with <10 degrees valgus, <20 degrees dorsiflexion and neutral rotation after arthrodesis, evidenced by improvement in pain, cosmesis, functional activity, footwear, callosities and hygiene, as well as by significant improvement in the measures of the MTP and the intermetatarsal angles (IMA) by postoperative radiographs. Neither non-union (pseudoarthrosis) nor recurrence of the deformity developed. Complications included superficial wound slough in a single case. Using the modified American Orthopaedic Foot Ankle Society (AOFAS) Hallux Metatarsophalangeal-Interphalangeal Scale, 18 feet (75%) were classified as excellent and six feet (25%) as good. Neither fair nor poor cases were recorded. CONCLUSION: Hallux valgus deformity in adolescents with spastic cerebral palsy is best treated by great toe MTP arthrodesis to improve segmental foot malalignment and dynamic foot deviation.

PURPOSE: Residual midfoot and hindfoot deformities in rigidly deformed feet present a very complicated surgical dilemma. A plantigrade foot is desirous for proper lower extremity mechanics in a child with ambulatory potential. In this group of patients, soft tissue procedures are no longer an appropriate option, and well-recognized hindfoot procedures, such as talectomy, have many disadvantages. This study reviews the results obtained using multiplanar supramalleolar osteotomy as a salvage procedure to correct deformities of the complex rigid foot in children. METHODS: A retrospective review was conducted of 27 multiplanar supramalleolar osteotomies in 18 children. The underlying diagnosis of the patients included seven severely rigid idiopathic clubfeet, five arthrogryposis, two myelodysplasia, one Ellis-van Creveld, one Streeter’s, one cerebral palsy, and one severe burn contracture. The average age at surgery was 5.6 years, and follow-up averaged 8 years. A successful outcome was deemed a plantigrade foot on physical exam with follow-up of at least 2 years and no subsequent tibial surgeries. All failures were included regardless of the length of follow-up. RESULTS: A plantigrade attitude of the hindfoot was obtainable at the time of surgery in all cases. Eighteen of the 27 feet had a successful outcome. Nine of 27 (33%) feet had recurrence of the foot deformity requiring additional surgery. Time to recurrence averaged 5.7 years (9 months-13 years). Complications from the surgery included four minor wound healing problems, two delayed unions, and one screw recession, all of which healed without consequences. There was no evidence of nonunion, growth plate closure, infection, or fracture above or through screw holes. CONCLUSION: The multiplanar supramalleolar osteotomy appears to be a reasonable salvage procedure for severely scarred and complex rigid foot deformities and can be reinstituted for failures due to remaining growth.

PURPOSE: The purpose was to assess the local and distant effects of isolated calf muscle lengthening in ambulant children with cerebral palsy. METHODS: The study included fifteen ambulant children with cerebral palsy (nine with diplegia and six with hemiplegia), average age 8.8 years, Gross Motor Function Classification System (GMFCS) level I and II. None of the children had previously undergone orthopaedic surgery, apart from one child who had tendo-achilles lengthening (TAL) nine years earlier. All the children underwent pre and post-operative clinical examination and three-dimensional gait analysis (gait analysis). Twenty calf muscle lengthenings were performed, ten TAL and ten gastrocnemius recessions (GR). RESULTS: Post-operative ankle kinematics showed significant improvements in all parameters. Ankle power during push-off increased, but only significantly after TAL. Only one limb (5%) was over-corrected. Four limbs (20%) were under-corrected and one of these limbs remained in mild equinus position in stance. There was one recurrent equinus (5%) during the follow-up period of three years (range: 13-55 months). Distant effects on joints and segments were more marked in diplegia than in hemiplegia. Ten of 17 kinematic parameters distant from the ankle joint improved significant post-operatively when the preoperative values were 1SD below or above the mean of the normal material. There was no significant deterioration in any of the measured parameters. CONCLUSION: The improvement in ankle kinematics and kinetics supported the experience of other studies. The distant effects, which have previously not been evaluated in three planes, showed improvement in several kinematic parameters indicating that additional surgery in selected patients could be abandoned or delayed.

PURPOSE: The purpose of this study was to evaluate the results of distal femur extension osteotomy and medial hamstring lengthening in the treatment of fixed knee flexion deformity in patients with spastic diparetic cerebral palsy. METHODS: A retrospective study was done in a group of 12 diparetic cerebral palsy patients. A distal femur extension osteotomy was performed as part of multilevel surgery on lower limbs. The fixed knee flexion deformity was measured during physical examination, whereas hip and knee flexion in the stance phase and anterior pelvic tilt were both analyzed at kinematics. The pre- and post-surgery results were compared and analyzed statistically. A medical record review was done in order to identify the complications. The mean follow-up was 28 months. RESULTS: A significant reduction of fixed knee flexion deformity at physical examination and knee flexion in the stance phase at kinematics was observed, but with no decrease in hip flexion. As a non-desired effect, there was an increase in anterior pelvic tilt after surgical procedures. With regard to complications, a single patient had skin breakdown at a calcaneous area on one side and the recurrence of deformity was seen in 27% of cases. CONCLUSIONS: In this study, in which fixed knee flexion deformity did not exceed 40 degrees before surgery, the distal femur extension osteotomy was effective in increasing knee extension in the stance phase. However, an increase in anterior pelvic tilt, deformity recurrence and necessity for walking aids are possible complications of this procedure.

PURPOSE: To prospectively evaluate long-term effects of repeated botulinum toxin A (BTX-A) injections in children with cerebral palsy (CP). METHODS: Repeated injections of BTX-A were offered to children with CP, according to clinical indications, for a maximum of four injections within a period of two years. Injections were administered into lower extremity muscles of 26 consecutive children (age 3.7 +/- 1.2 years, 16 boys) with hemiplegic or diplegic CP. Clinical assessments before and one month following each injection included a gross motor function measure (GMFM), a modified Ashworth scale, and range-of-motion of knee extension and ankle dorsiflexion. RESULTS: Twelve children received two injections, six received three injections, five received one injection, and three received four injections. The most common reason for discontinuing treatment was the need for orthopedic surgery (n = 17). A long-term effect was demonstrated by a significant increase of the GMFM score before the first injection compared with the last injection for each patient (P < 0.0001). There was no comparable change in the muscle tone or range-of-motion. The mean rate of GMFM change during the study was significantly higher than literature norms for CP children (13.2 vs. 5.37 per year, respectively, P < 0.01). The increase of the GMFM score before and one-month after injection (short-term effect) was significantly higher after the first injection than after the last injection (P < 0.05). Similar results were found for the Ashworth scale and popliteal angle. CONCLUSIONS: Botulinum toxin A injections have a long-term effect on gross motor function in children with CP even though the effect on muscle tone is short-term. The effect apparently declines with repeated injections, with most children benefitting from 2 to 3 injections.

BACKGROUND: At birth, no difference exists between the hips of children with spastic cerebral palsy and the hips of other children. The typical pathologic deformities of the hips in children with spastic cerebral palsy develop as the child gets older. One of our goals was to evaluate the change in quality of life of children with cerebral palsy after hip reconstruction. For classification of the children’s condition, we assessed sitting and standing abilities and used the Gross Motor Function Classification System. To evaluate the effect of hip dislocation on the spine, we assessed symmetry while the patient was lying down, sitting, and standing. METHODS: The results of surgical reconstruction of hip joints in 54 patients with severe cerebral palsy who had 66 severely subluxated or dislocated hip joints were retrospectively analyzed. The study was based on a research questionnaire with a mean follow-up of 4 years 10 months. The purpose of the questionnaire was to evaluate the pain, functional abilities, symmetry, and independence of the patients by using the modified Barthel index. RESULTS: Pain was fully relieved in 62 and was eased in 35% of preoperatively painful hips. The functional abilities of sitting and standing were not influenced statistically significantly by the operation. An improvement in sitting symmetry was observed in 40% and improvement in lying symmetry was observed in 32% of the patients. The mean center-edge angle improved from -24.9 degrees +/- 39 degrees (mean +/- standard deviation) to 25.9 degrees +/- 14 degrees . The acetabular index improved from 30.2 degrees +/- 8 degrees to 17.1 degrees +/- 7 degrees . CONCLUSIONS: The independence of the patients, based on the modified Barthel index, did not change significantly after surgery. Most improvements in quality of life were observed in those patients who had pain in the hip before the operation as a result of reduction of pain and improved mobility of the hip.

INTRODUCTION: In patients with cerebral palsy, the prevention of hip dislocation should be the “primum momens” of early surgery. The surgery consists of a myotomy of the adductor medium, mayor and gracilis, and, in non-ambulatory cases, a neurectomy of the second branch of the obturator nerve. The purpose of this study was to examine whether the adductor myotomy should be performed on the contralateral side at one sitting. MATERIALS AND METHODS: In a study performed by our team on 1,474 patients, 792 patients (53.7%) had a unilateral procedure and 682 a bilateral procedure. RESULTS: The non-myotomized spastic adductors alter the movement of forces on the femoral head and, thus, 78.20% required a secondary contralateral procedure. The dislocation of the hip in patients whose operations were performed at two different sittings occurred in 20% of cases, and in those with a bilateral procedure at one sitting only in 1%. The increase in the cervicodiaphyseal angle occurred in 23.1% of patients with surgeries performed at two sittings and only in 10% of patients with bilateral procedures at one sitting. CONCLUSIONS: Our results suggest that the bilateral surgical procedure at one sitting should be recommended to equalize the movements of force in spastic hips.

PURPOSE: A pilot study with short-term outcomes of a combined surgical and medical intervention for management of generalized lower limb spasticity, hip displacement and contractures of adductors in children with bilateral spastic cerebral palsy. METHODS: A prospective cohort study of 16 children (9 boys and 7 girls) aged 2-6 years with bilateral spastic cerebral palsy was performed. At entry, 5 were classified as level III and 11 as level IV, according to the Gross Motor Function Classification System (GMFCS). The intervention consisted of surgical lengthening of adductor longus and gracilis combined with the phenolization of the anterior branch of the obturator nerve, using 1 ml of 6% phenol, applied under direct vision at the time of lengthening of adductor longus. The hamstring and calf muscles were each injected with Botulinum neurotoxin A at a dose of 4 U/kg/muscle. Serial clinical (hip, knee, ankle joint range of motion), radiographic (migration percentage) and functional data-taken from a functional mobility scale (FMS) or GMFCS-were collected at 3, 6, 12 and 24 months post-operatively. RESULTS: There was a significant increase in hip abduction, knee extension (popliteal angle) and ankle dorsiflexion, maintained for 24 months; mean hip migration percentage decreased from 29 to 21% (P < 0.001). Using a validated mobility scale, significant improvements were noted in gross motor function. There were no complications related to the intervention. CONCLUSIONS: The combined surgical-medical intervention resulted in a reduction of spastic hip subluxation and improvements in gross motor function, as determined by the FMS. The combined intervention is, thus, useful as a temporizing measure, before definitive decisions are made considering such interventions as dorsal rhizotomy, intrathecal baclofen and single-event, multilevel surgery.