Child's Nervous System最新文献

Background: Rhabdomyosarcoma (RMS) is the most prevalent soft tissue sarcoma in children, with approximately 30% of head and neck RMS occurring in the orbit. The management of orbital RMS is complex, requiring a multidisciplinary approach and careful surgical planning. The objective of the present paper is to provide the neurosurgeon with an update on this challenging tumor.

Case description: A 12-year-old boy was admitted to our department after the onset of diplopia in his right eye, associated with quickly worsening ipsilateral eyelid edema and mild proptosis. MRI revealed an anterior superomedial, extraconal, and intraorbital expansive lesion. Complete tumor removal was performed through a trans-orbital approach. Histological diagnosis of embryonal RMS was done. The patient underwent radio-chemotherapy in accordance with the EpSSG RMS2005 subgroup C protocol. The patient is alive and disease-free.

Discussion and conclusion: The intricacies of treating orbital tumors necessitate meticulous planning to safeguard vital structures while guaranteeing comprehensive oncological treatment. A multidisciplinary approach with a specific protocol depending on the location and characteristics of the tumor is required. The available treatment options include surgical intervention or combination therapies, such as chemotherapy and radiotherapy. The successful management of these diseases depends on the careful coordination and application of the available techniques.

Purpose: Sleep apnea, posing significant health risks, is frequently associated with Chiari malformation (CM), characterized by cerebellar tonsil herniation through the foramen magnum. Central sleep apnea (CSA) in CM results from impaired brain-to-muscle signaling and requires treatment. Conversely, obstructive sleep apnea (OSA), arising from throat muscle relaxation, typically unrelated to CM, often coexists. This study evaluates the effectiveness of posterior fossa decompression (PFD) on sleep apnea.

Methods: A retrospective chart review was conducted of pediatric patients with CM-1 and sleep apnea who underwent PFD between April 1, 2004, and September 30, 2022. Data collected included demographics, clinical characteristics, adenotonsillectomy status, PFD details, and sleep study parameters like the apnea-hypopnea index and respiratory disturbance index. Statistical analysis assessed the surgery's impact on sleep apnea severity.

Results: The study included eleven patients, predominantly male (63.6%). All had OSA (100%), with 63.6% also having CSA. Preoperative sleep studies classified OSA severity as 36.4% mild, 18.2% moderate, and 45.5% severe, with no change post-surgery. CSA severity initially included seven mild cases, which became three mild, one moderate, and three resolved cases post-surgery. Among seven patients who had adenotonsillectomy before decompression, five showed no improvement in OSA severity post-surgery.

Conclusion: This study elucidates the complex relationship between CM-1, sleep apnea, and PFD. The findings show the persistence of sleep apnea in some patients and highlight the need for continuous monitoring of these patients in order to optimize their care after surgery.

Objective: The objective of this study was to evaluate the prevalence of tethered cord among patients in the postoperative period of open and occult spina bifida. To identify warning signs for its early diagnosis, as well as outcomes after the new surgical approach.

Methods: Retrospective study of patients followed at the Pediatric Neurosurgery Department of the Federal University of São Paulo with spinal dysraphism. Signs and symptoms indicating reoperation were collected, and postoperative results were classified as improved, unchanged, or worsened.

Results: 222 medical records of patients diagnosed with spinal dysraphism were evaluated. Symptomatic Tethered Cord Syndrome (STCS) was identified in 30 patients (13.51%), with clinical manifestations related to orthopedic deformities (66.7%), neurological deficits (56.7%), urological dysfunction (50%), and intestinal dysfunction (40%). 20 cases underwent surgery for tethered cord release. The mean age at the time of surgery was 7.7 ± 4.9 years, with 13 female patients (65%). In the postoperative evaluation, improvement in low back pain (90.9%), urological pattern, and urinary tract infection episodes (45.4%) were particularly noteworthy. 3 patients (33.3%) with constipation showed improvement, and one worsened (11.1%). Improvement in ambulation was seen in two cases (16.7%). Low back pain was the first symptom to improve after surgery, with an average time of 1.3 months, followed by changes in the urological pattern at 15.6 months. Improvement in constipation was observed in the first month in 2 cases (66.7%), positive changes in ambulation were observed around 7 months after surgery, and only one case showed improvement in clubfoot correction.

Conclusions: The prevalence of tethered cord recurrence after primary correction surgery for open or occult neural tube closure defects was similar to that found in the literature. The results were encouraging, with good postoperative evolution of patients, especially in the improvement of low back pain and urological symptoms.

Ventriculoperitoneal (VP) shunt placement, essential for managing hydrocephalus, often risks catheter malpositioning, especially in patients with small ventricles. We present a novel technique combining neuronavigation with intraoperative cone-beam computed tomography using the BrainLab system and Loop-X mobile imaging unit. This approach enables real-time verification of catheter placement by integrating preoperative MRI data with intraoperative CT imaging. In a 12-year-old boy with therapy-refractory idiopathic intracranial hypertension, neuronavigation was guided by the BrainLab Skull Fix and Cushing canula, ensuring precise catheter insertion into the right frontal horn. Post-placement, Loop-X facilitated immediate verification of the catheter's trajectory and positioning, corroborated by postoperative MRI. This technique demonstrated high precision and minimized radiation exposure, emphasizing its utility in reducing revision rates due to suboptimal catheter placement.

Purpose: Peripheral nerve sheath tumors (PNSTs) are rare in pediatric patients, especially in the brachial plexus. Research on PNSTs is lacking. This article presents a retrospective cohort study of pediatric patients diagnosed and treated with PNSTs, specifically brachial plexus tumors.

Methods: All pediatric patients intervened in a single center between 2007 and 2023 with brachial plexus tumors were systemically analyzed.

Results: Eleven pediatric patients with 14 brachial plexus PNSTs were studied. The gender distribution was 64% female and 36% male, with an average age of 10.7 years. Ninety-one percent had a previous NF-1 diagnosis. Right brachial plexus presented a higher prevalence (64%). Pain, Tinel's sign, and stiffness masses were common during diagnosis. Motor deficits were noted in 43% of the patients. Surgery was indicated for symptoms, particularly pain and rapid growth, increasing malignancy risk. Due to suspected malignancy, an en bloc resection with safety margins was performed. Among the patients, 57% received a histopathological diagnosis of MPNST (malignant peripheral nerve sheath tumor). Treatment included radiotherapy and chemotherapy. Clinical follow-up was conducted for all cases, involving clinical and oncological evaluations for all MPNSTs.

Conclusions: This article present a series of pediatric brachial plexus tumors, especially in NF-1, and emphasizes the importance of thorough evaluation for this group. Swift diagnosis is crucial in pediatrics, enabling successful surgery for small lesions with limited neurological symptoms, improving long-term outcomes. Prompt referral to specialized services is urged for suspected masses, irrespective of neurological symptoms. Benign tumor postsurgical progression shows better outcomes than MPNSTs, with complete resection as the primary goal. Needle-guided biopsy is not recommended.

Motor-evoked potential (MEP) monitoring by transcranial electrical stimulation (TES) is important for intraoperative motor function assessment in neurosurgery; however, false-negative results sometimes occur, and these findings should be interpreted with caution. Herein, we report an interesting MEP change resulting from a pons transection. The patient was a boy aged 5 years and 2 months. He underwent multiple craniotomies for cerebellar anaplastic ependymoma and was already paralyzed in the right upper and lower limbs. Therefore, we decided to remove the recurrent lesion from the left anterior pons. MEPs were recorded on both the right and left sides after the start of surgery but disappeared 1 h 30 min after the start of surgery in the TES on the operative side, even when the stimulation intensity was increased. The contralateral TES consistently recorded stable MEPs throughout the surgery. The tumor was completely resected on imaging. Immediately postoperatively, the patient experienced flaccid paralysis on the right side of the body, which recovered to preoperative levels over time. A transcranial MEP cannot be derived if the corticospinal tract is transected at the pons. Transcranial MEP findings may accurately reflect the corticospinal tract function if the injury is caudal to the pons.

Introduction: In addition to seizure care, it has been well-established that a holistic approach to the treatment of children with epilepsy that addresses the social, behavioral, and psychological dimensions also benefits their quality of life (QoL). The aim of this study was to investigate the patient and parental perceived stigma, seizure self-efficacy of children with epilepsy, and the relationship with quality of life in terms of sociodemographic and epilepsy-related factors.

Method: The study group consisted of children, aged between 8 and 18 years and with a diagnosis of epilepsy of at least six months duration and their parents. Pediatric Quality of Life Inventory (PedsQL), Child-Parent Stigma Scales, and the Seizure Self-Efficacy Scale for Children (SSES-C) were used for evaluation.

Results: One hundred and forty-four children (mean age 12.6 ± 2.9 years) took part, of whom 48.6% were female. The mean Child Stigma Scale score was 1.77 ± 0.82, Parent Stigma Scale score 2.41 ± 0.75, SSES-C score was 3.37 ± 0.98, and PedsQL score was 72.8 ± 18.6 for children self-reports (CsR) and 73.2 ± 18.8 for parent proxy-reports. The intraclass correlation coefficient for parent-child PedsQL indicated a good level of agreement. There was a significant negative correlation between the Child Stigma Scale and the PedsQL-CsR scores. A significant positive correlation was identified between the SSES-C scores and the PedsQL-CsR scores.

Discussion: Perceptions of stigma in children with epilepsy and their parents were high in this study population. Of note, the elevated stigma perception reported by the patients had a detrimental impact on seizure self-efficacy. This relationship may affect the children's QoL and further complicates epilepsy management in this patient group.

Purpose: Spontaneous regression of Chiari malformation type 2 (CM2) is observed rarely, as CM2 is associated with meningomyelocele (MMC) that is surgically repaired either pre- or early postnatally. While the radiological regression of CM2 occurs frequently following prenatal repair of MMC, it has been reported in only a few studies after postnatal repair.

Methods: From the consecutive series of children with postnatally repaired MMC, we reviewed the clinical and radiological data regarding CM2, particularly its regression either spontaneously or following CSF diversion.

Results: Eighteen children underwent postnatal repair of MMC between February 2011 and April 2023. CM2 was present in 16 (89%), and hydrocephalus in 15 children (83%), requiring shunting in 14 of them. During the mean clinical observation time (from birth to April 2023) of 59 ± 51 months, three children with CM2 (19%) underwent 1-2 foramen magnum decompressions (FMD), five children (28%) 1-4 surgical untethering procedures and 13 children with shunted hydrocephalus (93%) 1-5 shunt revisions. Out of sixteen children with CM2, we observed regression of CM2 on MRI in only one case (6%) during the mean radiological follow-up (from birth to the last MRI taken) of 49 ± 51 months.

Conclusion: In our experience, spontaneous regression of CM2 in children with postnatally repaired MMC occurs quite rarely. Pathophysiological mechanisms behind the development of CM2 in children with MMC remain unclear, but our observation supports the hypothesis of an association between the downward displacement of the hindbrain and the low intraspinal pressure secondary to CSF leakage in children born with MMC.