Child's Nervous System最新文献

Introduction: Intracranial hemorrhage in the newborn poses a significant challenge to their well-being and subsequent development. In pre-term neonates, germinal matrix hemorrhage is the most common cause of intracranial hemorrhage with the prevalence varying from 20 to 59%. However, in term neonates, the prevalence and type of intracranial hemorrhage have not been well elucidated. The type of hemorrhage occurring in term neonates differs from those occurring in pre-term neonates. We conducted a systematic review to assess the prevalence, type, and risk factors of intracranial hemorrhage occurring in term neonates.

Methods: We conducted a systematic review and meta-analysis by searching the PubMed and Embase databases. Inclusion criteria were studies reporting intracranial hemorrhage in term neonates (37 weeks or more gestational age at the time of birth). Exclusion criteria were as follows: (1) studies which examined only a specific type of hemorrhage, (2) studies which examined only a single risk factor, (3) sample size < 20, (4) not in English language, and (5) full text not accessible/available. The studies which met the eligibility criteria were assessed and evaluated by two authors, and data was extracted using a predesigned template. The risk of bias in the included studies was assessed using the Joanna Briggs Institute bias assessment tool. The meta-analysis for prevalence, type of hemorrhage, and risk factor was conducted using MetaXL extension for Microsoft Excel.

Results: A total of 1226 records were identified in a database search of PubMed and Embase. After screening, 20 studies were included in the final analysis. The overall prevalence of intracranial hemorrhage in term neonates was 9.3% (95% CI, 4.9-14.9%). The prevalence of intracranial hemorrhage in asymptomatic patients was 5.8% (95% CI, 2.5-10.2%) and in symptomatic patients was 29.3% (95% CI, 15.3-44.8%). Sub-group analysis of the modality of detection shows that the prevalence of hemorrhage detection was higher with computed tomography/magnetic resonance imaging (CT/MRI) than with ultrasonography (USG). Extra-axial hemorrhage was the most common type of hemorrhage detected, comprising about 32.4% of the overall. The odds of having an intracranial hemorrhage was significantly higher with an instrumental delivery (OR = 3.75, 95% CI, 1.4-10.02).

Conclusion: The present study shows that the prevalence of intracranial hemorrhage is relatively high in symptomatic and asymptomatic patients with extra-axial hemorrhage being the most common type of hemorrhage. The prevalence of hemorrhage varied according to the type of imaging used for detection and ideal imaging modality for evaluation, and the consequences of intracranial hemorrhage in term infants on development remain to be determined.

Split notochord syndrome (SNS) is a rare congenital anomaly characterized by incomplete midline notochordal integration during gastrulation, leading to segmental clefts of the spine. This case report describes a female patient with symptomatic cervico-dorsal SNS associated with a neurenteric cyst (NEC), intrathoracic gut duplication, and secondary hydrocephalus. Multistep surgical interventions were performed, including hydrocephalus management, excision of the cyst and gut duplication, and detethering with sectioning of the filum. Although there were no procedural complications, bladder and bowel dysfunction persisted on follow-up, while normal motor function was preserved. This case illustrates the diverse and complex clinical manifestations of SNS, underscoring the necessity of interdisciplinary collaboration and personalized patient care. While conservative management may be appropriate for asymptomatic cases, surgical intervention is typically recommended for symptomatic patients to address complications and improve outcomes. Given the lack of established guidelines, customized management based on clinical judgment is essential. Further research is needed to enhance understanding of SNS, improve the quality of care, and achieve better patient outcomes.

Central nervous system germ cell tumours are most commonly seen in the midline structures. Eccentric germinomas are rare but can be found in the basal ganglia, lobar, brainstem, and cranial vault. We present a 14-year-old boy who presented with signs and symptoms of overt diabetes insipidus and a radiological diagnosis of left frontal high-grade glioma. Confirmative histopathology revealed germinoma. The patient had a resolution of hypernatremia post-surgery and maintained normal sodium homeostasis on a single dose of 0.1 mg of tablet desmopressin. This paper showcases a rare case of an eccentric central nervous system germinoma in a young male patient presenting with Diabetes Insipidus without any apparent sellar or suprasellar involvement.

Purpose: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, rapidly growing pigmented neoplasm originating from the neural crest. Predominantly affecting infants, it commonly occurs in the head and neck region. MNTI is often locally aggressive with a high recurrence rate. It can be clinically and histologically confused with high-grade malignancies despite being considered benign. We present four cases diagnosed as MNTI through histopathological examination alongside a literature review and would like to study the clinico-radiological findings and histopathological spectrum of this rare entity.

Materials and methods: This retrospective study (January 2018-March 2024) included cases diagnosed histologically as MNTI. Clinical data, including age, gender, and presentation, were collected. Histological and immunohistochemical analyses were performed using various markers.

Results: Four cases (age range, 4-17 months; mean, 5.2 months) were analyzed. Males were more affected than females (3:1). All presented with scalp swelling. MRI revealed large extra-axial masses with varying contrast enhancement. Histopathology showed biphasic cellular morphology with primitive small round cells and epithelioid cells containing melanin. All cases exhibited brisk mitotic activity and extensive desmoplasia. Immunohistochemically, primitive cells were strongly positive for synaptophysin, while epithelioid cells were positive for cytokeratin and HMB45. Ki-67 indices ranged from 30 to 80%. Follow-up revealed one patient succumbed to sepsis and one had a recurrence.

Conclusion: Understanding the clinical and pathological spectrum of MNTI is essential for accurate diagnosis and effective treatment. This study contributes to the existing knowledge by expanding the cohort and enhancing the understanding of this rare tumor.

Cranioplasty to restore calvarial defects involves reconstruction with alloplastic materials or autologous tissues in order to provide the best protection to all intracranial contents. Sometimes, autologous bone may not be available; therefore, different materials have emerged such as polymethylmethacrylate plate, titanium mesh, and hydroxyapatite. However, when it is impossible to replace the autologous bone, the aesthetic result is generally unsatisfactory. Some techniques like neuronavigation and computer-aided design can help overcome these problems. However, these techniques cost too much. By combining these techniques and that of 3D printing, some authors have provided an aesthetically precise and cost-effective implant using polymethylmethacrylate in patients with large craniectomy defects. Here, the authors report a case of a 6-year-old child with history of two previous surgeries for osteolytic extradural and complicated hydatid cyst followed by intracerebral dissemination who was admitted for recurrence of his hydatid pathology. The child was scheduled for hydatid cysts resection and cranioplasty using the polymethylmethacrylate, after removal of the hydatid cysts, using a bone flap cast on a pre-printed cost-effective polylactic acid 3D mold.

Purpose: Povidone-iodine, or Betadine® (Atlantis Consumer Healthcare Inc., Bridgewater, NJ), is a commonly used agent for surgical site preparation. Although commonly used, it carries the risk of skin reactions, and multiple cases of intra-operative contact dermatitis and chemical burns have been reported. However, to our knowledge, there are currently no published cases of povidone-iodine-induced skin lesions in neurosurgical patients.

Methods: A single-center retrospective chart review was performed to identify patients who developed scalp lesions secondary to chemical toxicity following neurosurgical procedures between October 1, 2021, and June 30, 2024.

Results: Three patients were identified, ranging from 2.5 months to 14 years old. Two were positioned prone and the third in lateral decubitus. All patients' heads were supported by a horseshoe headrest covered with a gel pad and wrapped in a cotton roll. For two patients, Reston™ foam (3M©, St. Paul, MN) was added on the horseshoe. Surgical sites were prepped with isopropyl alcohol, Betadine®, and chlorohexidine. Two patients had their heads intermittently lifted throughout the procedure. Lesions were identified immediately after returning the patient to the supine position in the operating room and steadily improved over the course of one to five months with local wound care.

Conclusions: Careful preparation of the surgical site is an essential step in the prevention of surgical site infections. However, caution should be exercised during skin preparation to avoid pooling of povidone-iodine around dependent regions. Additional steps, such as scheduled head elevations and pressure dispersion, should be taken to mitigate factors contributing to these lesions.

Introduction: Idiopathic intracranial hypertension (IIH) is a rare clinical entity in the paediatric population. Clinical presentation is mostly similar to adult counterpart and can include headaches, vomiting, papilloedema, deterioration in visual acuity or fields, and 6th cranial nerve palsy, leading to significant morbidity. Therapeutic lumbar puncture and medical treatment with acetazolamide are usually the first-line treatments. In a minority of refractory cases, particularly where visual function is threatened, CSF diversion has been traditionally used in clinical practice. CSF diversion in IIH patients can be associated with high rate of shunt malfunction and revision due to small ventricular size leading to repeated procedures. We describe our experience with cranial vault expansion as a method of treatment of paediatric IIH cases refractory to medical treatment as a CSF diversion sparing method.

Methods: Following review of IIH cases undergoing surgical treatment in our unit over 15 years (years 2009-2024), cases receiving cranial vault expansion as primary surgical treatment were selected and further analysed. Inclusion criteria were paediatric cases (age < 16) undergoing vault expansion as first surgical treatment for IIH refractory to pharmacological treatment for vision-threatening papilloedema. All cases were discussed within multidisciplinary meeting and selected following consideration of all management options.

Results: Over the studied period, we identified two cases of refractory IIH with severe papilloedema undergoing cranial vault expansion as primary shunt-sparing surgery. Both patients presented with symptoms of headaches, vomiting, and blurred vision with ophthalmological confirmation of severe papilloedema despite pharmacological therapy. Following multidisciplinary discussions, both underwent successful supratentorial vault expansion. Pharmacological treatment was discontinued in both cases following surgery. Evaluation at latest follow-up showed resolution of symptoms and papilloedema with no need for subsequent cerebrospinal fluid diversion at the latest follow-up.

Conclusion: Cranial vault expansion is a viable and safe alternative surgical option in paediatric IIH cases refractory to medical treatment obviating the need for repeated shunt procedures. In cases with extremely small ventricles or where parental choice precludes CSF diversion, cranial vault expansion can be considered.

Purpose: Germinal matrix hemorrhage/intraventricular hemorrhage (GMH-IVH) affects primarily preterm infants and causes significant morbidity and mortality. Health disparities are a reality for underserved populations, such as those rural patients. As our institution serves a substantial portion of rural patients, we examined mortality rates and rates of permanent cerebrospinal fluid (CSF) diversion in newborns with GMH-IVH.

Methods: In this retrospective cohort study of patients with GMH-IVH admitted to our neonatal intensive care unit in 2014-2019, the primary outcomes were rates of mortality and permanent CSF diversion. We examined if demographic, socioeconomic, and disease-specific factors were associated with these outcomes. Analysis was conducted with two definitions of rurality.

Results: A total of 162 patients met study criteria, and there were 26 (15.6%) in-hospital mortalities. Mortality was associated with younger gestational age at birth as quantified by a modified World Health Organization prematurity subcategory (odds ratio [OR] 0.5, 95% confidence interval [CI] 0.2-0.9, p = .04) and with higher grade GMH-IVH (OR 1.8, 95% CI 1.2-2.7, p = .007). Permanent CSF diversion was associated with higher grade GMH-IVH (OR 8.7, 95% CI 3.2-40.4, p < .001). Rurality did not meet univariable screening criteria for logistic regression and was considered non-significant.

Conclusions: The mortality rates of this substantially rural cohort mirrored published rates, and rurality was not associated with increased mortality or hydrocephalus. However, further research with differing definitions of rurality and more patients may reveal healthcare disparities for which actionable interventions can be designed.

Background: Surgical selective dorsal rhizotomy (SDR) in appropriate pediatric cerebral palsy patients is an effective treatment for spasticity. However, there remains heterogeneity reported in postoperative pain management with and without opioid medication in this delicate cohort. The objective of this study was to aggregate pertinent metadata by means of systematic review to summarize all relevant postoperative pain regimens in the literature.

Methods: Searches of multiple electronic databases from inception to June 2024 were conducted following PRISMA guidelines. Articles were screened against pre-specified criteria. Outcomes and regimens were then summarized.

Results: A total of 16 cohort studies were included in this study published between 1989 and 2024. Amongst all studies, outcomes were reported for a total of 636 with median cohort age 6.3 years, and median cohort male proportion 62% was reported. Four studies reported regimens involving systemic analgesia, 8 studies reported regimens involving epidural analgesia, and the remaining 4 studies reported regimens involving intrathecal analgesia. All studies primary pain management involved opioid medication, with 8 studies having opioid medication available as indicated, 3 studies having opioid medication as a single dose, and the remaining studies having opioid medication as a continuous agent in the immediate postoperative period. Across all studies, rates of desaturations, nausea and/or vomiting, and pruritis ranged from 0 to 55%, 25 to 82%, and 15 to 70% respectively. Eleven of the 16 studies included a comparative component, demonstrating that their regimen was at least comparable to their control regimen, if not superior.

Conclusions: Multiple variations of postoperative pain management in pediatric cerebral palsy patients following SDR have been reported in the literature, involving systemic, epidural, and intrathecal analgesia. Concerns for adverse effects with the utilization of opioid medication has led to the trend towards multimodal pain management relying more on non-opioid medication regimens in the more recent literature.