Amniotic band syndrome with CNS involvement: a pediatric neurosurgeon's dilemma-a case series and literature review.

BACKGROUND: Amniotic band syndrome (ABS) is a rare congenital disease characterized by a broad spectrum of congenital anomalies resulting from the strangulated developing organ(s) by the detached fibrous amniotic band. The prevalence of CNS involvement in ABS is rare, but the mortality rate in these cases is high, while morbidity among the surviving patients is inevitable. CASE REPORT: Three-month-old male, 9-month-old female, and newborn female babies were presented with head lump(s), severe facial cleft, syndactyly, and finger amputation. The patient's head imaging confirmed meningoencephalocele as the cause of the head lump in 2 patients; meanwhile, a porencephalic cyst was identified as the origin of head lumps in the other patient. VP shunt placement surgery was performed as the initial management in 2 patients, while one patient directly underwent meningoencephalocele resection surgery. Craniofacial and limb reconstructions were planned as the follow-up management in all cases. Unfortunately, one patient died of complications from suspected aspiration, while another never returned for follow-up treatment. CONCLUSION: Here, we report 3 ABS cases with CNS involvement. Despite the severe disfigurement and disability, the inexistence of fatal malformation might lead to long-term survival. The treatment of malformation(s) that might predispose to another fatal condition and surgery(-ies) to improve functional outcomes and patient's social acceptability should be prioritized in managing the surviving ABS patients.

Periventricular hypodensity is associated with the incidence of pre-shunt seizure in hydrocephalic children.

BACKGROUND: The seizure incidence in hydrocephalic children has been acknowledged in a lot of studies previously; nonetheless, seizure pathogenesis in these children remains unclear. Its high proportion of hydrocephalic children who underwent shunt surgery suggests that the seizure might be associated with the protocol of shunt placement and/or the shunt existence intracranially; however, this hypothesis could not explain the pre-shunt seizure incidence in hydrocephalic children. OBJECTIVE: This study aims to evaluate the patients' characteristics and CT findings in pre-shunt hydrocephalic children to identify the possible seizure etiology in these patients. METHODS: Three hundred and thirty-four children with hydrocephalus were included in this study, including 147 hydrocephalic children with the pre-shunt seizure history and 187 hydrocephalic children presented without the pre-shunt seizure history. The following information was retrieved from the patients' medical records: gender, age, pediatric Glasgow Coma Scale (pGCS) upon admission, and hydrocephalus diagnoses. CT findings were re-evaluated to assess the compression association of sulci and gyri, Sylvian fissures, cisterns, FH/ID ratio, Evan's ratio, and periventricular hypodensity with pre-shunt seizure. RESULTS: The results show that the pre-shunt seizure incidence is significantly higher in hydrocephalic children aged 1 to 5 years old (63/113 (55%), p = 0.0001), diagnosed with communicating hydrocephalus (97/163 (59%), p = 0.0001) or infectious hydrocephalus (80/109 (73%), p = 0.0001). The presence of periventricular hypodensity is significantly associated with the pre-shunt seizure incidence (132/205 (64.3%), p = 0.0001). Results from univariate analyses suggest significant association between periventricular hypodensity in every location and pre-shunt seizure (p < 0.0001). Multivariate analyses identify that temporal horn in the right lateral ventricle as the location of periventricular hypodensity has the strongest association with the pre-shunt seizure. CONCLUSION: The presence of periventricular hypodensity in head CT scan is significantly associated with the pre-shunt seizure incidence. Further investigation to confirm this finding and evaluate the possible roles of inflammation in the pre-shunt seizure in hydrocephalic children is important to seek its possible implication on the treatment of pre-shunt seizure in these children.

Neurodevelopmental outcomes after ventriculoperitoneal shunt placement in children with non-infectious hydrocephalus: a meta-analysis.

BACKGROUND: Hydrocephalus is diagnosed when an accumulating amount of cerebrospinal fluid (CSF) fails to circulate and/or absorbed in the ventricular system. Based on its etiology, hydrocephalus can be classified into infectious and non-infectious hydrocephalus. In children, non-infectious hydrocephalus includes congenital hydrocephalus, posthemorrhagic hydrocephalus, neural tube defect-related hydrocephalus, and tumor-related hydrocephalus. Regardless of the cause, a CSF diversion device is placed to divert the excess fluid from the ventricles into peritoneal cavity. Among all, ventriculoperitoneal (VP) shunt is arguably the most commonly used CSF diversion device to date. Until now, the long-term neurodevelopmental impact of VP shunt placement in non-infectious hydrocephalus patients remained unclear. OBJECTIVE: This study aims to evaluate the neurodevelopmental outcomes in children with non-infectious hydrocephalus who had VP shunt placement. MATERIALS AND METHODS: Systematic searches were performed using PubMed, Google Scholar, Scopus databases, and reference lists. Publications that fulfilled the inclusion criteria were included in the meta-analysis. Calculation of Mantel-Haezel risk ratio (RR) was applied, and heterogeneity index (I2) test was used to evaluate the existence of heterogeneity in all studies. Risk of bias was assessed based on the criteria from the Newcastle-Ottawa Scale (NOS). RESULTS: Of the 1929 studies identified, 12 publications were concluded to have fulfilled the inclusion criteria. Results from the meta-analysis showed that the risks of cerebral palsy, visual and hearing impairment, epilepsy, or seizures are significantly higher in children with non-infectious hydrocephalus who already had VP shunt placement (shunted non-infectious hydrocephalus, S-NIH) compared to that of the healthy control. The meta-analysis on intelligent quotient (IQ) and mental development index (MDI) showed that S-NIH children tend to score lower IQ and acquire risk of having mental development delay. On motoric development, S-NIH children scored lower motoric score and have significantly higher risk of motor development delay compared to control. Although normal children tend to have more internalizing behavior compared to S-NIH children, overall assessment on the risk of behavioral abnormalities showed that the differences between these two groups are insignificant. CONCLUSION: S-NIH children have significantly higher risks of disabilities and mental and motoric development delays; thus, planning on continuous rehabilitation for children with non-infectious hydrocephalus who already had placement of VP shunt is important to acquire their optimum potentials and quality of life.

Nasofrontal encephalocele: A case report with literature and management review.

Encephalocele is a rare congenital anomaly characterized by the protrusion of intracranial contents through a defect in the skull base or calvarial. In Southeast Asia, frontoethmoidal encephaloceles are more frequently observed compared to their occurrence in Western countries. Typically, frontoethmoidal encephaloceles present as a visible mass adjacent to the nasal region. In this report, we delineate the case of a 9-year-old boy who presented with a palpable mass on the nasal bridge. Subsequent ultrasound and CT scan evaluations identified a nasofrontal defect with a communicating connection to the intracranial compartment.

Impact of COVID-19 on Neurosurgical Training in Southeast Asia.

OBJECTIVE: Neurosurgery departments worldwide have been forced to restructure their training programs because of the coronavirus disease 2019 (COVID-19) pandemic. In this study, we describe the impact of COVID-19 on neurosurgical training in Southeast Asia. METHODS: We conducted an online survey among neurosurgery residents in Indonesia, Malaysia, Philippines, Singapore, and Thailand from May 22 to 31, 2020 using Google Forms. The 33-item questionnaire collected data on elective and emergency neurosurgical operations, ongoing learning activities, and health worker safety. RESULTS: A total of 298 of 470 neurosurgery residents completed the survey, equivalent to a 63% response rate. The decrease in elective neurosurgical operations in Indonesia and in the Philippines (median, 100% for both) was significantly greater compared with other countries (P < 0.001). For emergency operations, trainees in Indonesia and Malaysia had a significantly greater reduction in their caseload (median, 80% and 70%, respectively) compared with trainees in Singapore and Thailand (median, 20% and 50%, respectively; P < 0.001). Neurosurgery residents were most concerned about the decrease in their hands-on surgical experience, uncertainty in their career advancement, and occupational safety in the workplace. Most of the residents (n = 221, 74%) believed that the COVID-19 crisis will have a negative impact on their neurosurgical training overall. CONCLUSIONS: An effective national strategy to control COVID-19 is crucial to sustain neurosurgical training and to provide essential neurosurgical services. Training programs in Southeast Asia should consider developing online learning modules and setting up simulation laboratories to allow trainees to systematically acquire knowledge and develop practical skills during these challenging times.

Cystic pilomyxoid astrocytoma on suprasellar region in 7-year-old girl: Treatment and strategy.

Pilomyxoid astrocytoma (PMA) is a recently described entity with similar features to pilocytic astrocytoma but with a rare occurrence. As a new diagnosis, no treatment guideline of PMA has been established; but generally, as for any low-grade gliomas, radical resection is performed if the location is favorable. In this report, we wished to share our experience treating the PMA. The authors presented a case of a 7-year-old girl with bitemporal hemianopia. From the history, the patient had a 4-month history of headache, following with nausea and projectile vomiting 1 week before hospital admission. Past history of seizure, weakness of left extremities, and decreased consciousness were reported. Computed tomography (CT) scanning showed acute obstructive hydrocephalus and an isohypodense mass at suprasellar region with the cystic component. We performed ventriculo-peritoneal-shunt to reduce the acute hydrocephalus, followed by craniotomy tumor removal 2 weeks later. The patient underwent radiotherapy and medical rehabilitation. Diagnosis of PMA was made on the basis of pathologic anatomy result, which showed a myxoid background with pseudorosette. Postoperative CT showed a residual tumor at right parasellar area without hydrocephalus. After the surgery, the treatment was followed with radiotherapy for 20 times within 2 months. Postradiation CT performed 1-year later showed a significant reduction of the tumor mass. There were no new postoperative deficits. The patient had improvement of the visual field and motor strength. The authors reported a case of a 7-year-old girl with PMA. Surgical resection combined with radiotherapy was performed to control the growth of PMA. More observation and further studies are required to refine the treatment methods.