Child's Nervous System最新文献

Introduction: Diffuse intrinsic pontine gliomas are associated with dismal survival outcomes. Conventional fractionation radiation to a dose of 60 Gy is the standard of treatment. This retrospective review aims to compare survival and toxicity outcomes of patients treated with conventional fractionation (CF) and hypofractionation (HF) radiotherapy.

Materials and methods: Treatment-naïve diffuse intrinsic pontine glioma patients undergoing radical radiation were analyzed. CF was delivered to a dose of 50-60 Gy in 25-30 fractions, while HF was delivered as 38-40 Gy in 12-15 fractions. All patients were planned via the volumetric modulated arc therapy (VMAT) technique.

Results: A total of 64 patients were eligible for analysis. The median age of presentation was 10 years. Motor deficit was the most common presenting complaint in 51.6% of the patients, with a median symptom duration of 2 months. The pons was the most frequent site of disease epicenter in 71.8% of the patients. After a median follow-up of 9.45 months (range 0.23-72.63 months), 23 patients died, and 28 patients experienced disease progression. The unadjusted hazard ratio (HR) for death in patients treated with HF as compared to CF was 1.330 (95% CI 0.522-3.386) (p-value 0.550, by Cox regression analysis). The median OS for the entire cohort was 13.9 months, while it was 9.7 months (95% CI 5.65-13.74) and 15.1 months (95% CI 9.02-21.18) (p-value = 0.547) with CF and HF, respectively. On multivariate analysis, disease epicenter in the pons was the only significant factor associated with PFS. Hypofractionation was associated with a significantly higher aspiration rate and Ryle's tube requirement (p-value 0.027).

Conclusion: Hypofractionated radiation can be considered for diffuse intrinsic pontine glioma with optimum supportive care.

Purpose: In this study, we aimed to define the scope of paediatric epilepsy surgery performed in Asia and Australasia.

Methods: Representative surgeons from each county were emailed a survey about paediatric epilepsy surgery as it is practiced in their country. The survey covered topics including what investigations, operations and number of surgeries are performed and by whom (adult or paediatric neurosurgeons).

Results: Epilepsy surgery programmes are for the most part more common in larger countries and countries with a higher gross domestic product (GDP). Countries performing epilepsy surgery commonly have access to the full range of investigations except magneto-encephalography (MEG). Intraoperative electrocorticography (ECOG) and stereotaxy are typically available and most have access to extra-operative monitoring. There is limited availability of laser interstitial therapy (LIT) and responsive neuro-stimulation (RNS). China performs approximately 4000 paediatric epilepsy cases per year which is more than all of the other countries combined. Epilepsy surgery case numbers range from 0.2 to 22/ million population per year, and there is a correlation between the number of cases performed and the countries' GDP. A large number of children have no access to epilepsy surgery.

Conclusion: There is a wide variation in access to epilepsy surgery in Asia and Australasia. Some counties have surgical numbers comparable with high volume Western countries, while hundreds of millions of children have no access to surgery at all.

Purpose: This study aimed to summarize the existing English-language literature on central nervous system (CNS) meningeal melanocytomas in children, and additionally describe our institutional case report.

Methods: PubMed database was screened on September 2, 2024, for English-language papers reporting on pediatric patients with CNS meningeal melanocytoma.

Results: A total of 17 papers reporting on 18 patients with 19 CNS meningeal melanocytomas were found in the literature. Additionally, we reported on a 15-year-old male patient with C2-C6 meningeal melanocytoma. Pediatric cohort analysis showed nearly equal sex distribution and a mean age at diagnosis of 11.9 years. There were fifteen intracranial (75%) and five spinal tumors (25%). Four lesions (20%) were diagnosed as intermediate-grade melanocytomas, while the remaining sixteen (80%) were benign meningeal melanocytomas. Most tumors were hyperintense on T1-weighted imaging (85%) and hypointense on T2-weighted imaging (73%). All tumors showed positivity for S100 and Melan-A. Most tumors were characterized by a lack of CNS invasion (91%). Gross-total resection (GTR) was performed in 61% of tumors. Adjuvant radiotherapy (RT) was applied in 50% of patients with incomplete tumor resection. Postoperatively, 62% of patients achieved a favorable outcome. We found 1, 2, 3, and 4-year overall survival of 80%, 71%, 71%, and 50%, respectively. The recurrence rate was 15% after a mean time of 10 months.

Conclusions: Meningeal melanocytomas constitute a rare subgroup of CNS tumors. Surgical tumor removal aiming at maximally safe GTR remains a standard approach, resulting in favorable postoperative outcomes. Considering high recurrence rate, long-term follow-up is needed.

Background: Subdural hematoma (SDH) typically occurs due to traumatic brain injury but can arise as a rare complication of procedures like endoscopic third ventriculostomy (ETV).

Case presentation: We report an unusual case in a 9-year-old male with previous resection of a fourth-ventricle ependymoma at 2 years of age. Seven years post-surgery, he presented with worsening hydrocephalus and underwent ETV. One month later, he developed severe headaches and motor difficulties. Imaging revealed a significant right SDH, necessitating urgent drainage. Postoperative recovery was uneventful, and follow-up imaging showed resolution of the hematoma.

Literature review and discussion: ETV is generally preferred for obstructive hydrocephalus due to lower complication rates compared to shunt procedures. However, cases of SDH post-ETV remain reported, albeit rarely. Potential mechanisms include altered cerebrospinal fluid dynamics and intraoperative vessel injury. This case aligns with literature findings and reinforces the importance of postoperative monitoring and prompt intervention in symptomatic cases to prevent complications.

Conclusion: Clinicians should consider SDH in pediatric patients with new symptoms post-ETV. Further research should focus on understanding the risk factors and mechanisms for SDH development.

A 5-month-old male patient presented with obstructive hydrocephalus caused by pineal cyst apoplexy. Ventriculoperitoneal shunt surgery was performed for hydrocephalus treatment. During follow-up, spontaneous shrinkage of the cyst was observed. At 24 months of follow-up, no complications were reported. Pediatric cases of pineal cyst apoplexy are rare, and little is known about its conservative management. This case report emphasizes the effectiveness of conservative treatment and close monitoring, aiming to contribute to the understanding of pineal cyst apoplexy management in the pediatric population.

Purpose: We sought to evaluate the incidence, natural history, and management of cystic spinal lesions following myelomeningocele/myeloschisis closure.

Methods: We performed a single-center retrospective review of all patients who underwent myelomeningocele/myeloschisis closure from 2013 to 2018 with follow-up to 5 years old.

Results: We analyzed 100 fetal repairs and 81 postnatal closures from 305 total surgeries. Patients within this cohort systematically underwent serial MRI scans of the lumbar spine and had clinical follow-up until at least 5 years of age. Ninety-three (51.2%) developed radiographic evidence of cystic lesions with 28 (30.1%) requiring surgical intervention. Presence of cysts was higher in fetal repair (67/100, 67%) compared with postnatal (26/81, 32.1%; p < 0.01). Of the 93 patients with radiographic cysts, 28 (30.1%) underwent surgical resection at a median age of 27.6 months old ([Q1, Q3], [13.0, 48.6 months]). Fetal repair patients had a higher rate (26/67, 38.8%) of cysts requiring surgical resection compared with postnatal closure (2/26, 7.7%, p value < 0.01). Pathology demonstrated 18 of resected cysts were dermoid, 8 were epidermoid, and 2 were fibrous tissue. Post-operatively, no patients experienced a worsened ambulation status. Bladder compliance showed a non-significant trend toward improvement.

Conclusions: Cystic lesions in myelomeningocele/myeloschisis patients are common findings that result in nerve root tethering. We propose regular screening in both symptomatic and asymptomatic patients to circumvent nerve injury. Most cystic lesions do not require surgical resection though fetal repair is associated with a higher incidence of operative cysts. However, these lesions can be safely surgically resected with maintenance of ambulation and urologic function.

Objective: Investigate the clinical features and treatment outcomes of neonatal cephalohematoma and ossified cephalohematoma.

Methods: A retrospective review was conducted on the clinical features of 281 children with neonatal cephalohematoma and ossified cephalohematoma managed over the previous 10 years.

Results: Of the neonatal cephalohematomas, 75 underwent puncture and aspiration, while 98 neonatal ossified cephalohematomas were treated with neurosurgery without cranioplasty. The majority (83%) required only a single puncture, with no cases of infection or ossification in this group. Pathological examination revealed that ossified cephalohematomas resulted from the progression of neonatal cephalohematomas and were characterized by subperiosteal osteogenesis.

Conclusion: Our findings suggest that a more aggressive therapeutic approach and early surgical intervention for cephalohematoma are warranted. Puncture and aspiration of neonatal cephalohematoma is a safe and effective procedure that can prevent ossification. Early neurosurgery for ossified cephalohematoma is recommended as it is technically less challenging and can minimize the risk of severe surgical trauma.

Cerebral cavernous malformations (CCMs) are angiographically occult vascular lesions that present with a variety of neurological symptoms, including seizures, features of raised intracranial pressure and focal neurological deficits. In extremely rare circumstances, CCMs have presented with concomitant brain abscess formation. To date, five cases have previously been reported, the majority of which have affected patients aged 16 years or older. We present a case of a 7-year-old child with recent chickenpox infection, who presented with headache and left hemiparesis and was diagnosed with a large, deep-seated right-sided CCM with concurrent infection caused by Streptococcus pyogenes, an organism which itself is an extremely rare cause of brain abscess and may be a complication of recent chickenpox infection. The patient underwent surgical aspiration of the infected collection, completed a prolonged course of antibiotic therapy and made a good clinical recovery. Based on our review of the literature, this case represents the first reported case of Strep. pyogenes as a cause of infected CCM and only the second in a patient aged under 16 years.