Pediatric Neurosurgery最新文献

Introduction: Slit ventricle syndrome (SVS) remains a challenging problem in the early-shunted paediatric population. Various surgical and non-surgical treatments have been devised for this condition. However, there is currently no gold standard for its optimal management. Among various treatment modalities, subtemporal decompression (STD) is often performed as a last resort. We present our experience of STD in paediatric patients with SVS in whom initial treatment with programmable valves and anti-syphon device were not successful.

Methods: This is a single-centre retrospective observational study and survival analysis. Patients who underwent STD for SVS were included. Pre- and post-operative imaging data and clinical outcomes were collected.

Results: There were 20 patients (12 M, 8 F) with a mean age of 9 years (SD: 4) at first STD. 90% (n = 18) of patients had multiple shunt revisions pre-STD. At first STD, 70% (n = 14) and 30% (n = 6) of patients had unilateral or bilateral STD, respectively. STD led to a reduction in the frequency of shunt revisions in 60% (n = 12) of patients. The median time required before further STD, shunt surgery, or cranial vault surgery was 14 months. The median time before a further STD was required (either revision or contralateral side) was 89 months. At a median follow-up of 66.5 months (range: 1-159), 65% (n = 13) of patients had improvement in symptoms.

Conclusions: A large proportion of patients with persistent SVS symptoms, refractory to multiple shunt revisions, benefitted from STD in combination with shunt optimization. It was also safe and well-tolerated. Therefore, in patients who have multiple failed shunts, STD may reduce the morbidity associated with further shunt revisions and can significantly improve symptomatology.

Introduction: Brain arteriovenous malformations (AVMs) are increasingly being treated with Onyx liquid embolic agent (Onyx, Medtronic, Inc.). The phenomenon of delayed Onyx migration is not well documented in the literature. Moreover, the clinical presentation associated with Onyx migration is not well understood.

Case presentation: A pediatric patient with a history of neonatal seizures was referred to our institution upon experiencing daily headaches with photophobia, phonophobia, and sleep disturbance. Cerebral angiography revealed an AVM of the medial left cerebellar hemisphere. Preoperative embolization with Onyx liquid embolic achieved 25% closure of the AVM nidus. Upon developing worsening headaches the following day, new perinidal parenchymal edema was revealed on MRI, and urgent angiography demonstrated delayed migration of Onyx into the venous drainage. The patient underwent emergency resection of the AVM due to the risk of hemorrhage resulting from venous outflow obstruction.

Conclusion: Our report and literature review demonstrate that while the delayed, unexpected migration of Onyx embolic material has been alluded to in a handful of papers, this phenomenon is not well documented. Future research is needed to understand the frequency of delayed Onyx migration from brain AVMs and the possible clinical presentations to look for. The sudden development of headaches and other signs of perilesional edema, in particular, should prompt repeat angiographic examination due to the possibility of delayed liquid embolic migration.

Introduction: Resection of brain lesions associated with refractory epilepsy to achieve seizure control is well accepted. However, concurrent behavioral effects of these lesions such as changes in mood, personality, and cognition and the effects of surgery on behavior have not been well characterized. We describe 5 such children with epileptogenic lesions and significant behavioral abnormalities which improved after surgery.

Case descriptions: Five children (ages 3-14 years) with major behavioral abnormalities and lesional epilepsy were identified and treated at our center. Behavioral problems included academic impairment, impulsivity, self-injurious behavior, and decreased social interaction with diagnoses of ADHD, oppositional defiant disorder, and autism. Pre-operative neuropsychiatric testing was performed in 4/5 patients and revealed low-average cognitive and intellectual abilities for their age, attentional difficulties, and poor memory. Lesions were located in the temporal (2 gangliogliomas, 1 JPA, 1 cavernoma) and parietal (1 DNET) lobes. Gross total resection was achieved in all cases. At mean 1-year follow-up, seizure freedom (Engel 1a in 3 patients, Engel 1c in 2 patients) and significant behavioral improvements (academic performance, attention, socialization, and aggression) were achieved in all. Two patients manifested violence pre-operatively; one had extreme behavior with violence toward teachers and peers despite low seizure burden. Since surgery, his behavior has normalized.

Conclusion: We identified 5 patients with severe behavioral disorders in the setting of lesional epilepsy, all of whom demonstrated improvement after surgery. The degree of behavioral abnormality was disproportionate to epilepsy severity, suggesting a more complicated mechanism by which lesional epilepsy impacts behavior. We propose a novel paradigm in which lesionectomy may offer behavioral benefit even when seizures are not refractory. Thus, behavioral improvement may be an important novel goal for neurosurgical resection in children with epileptic brain lesions.

Background: Diffuse intrinsic pontine gliomas (DIPGs) are high-grade gliomas (HGGs) that occur primarily in children, and represent a leading cause of death in pediatric patients with brain tumors with a median overall survival of only 8-11 months.

Summary: While these lesions were previously thought to behave similarly to adult HGG, emerging data have demonstrated that DIPG is a biologically distinct entity from adult HGG frequently driven by mutations in the histone genes H3.3 and H3.1 not found in adult glioma. While biopsy of DIPG was historically felt to confer unacceptable risk of morbidity and mortality, multiple studies have demonstrated that stereotactic biopsy of DIPG is safe, allowing not only for improved understanding of DIPG but also forming the basis for protocols for personalized medicine in DIPG. However, current options for personalized medicine in DIPG are limited by the lack of efficacious targeted therapies for the mutations commonly found in DIPG. Multiple treatment modalities including targeted therapies, immunotherapy, convection-enhanced delivery, and focused ultrasound are in various stages of investigation.

Key message: Increasing frequency of biopsy for DIPG has identified distinct driving mutations that may serve as therapeutic targets. Novel treatment modalities are under investigation.

Background: Central nervous system tumors are the most common solid tumors in childhood. Treatment paradigms for pediatric central nervous system malignancies depend on elements including tumor histology, age of patient, and stage of disease. Radiotherapy is an important modality of treatment for many pediatric central nervous system malignancies.

Summary: While radiation contributes to excellent overall survival rates for many patients, radiation also carries significant risks of long-term side effects including neurocognitive decline, hearing loss, growth impairment, neuroendocrine dysfunction, strokes, and secondary malignancies. In recent decades, clinical trials have demonstrated that with better imaging and staging along with more sophisticated radiation planning and treatment set-up verification, smaller treatment volumes can be utilized without decrement in survival. Furthermore, the development of intensity-modulated radiotherapy and proton-beam radiotherapy has greatly improved conformality of radiation.

Key messages: Recent changes in radiation treatment paradigms have decreased risks of short- and long-term toxicity for common histologies and in different age groups. Future studies will continue to develop novel radiation regimens to improve outcomes in aggressive central nervous system tumors, integrate molecular subtypes to tailor radiation treatment, and decrease radiation-associated toxicity for long-term survivors.

Introduction: Lipoblastoma and lipoblastomatosis are rare benign mesenchymal adipose tumors that originate from embryonic white adipocytes and occur most commonly in infancy and early childhood. Lipoblastomas occur in the extremities and trunk, including the retroperitoneum and peritoneal cavity. Therefore, infiltration into the spinal canal has rarely been reported.

Case presentation: A 4-year-old girl presented to our clinic because of difficulty sitting on the floor with her legs straight. She also complained of enuresis and constipation for the past 6 months with persistent headaches and back pain evoked by body anteflexion. A magnetic resonance imaging revealed a massive lesion of the psoas major muscle, retroperitoneal, and subcutaneous spaces, extending into the spinal epidural space between L2 and S1. The patient underwent surgery which resulted in gross total removal of the tumor from the spinal canal. The mass was yellowish, soft, lobulated, fatty, and easily removed from the surrounding structures. Pathology confirmed the diagnosis of lipoblastoma. The postoperative course was uneventful, and the patient was discharged without any signs of neurological deficit.

Conclusion: We herein discuss a rare case of lipoblastoma extending into the spinal canal, resulting in neurological symptoms. Although this tumor is benign with no potential for metastasis, it is prone to local recurrence. Therefore, close postoperative observation should be performed.

Introduction: Extraskeletal myxoid chondrosarcoma of the jugular foramen is a rare clinical entity, especially in the pediatric population. Thus, it can be confused with other pathologies.

Case presentation: We report an extremely rare case of a 14-year-old female patient with jugular foramen myxoid chondrosarcoma that was completely removed through microsurgical resection.

Conclusion: The primary purpose of the treatment is gross total resection of the chondrosarcomas. However, adjuvant methods such as radiotherapy should additionally be applied in patients who have high-grade diseases or cannot undergo gross total resection because of anatomic localization.

Introduction: Chronic pleural cerebrospinal fluid (CSF) effusion is a rare complication after ventriculoperitoneal (VP) shunt insertion and only 18 cases in children and adults have been described so far without catheter dislocation to the intrathoracic cavity.

Case presentation: We report on a 4-year-old girl with a complex history of underlying neurogenetic disorder, a hypoxic-ischemic encephalopathy after influenza A infection with septic shock and severe acute respiratory distress syndrome, followed by meningitis at the age of 10 months. In consequence, she developed a severe cerebral atrophy and post-meningitic hydrocephalus requiring placement of a VP shunt. At age 4, she was admitted with community-acquired mycoplasma pneumonia and developed increasing pleural effusions leading to severe respiratory distress and requiring continuous chest tube drainage (up to 1,000-1,400 mL/day) that could not be weaned. β trace protein, in CSF present at concentrations >6 mg/L, was found in the pleural fluid at low concentrations of 2.7 mg/L. An abdomino-thoracic CSF fistula was finally proven by single photon emission computerized tomography combined with low-dose computer tomography. After shunt externalization, the pleural effusion stopped and the chest tube was removed. CSF production rate remains high above 500 mL/24 h. An atrial CSF shunt could not be placed, since a hemodynamically relevant atrial septum defect with frail circulatory balance would not have tolerated the large CSF volumes. Therefore, she underwent a total bilateral endoscopic choroid plexus laser coagulation (CPC) within the lateral ventricles via bi-occipital burr holes. Postoperatively CSF production rate went close to 0 mL and after external ventricular drain removal no signs and symptoms of hydrocephalus developed during a follow-up of now 2.5 years.

Conclusion: In summary, pleural effusions in patients with VP shunt can rarely be caused by an abdomino-thoracic fistula, with non-elevated β-trace protein in the pleural fluid. The majority of reported cases in literature were treated by ventriculoatrial shunt. This is the 2nd reported case, which has been successfully treated by radical CPC alone including the temporal horn choroid plexus, making the child shunt independent.

Introduction: Craniosynostosis is a common pediatric presentation in which the premature fusion of one or more cranial sutures results in a misshapen skull. This birth defect is often associated with comorbidities due to structural impacts on nearby anatomical features. While there is some evidence for a male predominance among craniosynostosis patients, little has been investigated regarding sex differences in comorbidities of this condition. This study seeks to explore potential sexual dimorphisms in craniosynostosis patients at the time of presentation.

Methods: We conducted a retrospective, cross-sectional review of male and female non-syndromic craniosynostosis (NSC) patients between the ages of 1 month and 9 years that were evaluated at a 500-bed academic hospital or a 977-bed private hospital in Lubbock, TX, USA. Common comorbidities including ophthalmologic diagnoses, developmental delays, obstructive sleep apnea, chronic otitis media, hearing loss, chronic headaches, and seizure disorders were evaluated. The NSC cohort was compared to a similarly aged trauma group that represented the normal population.

Results: 175 NSC patients fit the inclusion criteria, of which 109 (62%) were male. A diagnosis of craniosynostosis was significantly associated with ophthalmological diagnoses (p < 0.0001), chronic otitis media (p < 0.0001), developmental delays (p < 0.0001), and hearing loss (p = 0.0047). Male NSC patients were less likely to present with ophthalmological diagnoses (p = 0.0010) or hearing loss (p = 0.0052) than females.

Conclusions: Our findings expand on current literature evaluating possible comorbidities of NSC, particularly supporting the association with ophthalmological diagnoses, chronic otitis media, developmental delays, and hearing loss. We also report sex differences in ophthalmological diagnoses and hearing loss for NSC patients. These findings can serve to educate physicians of symptoms requiring prompt recognition and management in these patients.