Peripheral and Intracranial Compartment Serum Level of Selenium in Pediatric Patients with Intracranial Tumor in Department of Neurosurgery, Faculty of Medicine, Universitas Padjadjaran–Dr. Hasan Sadikin Hospital, Bandung, Indonesia: a Preliminary Study and Literature Review

Objective: To determine the level of Selenium (Se) in peripheral and intracranial serum in pediatric patients with intracranial tumor. Selenium has chemopreventive potentials and acts as neuromodulator.Methods: This study was conducted on 13 pediatric patients with intracranial tumors who were treated in Dr. Hasan Sadikin Hospital, Bandung in the period of February 2014 to February 2015. Samples were taken from peripheral and intracranial serum. The results were analyzed with independent t-test and Pearson correlation test. Significance is defined as p≤0.05 with 95% confidence interval.Results: The results showed that the average Se concentration in peripheral serum was significantly higher than the Se concentration in intracranial serum (95.92±20.95 μg/L and 66.62±22.37 μg/L, respectively). After classifying the subjects into groups based on sex, age, tumor location, and grades, the difference between Se concentrations were still statistically significant (p≤0.05), with the exception of the supratentorial group (p=0.0053). Pearson correlation test showed very-low to medium strength correlations between peripheral and intracranial serum Se concentration in all groups (r=0.16–0.59, p>0.05).Conclusions: A significant difference is seen between the peripheral and intracranial serum Se concentration means in pediatric patients with intracranial tumors, with higher concentrations observed in the peripheral serum. Further studies are required to investigate the roles of Se in the management of pediatric patients with intracranial tumors.Keywords: Pediatric intracranial tumor patients, selenium concentration in peripheral and intracranial serum DOI: 10.15850/ijihs.v4n2.83

Characteristics of the Central Nervous System Malformations Presented in Trisomy 13: A single-center Experience in Recognizing the Phenotype and Genotype

BACKGROUND: Patients who are diagnosed with trisomy 13 (Patau syndrome) are known to have a poor prognosis. It has been hypothesized that such poor outcomes are suspected to be attributed to their central nervous system (CNS)-malformations and cardiac-malformations. This study was conducted at Division of Neuropediatric, Department of Neurosurgery, Faculty of Medicine, Universitas Padjadjaran – Dr. Hasan Sadikin Hospital, Bandung (2012–2018). AIM: This study aimed to describe clinical characteristics and karyotype findings in patients who were diagnosed with Patau syndrome and treated at our center. Since Indonesia is still categorized as a lower middle-income country with limited resources, we expected that this study would provide a clinical reference on how a congenital disease with chromosomal abnormalities is confirmed. CASE PRESENTATION: Our cases indicate that CNS malformations are likely to be the cause of indirect mortality of patients’ early period of life. The median survival in our study is 7 days, while the longest survival is 30 days. The major cause of death is apnea, which found in 4 of 5 diagnosed infants. One patient died of severe infection. In most cases, where CNS malformations were observed microcephaly with sloping forehead, Dandy–Walker syndrome, lobar or alobar holoprosencephaly and ventriculomegaly were identified, as well as neural tube defects (NTDs) were identified, such as spina bifida and meningoencephalocele. CNS malformations, such as holoprosencephaly, may be associated with episodes characterized by temporary cessation of spontaneous breathing (apnea) as direct cause of mortality. CONCLUSION: We conclude that early treatment in Patau syndrome patient in our center should be focused on more life-threatening problem caused by CNS malformations than NTDs defects, such defects could be managed electively

Karakteristik Dan Luaran Tumor Otak Pada Anak

Latar belakang. Morbiditas dan mortalitas tumor otak pada anak masih menjadi masalah yang besar. Seringkali manifestasi klinis yang jelas muncul pada saat tumor dalam stadium lanjut. Saat ini luaran tumor otak di Indonesia masih belum memuaskan meskipun telah dilakukan berbagai terapi operatif maupun konservatif. . Tujuan. Mengetahui karakteristik dan luaran pasien tumor otak pada anak di Rumah Sakit Hasan Sadikin Bandung (RSHS). Metode. Dilakukan secara deskriptif retrospektif dengan data pasien tumor otak pada anak berusia < 18 tahun yang datang ke RSHS pada periode 1 Januari 2012 - 31 Desember 2018. Hasil. Selama penelitian terdapat 161 subjek dengan 88 pasien (54,6%) laki-laki. Tumor otak terjadi paling sering pada kelompok usia 6-12 tahun sebanyak 66 pasien (41%). Sebanyak 73 pasien (46,5%) memiliki tumor otak di area supratentorial, 80 pasien (51%) memiliki tumor di area infratentorial, dan 4 pasien (2,5%) memiliki tumor di area suprainfratentorial. Hasil patologi anatomi terbanyak yaitu meduloblastoma pada 36 pasien (22,3%), dengan derajat keganasan VI (46,5%), dengan manifestasi klinis paling sering adalah sakit kepala (64,5%). Pada pemeriksaan neurologis didapatkan parese saraf otak paling sering adalah gangguan nervus kranialis II (34%) dan hemiparesis (20,5%). Ditemukan pula adanya reflek patologis pada 55 pasien (34%). Hasil luaran paling banyak dari data yang tersedia adalah perbaikan dalam gejala klinis (60,3%). Kesimpulan. Meduloblastoma merupakan jenis tumor otak yang tersering (22,3%), pada anak dengan sakit kepala sebagai manifestasi paling banyak

Amniotic band syndrome with CNS involvement: a pediatric neurosurgeon’s dilemma—a case series and literature review

Abstract 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

FETUS INSIDE BRAIN - MATURE CYSTIC TERATOMA IN POSTERIOR FOSSA

Objective: To describe a rare case of posterior fossa cystic teratoma in central nervous system. Methods: A 2-year old boy was admitted with a chief complaint of decreased consciousness with tonic clonic seizure. Non-Contrast Head Computed Tomography (NCCT) scan showed acute hydrocephalus and a well-defined extra-axial cystic lesion mass, 3.4x3.9x4.2 cm in size, in the posterior fossa. Results: Patient underwent a total resection of the tumor. No systemic complication was found in ten days following the surgery. There was no evidence of tumor recurrence one year after the surgery. The histopathologycal diagnosis of the tumor was cystic teratoma. Conclusions: Mature cyst teratoma located in the posterior fossa is a rare case. Total resection and long-term follow up is the treatment of choice for mature teratoma

FETUS INSIDE BRAIN - MATURE CYSTIC TERATOMA IN POSTERIOR FOSSA

Objective: To describe a rare case of posterior fossa cystic teratoma in central nervous system. Methods: A 2-year old boy was admitted with a chief complaint of decreased consciousness with tonic clonic seizure. Non-Contrast Head Computed Tomography (NCCT) scan showed acute hydrocephalus and a well-defined extra-axial cystic lesion mass, 3.4x3.9x4.2 cm in size, in the posterior fossa. Results: Patient underwent a total resection of the tumor. No systemic complication was found in ten days following the surgery. There was no evidence of tumor recurrence one year after the surgery. The histopathologycal diagnosis of the tumor was cystic teratoma. Conclusions: Mature cyst teratoma located in the posterior fossa is a rare case. Total resection and long-term follow up is the treatment of choice for mature teratoma

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

Unilateral moyamoya disease mimicking intracranial hemorrhage in a pediatric patient: Surgical treatment with encephalo-duro-myo-synangiosis in a progressive disease

Background: Moyamoya disease (MMD) is characterized by progressive bilateral internal carotid artery stenosis and its distal branches. When angiographic findings are unilateral, the diagnosis is considered to be probable MMD. Surgery is recommended treatment of patients with deterioration caused by progressive cerebral ischemic events. Different techniques have been described, and the encephalo-duro-myo-synangiosis (EDMS) procedure is highly recommended for patients with rapid deterioration. A case of unilateral MMD mimicking intracranial hemorrhage in a pediatric patient is reported. Case description: A 14-year-old girl was referred to our center due to a decreased level of consciousness. Physical examination revealed right-sided hemiparesis; however, a brain CT scan showed multiple hyperdense masses mimicking a hemorrhagic lesion. A brain MRI showed a large hypointensity in the temporoparietal, suggesting a vascular lesion. In all children that exhibit encephalitis, vascular events such as MMD should be considered. An emergency surgical EDMS procedure was performed, and the patient regained consciousness and exhibited no TIAs during the follow-up period. A postoperative brain MRI showed an improvement in brain vascularity. Conclusion: After EDMS, a considerable degree of neurological recovery was observed in our patient with rare unilateral MMD mimicking intracranial hemorrhage. We found that the progression of clinical improvement after indirect revascularization in our case was due to EDMS. Surgical treatment with EDMS is reasonable for MMD because it allows for flexible revascularization that adequately addresses the requirement for new blood supply in the ischemic lesion. The EDMS procedure is followed by an observation period to preclude the possibility of the patient developing definite MMD. Keywords: Unilateral moyamoya disease, Encephalo-duro-myo-synangiosis, Ischemi