Nelci Zanon

Endoscopic surgery for the antenatal treatment of myelomeningocele: the CECAM trial

BACKGROUND A recent randomized clinical trial named Management of Myelomeningocele Study (MOMS trial) showed that prenatal correction of open spina bifida (OSB) via open fetal surgery was associated with improved infant neurological outcomes relative to postnatal repair, but at the expense of increased maternal morbidity. OBJECTIVE We sought to report the final results of our phase I trial (Cirurgia Endoscópica para Correção Antenatal da Meningomielocele [CECAM]) on the feasibility, safety, potential benefits, and side effects of the fetoscopic treatment of OSB using our unique surgical technique. STUDY DESIGN Ten consecutive pregnancies with lumbosacral OSB were enrolled in the study. Surgeries were performed percutaneously under general anesthesia with 3 ports and partial carbon dioxide insufflation. After appropriate surgical positioning of the fetus, the neuroplacode was released with scissors and the skin was undermined to place a biocellulose patch over the lesion. The skin was closed over the patch using a single running stitch. Preoperative, postoperative, and postnatal magnetic resonance imaging were performed to assess hindbrain herniation. Neurodevelopmental evaluation was performed before discharge and at 3, 6, and 12 months. All cases were delivered by cesarean delivery, at which time the uterus was assessed for evidence of thinning or dehiscence. RESULTS The median gestational age at the time of surgery was 27 weeks (range 25-28 weeks). Endoscopic repair was completed in 8 of 10 fetuses. Two cases were unsuccessful due to loss of uterine access. The mean gestational age at birth was 32.4 weeks with a mean latency of 5.6 weeks between surgery and delivery (range 2-8 weeks). There was 1 fetal and 1 neonatal demise, and 1 unsuccessful case underwent postnatal repair. Of the 7 infants available for analysis, complete reversal of hindbrain herniation occurred in 6 of 7 babies. Three babies required ventriculoperitoneal shunting or third ventriculostomy. Functional motor level was the same or better than the anatomical level in 6 of 7 cases. There was no significant maternal morbidity and no evidence of myometrial thinning or dehiscence. However, surgeries were complicated by premature rupture of membrane and prematurity. CONCLUSION Our study suggests that the antenatal treatment of OSB using a fetoscopic approach and our unique surgical technique can result in a watertight seal, reversal of the hindbrain herniation, and better than expected motor function. Our technique differs substantially from the classic repair of OSB used in prior open fetal surgery and fetoscopic studies, in which the dura mater is dissected and the defect is closed in multiple layers. Instead, we use a biocellulose patch placed over the lesion and simple closure of the skin. As such, our technique is an alternative to the current paradigms in the antenatal treatment of OSB. Our clinical outcomes are in line with the results of our extensive prior animal work. Maternal benefits of our approach and technique include minimal morbidity and no myometrial legacy. Current limitations of the approach include potential loss of access, premature rupture of membranes, and attendant prematurity. Phase II trials are needed to prevent these complications and to further assess the risks and benefits of our distinct surgical approach and technique.

Fetoscopic single-layer repair of open spina bifida using a cellulose patch: preliminary clinical experience.

Abstract Objective: To report our preliminary clinical experience in the antenatal correction of open spina bifida (OSB) using a fetoscopic approach and a simplified closure technique. Methods: Four fetuses with lumbar-sacral defects were operated in utero from 25 to 27 weeks. Surgeries were performed percutaneously under general anesthesia using three trocars and partial carbon dioxide insufflation. After dissection of the neural placode, the surrounding skin was closed over a cellulose patch using a single continuous stitch. Results: Surgical closure was successful in three of the four cases. All successful cases showed improvement of the hindbrain herniation and no neonatal neurosurgical repair was required in two cases. Delivery occurred between 31 and 33 weeks, and no fetal or neonatal deaths occurred. Ventriculoperitoneal shunting was not needed in two out of the 3 successful cases. Conclusions: Our preliminary experience suggests that definitive fetoscopic repair of OSB is feasible using our innovative surgical technique. A phase I trial for the fetoscopic correction of OSB with this technique is currently being conducted.

A comparison of treadmill training and overground walking in ambulant children with cerebral palsy: randomized controlled clinical trial

Objective: Compare the effects of treadmill training and training with overground walking (both without partial weight support) on motor skills in children with cerebral palsy. Design: Randomized controlled clinical trial. Setting: Physical therapy clinics. Subjects: Thirty-six children with cerebral palsy (levels I–III of the Gross Motor Functional Classification System) randomly divided into two intervention groups. Interventions: Experimental group (17 children) submitted to treadmill training without partial weight support. Overground walking group (18 children) submitted to gait training on a fixed surface (ground). Training was performed for seven consecutive weeks (two sessions per week), with four subsequent weeks of follow-up. Results: Both groups demonstrated improvements on the 6-minute walk test (experimental group from 227.4 SD 49.4 to 377.2 SD 93.0; overground walking group from 222.6 SD 42.6 to 268.0 SD 45.0), timed up-and-go test (experimental group from 14.3 SD 2.9 to 7.8 SD 2.2; overground walking group from 12.8 SD 2.2 to 10.5 SD 2.5), Pediatric Evaluation Disability Inventory (experimental group from 128.0 SD 19.9 to 139.0 SD 18.4; overground walking group from 120.8 SD 19.0 to 125.8 SD 12.2), Gross Motor Function Measure-88 (experimental group from 81.6 SD 8.7 to 93.0 SD 5.7; overground walking group from 77.3 SD 7.0 to 80.8 SD 7.2), Berg Balance Scale (experimental group from 34.9 SD 8.5 to 46.7 SD 7.6; overground walking group from 31.9 SD 7.0 to 35.7 SD 6.8) after treatment. The experimental group demonstrated greater improvements than the overground walking group both after treatment and during follow up (p < 0.05). Conclusion: Treadmill training proved more effective than training with overground walking regarding functional mobility, functional performance, gross motor function and functional balance in children with cerebral palsy.

Effect of a single session of transcranial direct-current stimulation on balance and spatiotemporal gait variables in children with cerebral palsy: A randomized sham-controlled study

Background: Transcranial direct-current stimulation (tDCS) has been widely studied with the aim of enhancing local synaptic efficacy and modulating the electrical activity of the cortex in patients with neurological disorders. Objective: The purpose of the present study was to determine the effect of a single session of tDCS regarding immediate changes in spatiotemporal gait and oscillations of the center of pressure (30 seconds) in children with cerebral palsy (CP). Method: A randomized controlled trial with a blinded evaluator was conducted involving 20 children with CP between six and ten years of age. Gait and balance were evaluated three times: Evaluation 1 (before the stimulation), Evaluation 2 (immediately after stimulation), and Evaluation 3 (20 minutes after the stimulation). The protocol consisted of a 20-minute session of tDCS applied to the primary motor cortex at an intensity of 1 mA. The participants were randomly allocated to two groups: experimental group - anodal stimulation of the primary motor cortex; and control group - placebo transcranial stimulation. Results: Significant reductions were found in the experimental group regarding oscillations during standing in the anteroposterior and mediolateral directions with eyes open and eyes closed in comparison with the control group (p<0.05). In the intra-group analysis, the experimental group exhibited significant improvements in gait velocity, cadence, and oscillation in the center of pressure during standing (p<0.05). No significant differences were found in the control group among the different evaluations. Conclusion: A single session of tDCS applied to the primary motor cortex promotes positive changes in static balance and gait velocity in children with cerebral palsy.

Aplasia cutis congenita: management of a large skull defect with acrania.

Aplasia cutis congenita is a rare disorder characterized by absence of skin. Lesions typically occur on the vertex and are sometimes small, but they can affect deep tissues such as the skull bone and dura. Mortality is related to the depth and size of the lesion and can amount to a rate of more than 50% when full thickness is involved. The treatment remains controversial -- both surgical and conservative managements are described. Minor lesions can be controlled with nonsurgical treatment, but large defects require early surgery. We report the case of a female newborn with acrania and scalp aplasia cutis congenita, which was treated with a bipedicle scalp flap based on the temporal vessels. Full- and partial-thickness skin grafts were used to cover the donor site on the temporo-occipital region. Postoperatively, the patient developed a liquorice cyst, which was treated with a shunt, and she has been followed up for evaluation of the bony defect closure and skull morphology. Her neuropsychomotor development is normal.

Transcranial Direct Current Stimulation Combined with Treadmill Gait Training in Delayed Neuro-psychomotor Development

[Purpose] The aim of the present study was to describe the results of transcranial direct current stimulation combined with treadmill training in a child with delayed neuro-psychomotor development. [Subject and Methods] Transcranial direct current stimulation (intensity: 1 mA) was applied over the primary motor cortex for 20 minutes during simultaneous treadmill training (2.5 km/h) in ten sessions. [Results] Clinically significant improvement was found in motor development (fine motor subscale, 23 to 25; gross motor subscale, 32 to 41). Reductions in mean oscillation of the center of pressure were found in the anteroposterior (239.2 to 146.5 mm) and mediolateral (177.4 to 149.2 mm) directions. Increases occurred in cadence (106 to 123 steps/minute), step length (0.16 to 0.23 m), step width (0.09 to 0.14 m) and gait velocity with support (0.3 to 0.7 m/s). [Conclusion] After treatment, the child was able to initiate the standing position for the first time and walk without support.

Correlation between Pediatric Balance Scale and Functional Test in Children with Cerebral Palsy

[Purpose] To investigate the correlation of functional balance with the functional performance of children with cerebral palsy. [Subjects and Methods] This was a cross-sectional study of children with cerebral palsy with mild to moderate impairment. The children were divided into 3 groups based on motor impairment. The evaluation consisted of the administration of the Pediatric Balance Scale (PBS) and the Pediatric Evaluation Disability Inventory. Correlations between the instruments were determined by calculating Pearson’s correlation coefficients. [Results] In Group 1, a strong positive correlation was found between the PBS and the mobility dimension of the Pediatric Evaluation Disability Inventory (r=0.82), and a moderate correlation was found between the PBS and self-care dimension of the Pediatric Evaluation Disability Inventory (r=0.51). In Group 2, moderate correlations were found between the PBS and both the self-care dimension (r=0.57) and mobility dimension (r=0.41) of the Pediatric Evaluation Disability Inventory. In Group 3, the PBS was weakly correlated with the self-care dimension (r=0.11) and moderately correlated with the mobility dimension (r=0.55). [Conclusion] The PBS proved to be a good auxiliary tool for the evaluation of functional performance with regard to mobility, but cannot be considered a predictor of function in children with cerebral palsy.

Cerebellar transcranial direct current stimulation in children with ataxic cerebral palsy: A sham-controlled, crossover, pilot study

ABSTRACT Objective: The aim of the present study was to analyze the use of anodal tDCS of the cerebellar region combined with treadmill training to improve balance and functional performance in children with ataxic cerebral palsy. Design: Single-blind, sham-controlled, crossover, pilot study. Setting: Rehabilitation center and research motion analysis laboratory. Participants: Children (N = 6) with ataxic cerebral palsy and balance deficit. Main Outcome Measures: Static balance (oscillations of the center of pressure), functional balance (Pediatric Balance Scale) and functional performance (Pediatric Evaluation of Disability Inventory) were evaluated. Results: Significant reductions occurred in oscillations of the center of pressure with eyes closed after active anodal tDCS only. The effects of treadmill training on functional balance and functional performance in mobility were maintained in the active tDCS group only. Conclusion: These preliminary data support the notion that anodal tDCS of the cerebellar region combined with treadmill training improves balance in children with ataxic cerebral palsy.

Does the choice of surgical approach to insert an intratumoral catheter influence the results of intratumoral cystic treatment

BACKGROUND In the literature, only a few articles are related to the surgical approach for insertion of an intratumoral catheter. No one has evaluated the complications related to the different surgical techniques for the treatment of pediatric cystic craniopharyngiomas. METHODS A cooperative, multicenter (France and Brazil) study was carried out and included 50 patients (aged between 9 months and 21 years) diagnosed as having cystic craniopharyngioma treated between 1990 and 2000. Forty-nine children were available for the final analysis. The patients were divided into 3 groups: group I, 24 children who underwent a craniotomy and catheter placement under direct vision using a surgical microscope; group II, 14 children who were submitted to a stereotactic approach for the placement of the intratumoral catheter; and group III, 11 children whose catheters were placed by a freehand approach through a burr hole. RESULTS Eight children (16.3%) presented complications related to the placement of the catheter, namely, misplacement or leakage. The rate of the complications did not appear to be related to one specific modality of the catheter insertion. CONCLUSION Intracystic antiblastic drug injection is one available option in the treatment of cystic craniopharyngiomas. Because of the toxic effect of these drugs on the brain, determination of the exact placement to avoid leakage is mandatory for correct treatment. However, the results of the present study appear to indicate an excessively high incidence of complications whichever technique is used.

Spared Primary Motor Cortex and The Presence of MEP in Cerebral Palsy Dictate the Responsiveness to tDCS during Gait Training

The current priority of investigations involving transcranial direct current stimulation (tDCS) and neurorehabilitation is to identify biomarkers associated with the positive results of the interventions such that respondent and non-respondent patients can be identified in the early phases of treatment. The aims were to determine whether: (1) present motor evoked potential (MEP); and (2) injuries involving the primary motor cortex, are associated with tDCS-enhancement in functional outcome following gait training in children with cerebral palsy (CP). We reviewed the data from our parallel, randomized, sham-controlled, double-blind studies. Fifty-six children with spastic CP received gait training (either treadmill training or virtual reality training) and tDCS (active or sham). Univariate and multivariate logistic regression analyses were employed to identify clinical, neurophysiologic and neuroanatomic predictors associated with the responsiveness to treatment with tDCS. MEP presence during the initial evaluation and the subcortical injury were associated with positive effects in the functional results. The logistic regression revealed that present MEP was a significant predictor for the six-minute walk test (6MWT; p = 0.003) and gait speed (p = 0.028), whereas the subcortical injury was a significant predictor of gait kinematics (p = 0.013) and gross motor function (p = 0.021). In this preliminary study involving children with CP, two important prediction factors of good responses to anodal tDCS combined with gait training were identified. Apparently, MEP (integrity of the corticospinal tract) and subcortical location of the brain injury exerted different influences on aspects related to gait, such as velocity and kinematics.