Child's Nervous System最新文献

Purpose: This retrospective systematic literature review aimed to summarize available data regarding epidemiology, etiology, presentation, investigations, differentials, treatment, prevention, monitoring, complications, and prognosis for radiation-induced cavernous malformations (RICMs) in pediatric patients.

Methodology: Review conducted per PRISMA guidelines. Google Scholar, PubMed, Trip Medical Database, and Cochrane Library searched utilizing a keyphrase, articles filtered per inclusion/exclusion criteria, duplicates excluded. Based on criteria, 25 articles identified, 7 further excluded from the systematic data but included in discussion (5 × insufficient data, 2 × other systematic reviews).

Results: Many studies did not contain all explored data. 2487 patients reviewed, 325 later found to have RICM (143 male, 92 female). Mean age at irradiation 7.6 years (range 1.5-19). Mean total radiation dose 56 Gy (12-112). Most common indications for radiation-medulloblastoma 133x, astrocytoma 23x, ependymoma 21x, germinoma 19x. Mean age at RICM diagnosis 18 years (3.6-57). Mean latency to RICM 9.9 years (0.25-41). Most common anatomic locations-temporal 36, frontal 36, parietal 13, basal ganglia 16, infratentorial 20. Clinical presentation-incidental 270, seizures 19, headache 11, focal neurological deficit 7, other 13. 264 patients observed, 34 undergone surgery. RICM bled in 28 patients. Mean follow-up 11.7 years (0.5-50.3). Prognostic reporting highly variable.

Conclusions: From our data, pediatric RICMs appear to display slight male predominance, present about 10 years after initial irradiation in late teen years, and present incidentally in majority of cases. They are mostly operated on when they bleed, with incidental lesions mostly being observed over time. Further prospective detailed studies needed to draw stronger conclusions.

Background: Insular epilepsy is a well-known cause of drug-resistant epilepsy (DRE) in the pediatric population. It can be a source of surgical epilepsy treatment failures when not ruled out pre-operatively. Non-invasive methods often provide limited information about its existence, being the invasive methods necessary to diagnose it in the vast majority of cases. The most used is stereoelectroencephalography (SEEG). We report a series of DRE pediatric patients in which insular SEEG was performed to rule out insular epilepsy.

Method: We performed a retrospective review of pediatric DRE patients operated on SEEG including insular electrodes between April 2016 and September 2022. We described the different trajectories used (orthogonal or oblique) and surgical techniques. After implantation, we assessed electrodes' precision using three measures: entry point location error (EPLE), target point location error (TPLE), and target deviation (TD). We also reported complications that occurred with this technique as well as the diagnostic information provided.

Results: Overall, 32 DRE patients were operated on SEEG including insular electrodes. Four hundred one electrodes were implanted, 148 (39.91%) of whom were directed to the insula. One hundred twelve followed an orthogonal trajectory, and 36 were oblique. The mean EPLE was 1.45 mm, TPLE was 1.88 mm and TD was 0.71 mm. Three patients suffered from frontal hematoma, two of them diagnosed on post-operative MRI and one who required surgery, with no sequelae. One patient suffered from meningitis treated with antibiotics with no permanent sequelae. Nine patients (28.13%) had the insula included in the epileptogenic zone.

Conclusions: Insular epilepsy has to be ruled out in DRE patients when little suspicion is obtained after non-invasive testing. This is especially important in the pediatric population, in which seizure semiology is more difficult to characterize and failures to control epilepsy have devastating consequences in neurocognitive development and scholarship. Given its relative low rate of relevant complications and potential benefits, we should consider widening the inclusion criteria for insular SEEG monitoring.

Background: Myelomeningocele (MMC) is a severe form of spina bifida characterized by spinal cord extrusion into a cerebrospinal fluid (CSF) filled sac which may lead to lifelong disability. Repair of these lesions have classically occurred shortly after birth, but more recently, prenatal myelomeningocele repair techniques have been elucidated. This study aimed to investigate the outcome of surgery, particularly with subsequent spinal cord tethering, in patients with prenatal myelomeningocele closure and those with postnatal repair surgery.

Materials and methods: In this retrospective study which assessed patients from April 2002 to April 2020, the data of 18 total patients with MMC were reviewed. Nine patients from each group were included, closed prenatally or postnatally, respectively. Demographic information including age and sex, birth week, infant comorbidities, presence of dermoid or lipoma, cutaneous stigmata, total number and timing of detethering procedures, presence of Chiari malformation, and need for VPS was obtained.

Results: Eighteen patients including nine infants closed prenatally and nine infants with myelomeningoceles closed postnatally were reviewed. The mean age was 4 ± 3 years and 6.22 ± 2.4 in prenatal and postnatal retrospectively. There was a significant relationship between the number of detethering procedures (p-value = 0.03) and the need for a ventriculoperitoneal shunt (VPS) (p-value = 0.01) between the groups, with the prenatal closure group having lower rates of each. There was no significant difference between the groups in regard to the mean age at the detethering procedure (p = 0.4), sex (p = 0.09), birth week (p = 0.8), comorbidities (p = 0.8), presence of intraspinal dermoid or lipoma (p = 0.09), presence of cutaneous stigma (p = 0.08), Chiari (p = 0.6), fatty filum (p = 0.08), syrinx (p = 0.4), bone anomaly (p = 0.4), and spina bifida neurological scale (p = 0.66).

Conclusion: There was a significant relationship between the two groups in terms of the number of detethering procedures, and the need for VPS. Our data represents a possible difference in the need for detethering surgeries and ventriculoperitoneal shunt placements between patients with prenatal and postnatal myelomeningocele closures.

Background: Cerebral aneurysms in children have a low incidence and accounts for less than 4% of all cerebral aneurysms. These aneurysms have been linked to various factors. Severe headache, seizures, and motor-sensory deficits are common presentations.

Case report: We describe the case of a 2-month-old male patient who presented with generalized tonic-clonic seizures for 4 days. At the hospital, he was stabilized with ventilatory support, sedation, and antiepileptic drugs. A NCCT (Head) showed intraparenchymal hemorrhage in the left fronto-parieto-temporal lobe and subarachnoid hemorrhage. Subsequently a CT angiogram revealed an aneurysm of the left M3 segment of MCA. Successfully, the patient underwent microsurgical clipping of aneurysm and evacuation of hematoma.

Conclusions: Pediatric cerebral aneurysms differ from their adult counterparts, mainly in their etiology and evolution. As per literature, aneurysmal clipping and neurological endovascular therapy have shown similar results.

Introduction: Giant extradural thoracic schwannomas are very rare tumors in the pediatric age group and often occur together with neurofibromatosis. Giant schwannomas span across more than two vertebral segments and have an extraspinal extension of over 2.5 cm. In this case, we report on a 5-year-old boy with a purely extradural giant schwannoma without accompanying neurofibromatosis.

Clinical presentation: A 5-year-old male patient was admitted to the orthopedics and traumatology outpatient clinic with complaints of difficulty in walking following waist and left leg pain after falling from a chair. Contrast-enhanced spinal MRI and cranial MRI showed an extradural spinal lesion measuring 22 × 18 × 35 mm that pushed the spinal cord to the right at the T10-12 level and extended into the left foramen at the T11-12 level. The patient was operated. The tumor was removed completely by performing bilateral laminoplasty at the T10-11-12 levels. Histopathology result reported schwannoma.

Conclusion: Giant schwannomas are slow-growing tumors that rarely occur in childhood. In these patients, spinal traumas can lead to serious neurological deficits. Early diagnosis and successful surgery can prevent permanent neurological damage.

Purpose: Pediatric brain tumor survivors can experience detrimental effects from radiation treatment. This cross-sectional, large cohort study examined late psychosocial and executive functioning effects in pediatric patients treated ≥ 3 years after proton radiation therapy (PRT).

Methods: Parents of 101 pediatric brain tumor survivors completed the Behavior Assessment System for Children and the Behavior Rating Inventory of Executive Function. Standard scores were compared to published normative means, rates of impairment (T-score > 65) were calculated, and demographic and clinical characteristics were examined.

Results: Mean age at PRT was 8.12 years and mean interval from PRT to assessment was 6.05 years. Half were female (49.5%), 45.5% received craniospinal irradiation (CSI), and 58.4% were diagnosed with infratentorial tumors. All mean T-scores were within normal range. Mean T-scores were significantly elevated compared to the norm on the withdrawal, initiate, working memory, and plan/organize scales. Rates of impairment were notably high in working memory (24.8%), initiate (20.4%), withdrawal (18.1%), and plan/organize (17.0%). Greater withdrawal was significantly associated with CSI and also with chemotherapy and diagnosis of hearing loss. Mean T-scores were significantly lower than the norm on the hyperactivity, aggression, conduct problems, and inhibition scales. No significant problems were identified with social skills or depression. Interval since treatment was not correlated with any scale.

Conclusion: Although psychosocial and executive functioning was within the normal range, on average, social withdrawal and metacognitive executive functioning (working memory, initiating, planning/organizing) were areas of concern. Targeted yearly screening and proactive executive skill and social interventions are needed for this population.

We have designed a novel device that facilitates the accurate placement of occipital ventricular catheters in ventriculoperitoneal shunt procedures. After 7 years of clinical use, this device has consistently demonstrated its simplicity, user-friendliness, and effectiveness. It enables both experienced surgeons and novices to confidently and accurately position the ventricular catheter to a satisfactory location.

Background: Raising a child with hydrocephalus can be very challenging, especially in low- and middle-income countries. In Pakistan, mothers being the primary caregivers for their hydrocephalic children are under tremendous stress.

Methods: This study explores the challenges faced by Pakistani mothers raising children with hydrocephalus, employing a qualitative methodology through focus group discussions comprising ten mothers of hydrocephalic babies at Tertiary Care Hospital in Pakistan.

Results: The findings highlight three main themes: emotional toll, social isolation, and financial strain. Mothers experience significant emotional stress due to societal stigma and a lack of support, particularly from their husbands and family. Social isolation is prevalent, as mothers fear sharing their burdens and face physical confinement due to their children's needs. Financial strain is another major issue, with high medical costs adding to their economic difficulties.

Conclusion: The study emphasizes improved access to specialized care, awareness campaigns to reduce stigma, financial assistance, and stronger community support networks to support these mothers better. Addressing these unmet needs is crucial for empowering Pakistani mothers in their caregiving roles and improving the quality of life for their children with hydrocephalus.

Purpose: The spheno-occipital synchondrosis (SOS) is an important site of endochondral ossification in the cranial base that closes prematurely in Apert, Crouzon, and Pfeiffer syndromes, which contributes to varying degrees of midface hypoplasia. The facial dysmorphology of Muenke syndrome, in contrast, is less severe with low rates of midface hypoplasia. We thus evaluated the timing of SOS fusion and cephalometric landmarks in patients with Muenke syndrome compared to normal controls.

Methods: Patients with Muenke syndrome who had at least one fine-cut head computed tomography scan performed from 2000 to 2020 were retrospectively reviewed. A case-control study was performed of patient scans and age- and sex-matched control scans. SOS fusion status was evaluated as open, partially closed, or closed.

Results: We included 28 patients and compared 77 patient scans with 77 control scans. Kaplan-Meier analysis demonstrated an insignificantly earlier timeline of SOS fusion in Muenke syndrome (p = 0.300). Mean sella-orbitale (SO) distance was shorter (44.0 ± 6.6 vs. 47.7 ± 6.7 mm, p < 0.001) and mean sella-nasion-Frankfort horizontal (SN-FH) angle was greater (12.1° ± 3.8° vs. 10.1° ± 3.2°, p < 0.001) in the Muenke group, whereas mean sella-nasion-A point (SNA) angle was similar and normal (81.1° ± 5.7° vs. 81.4° ± 4.7°, p = 0.762).

Conclusion: Muenke syndrome is characterized by mild and often absent midfacial hypoplasia, with the exception of slight retropositioning of the infraorbital rim. Interestingly, SOS fusion patterns in these patients are not significantly different from age- and sex-matched controls despite an increased odds of fusion. It is possible that differences in timing of SOS fusion may manifest phenotypically at the infraorbital rim rather than at the maxilla.