Surgical techniques in the management of supratentorial pediatric brain tumors: 10 years' experience at a tertiary care center in the Middle East.

BACKGROUND: The goal of this retrospective study is to present the first epidemiological data on pediatric supratentorial central nervous system (CNS) tumors in Lebanon and to review the various surgical management strategies used. METHODS: We conducted a retrospective case series of all pediatric patients who presented with a supratentorial CNS tumor and underwent surgery at our institution between 2006 and 2016. We collected and analyzed demographic characteristics, tumor location, clinical manifestations, histopathology, and surgical management strategies and outcome, and discussed them after dividing the tumors as per location and in view of published literature. RESULTS: Ninety-nine children were studied with a male-to-female ratio of 2.3:1 and a mean age of 8.5 years. The most common location was convexity (44%) and included low-grade and high-grade glial tumors, along with other miscellaneous lesions. The next location was sellar/diencephalic (34%), including craniopharyngiomas, hypothalamic/optic pathway/thalamic gliomas, hamartomas, and pituitary/Rathke's cyst, where there was notable use of endoscopic techniques (21%). Tumors in the pineal region (13%) were tectal gliomas, germ cell tumors, and pineoblastomas and were mostly treated endoscopically. The last group was lateral intraventricular tumors (8%) and was mostly choroid plexus lesions and ependymomas. Overall, the surgical objective was achieved in 95% with mild/moderate complications in 17%. CONCLUSION: A variety of pathologies may affect the pediatric population in the supratentorial region. Different surgical strategies, including microsurgical and endoscopic techniques, may be employed to remove, debulk, or biopsy these tumors depending on their location, suspected diagnosis, prognosis, and the need for treatment of possible associated hydrocephalus.

Outcomes following Craniosynostosis Surgery at a Tertiary Care Center in the Middle East.

BACKGROUND: Despite advancements in craniosynostosis surgery, open surgical approaches remain crucial for the management of infants >6 months of age and in those with complex synostosis. The clinical features of craniosynostosis remain poorly characterized in the Middle East. This study sought to assess the clinical features and outcomes of infants undergoing craniosynostosis surgery at a tertiary care center in Lebanon. METHODS: A retrospective review was performed of all patients who underwent craniosynostosis surgery from December 2006 to December 2018 at the American University of Beirut Medical Center, Lebanon. Clinicodemographic characteristics, complications, and recurrence outcomes were recorded and evaluated using descriptive statistics. RESULTS: Thirty-five infants met the inclusion criteria, with a mean age of 9.0 ± 4.0 months. The most common site of suture involvement was metopic (28.6%), followed by unilateral coronal (25.7%), sagittal (20.0%), bicoronal (8.6%), and multiple sites (17.1%). Five patients (14.3%) had syndromic synostosis. Median estimated blood loss was 200 mL, and median volume of transfused packed red blood cells was 180 mL. Two patients (5.7%) experienced postoperative complications, including postoperative blood transfusion (n = 1)and wire protrusion requiring removal (n = 1). Three patients (8.6%) required reoperation: 2 (5.7%) for resynostosis and 1 for traumatic fracture repair. Caregivers of all patients reported high satisfaction with cosmetic outcomes 4 weeks postoperatively. CONCLUSIONS: With appropriate perioperative precautions, open craniosynostosis surgery can be performed with minimal complications, low recurrence rates, and satisfactory cosmetic outcomes. Additional population-level data are needed to better characterize craniosynostosis patterns and outcomes in the Middle East.

Salvage therapy for vagal nerve stimulator infection; Literature review and report of a delayed recurrence.

BACKGROUND: Vagal Nerve Stimulation (VNS) is one of the most common neuro-modulation based approaches for the treatment of medically intractable epilepsy. Despite advances in technology and surgical techniques, hardware infection remains a recognized and feared complication in VNS placement. Management of such infections is scarce in the literature with the majority of data available in case reports. It ranges from immediate removal of the VNS device to conservative treatment with antibiotics in an attempt to salvage the device, particularly in patients who demonstrated significant improvement in seizure frequency and quality of life. METHODS: We performed a review of the literature in accordance with PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines to identify reported cases of salvaged VNS infection. A literature search for relevant English articles was conducted using Medline. References of relevant articles were also reviewed. Articles that comprised an attempt to salvage an infected VNS were included. RESULTS: We obtained 12 articles describing an attempt to salvage an infected VNS. Out of a total of 62 reported VNS infections and 43 salvage attempts using a variety of antibiotic-based approaches, 17 cases were successfully salvaged and 26 cases failed the salvage attempt and had to be explanted eventually. Moreover, we report a case of an 18-year-old male with Lennox-Gastaut syndrome who presented21 days after VNS placement with a MRSA deep tissue infection. An attempt was made to treat the infection with long-term culture-based intravenous antibiotics, but it recurred three years later with neck wound dehiscence and positive wound culture for the same organism, and ex-plantation was thus performed. CONCLUSION: The management of VNS infections remains a dilemma for neurosurgeons. Although the idea of salvaging an infected VNS seems appealing, hardware removal seems to be inevitable despite adequate antibiotic treatment.

Correlation of genetic alterations by whole-exome sequencing with clinical outcomes of glioblastoma patients from the Lebanese population.

INTRODUCTION: Glioblastoma (GBM) is an aggressive brain tumor associated with high degree of resistance to treatment. Given its heterogeneity, it is important to understand the molecular landscape of this tumor for the development of more effective therapies. Because of the different genetic profiles of patients with GBM, we sought to identify genetic variants in Lebanese patients with GBM (LEB-GBM) and compare our findings to those in the Cancer Genome Atlas (TCGA). METHODS: We performed whole exome sequencing (WES) to identify somatic variants in a cohort of 60 patient-derived GBM samples. We focused our analysis on 50 commonly mutated GBM candidate genes and compared mutation signatures between our population and publicly available GBM data from TCGA. We also cross-tabulated biological covariates to assess for associations with overall survival, time to recurrence and follow-up duration. RESULTS: We included 60 patient-derived GBM samples from 37 males and 23 females, with age ranging from 3 to 80 years (mean and median age at diagnosis were 51 and 56, respectively). Recurrent tumor formation was present in 94.8% of patients (n = 55/58). After filtering, we identified 360 somatic variants from 60 GBM patient samples. After filtering, we identified 360 somatic variants from 60 GBM patient samples. Most frequently mutated genes in our samples included ATRX, PCDHX11, PTEN, TP53, NF1, EGFR, PIK3CA, and SCN9A. Mutations in NLRP5 were associated with decreased overall survival among the Lebanese GBM cohort (p = 0.002). Mutations in NLRP5 were associated with decreased overall survival among the Lebanese GBM cohort (p = 0.002). EGFR and NF1 mutations were associated with the frontal lobe and temporal lobe in our LEB-GBM cohort, respectively. CONCLUSIONS: Our WES analysis confirmed the similarity in mutation signature of the LEB-GBM population with TCGA cohorts. It showed that 1 out of the 50 commonly GBM candidate gene mutations is associated with decreased overall survival among the Lebanese cohort. This study also highlights the need for studies with larger sample sizes to inform clinicians for better prognostication and management of Lebanese patients with GBM.

Anaplastic Meningioma Presenting as a Left Parietal Mass: A Case Report.

BACKGROUND Meningiomas are slow-growing tumors attached to the dura mater and are composed of neoplastic meningothelial cells. The tumors are most commonly located in convexities, and it is relatively rare to find such a growth in the parietal region such as the one presented in this case report. CASE REPORT Because of its uncommon presentation, we hereby report the case of a 67-year-old, previously healthy, white Lebanese female patient who presented with forgetfulness, unsteady gait, right-sided motor weakness, and dysphagia. She was found to have an anaplastic meningioma located in the left parietal lobe that was treated by surgical resection. The patient had an uneventful postoperative course, and was stable at later follow-ups. CONCLUSIONS This case report describes the clinical presentation, pathological findings, and the prognosis of this mass, which is atypical in this location, and has been rarely reported in the literature. We also review the literature on anaplastic meningiomas.

Evaluation of intractable epilepsy. Invasive monitoring.

Accurate localization of the epileptogenic zone is the fundamental key factor for successful epilepsy surgery. Despite the progress achieved in the field of neuroimaging, invasive intracranial recording is still the gold standard that helps recognize the patient population who may profit from surgery. Meticulous implantation of intracranial electrodes and judicious interpretation of their data is a definite need in a successful epilepsy program. Few centers in the Arab world are in that domain. Moreover, the society itself is not well informed to appreciate the potentials of surgical treatment of seizure disorders. In this review article, we will go over various types of intracranial recordings, discussing their indications, and the last updates for each. Some of the cases carried out at the American University of Beirut Medical Center, Beirut, Lebanon will be illustrated as examples of our current practice. Then, the future of epilepsy monitoring will be highlighted in brief.

Endoscopy in the management of intra-ventricular lesions: preliminary experience in the Middle East.

OBJECTIVES: The study is aimed at evaluating neuroendoscopic procedures in the management of various intra-cranial intra-ventricular lesions. METHODS: We retrospectively review 24 consecutive patients harboring various intra-ventricular lesions with or without associated hydrocephalus, operated and managed by the authors between December 2002 and December 2007. Depending on the preoperative imaging and working diagnosis, endoscopic biopsy or endoscopic resection/debulking, along with concomitant treatment of hydrocephalus via third ventriculostomy or fenestration of the septum pellucidum and ventriculo-peritoneal shunting, were done. RESULTS: The single endoscopic procedure was successful in most of the patients (22/24), where the goals of surgery in attaining a diagnostic biopsy or resection, and simultaneous treatment of hydrocephalus, were attained helping guide further therapy. The endoscopic procedure was the only procedure needed in most of the patients. CONCLUSIONS: The endoscopic procedures carried a high success rate in the management of intra-ventricular lesions, and were especially valuable in patients harboring chemo and/or radiosensitive deep seated tumors.

Quality of life after vagal nerve stimulator insertion.

AIM: Assess quality-of-life after vagal nerve stimulation and determine patient characteristics associated with improvement in quality-of-life. METHODS: Sixteen patients (11 children, 5 adults) who had vagal nerve stimulation at our center were studied. Quality-of-life was assessed pre- and post-vagal nerve stimulation using the Quality-of-Life in Childhood Epilepsy questionnaire for children and the Epilepsy Surgery Inventory-55 for adults. RESULTS: Sixteen patients who did not qualify for resective surgery were included; seven (43.75%) were males and 9 (56.25%) were females. Mean age at onset of seizures was 3.96 +/- 4.00 years and at surgery was 15.78 +/- 10.78. Follow-up time was 1.26 +/- 0.92 years. Fourteen patients (87.5%) were mentally retarded. Ten (62.5%) had cryptogenic etiology and 6 patients (37.5%) symptomatic etiology. Fifty percent had localization-related epilepsy. Six of 7 patients with generalized cryptogenic etiology (85.71%) had Lennox-Gastaut syndrome. Seizures dropped from 122.31 +/- 159.49 to 67.84 +/- 88.22 seizures/month. Seizure reduction (> 50%) correlated with improvement in total quality-of-life (p = 0.034). Post-vagal nerve stimulation, the total group scored significantly higher in the social domain (p = 0.039). In patients with localization-related epilepsy, significant improvements were detected in the social domain (p = 0.049) and in total quality-of-life (p = 0.042). CONCLUSION: Despite a diverse and small population size, we observed significant improvements in the social domain 1.26 years post-vagal nerve stimulation. In addition, there was an improvement in total quality-of-life amongst patients with partial seizures. Finally, seizure reduction was associated with quality-of-life improvement. Our results support previous studies from the West reporting improvement in quality-of-life following vagal nerve stimulation, contradict those studies that did not show such differences, and are the first coming from a developing country.

A child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau-Kleffner autistic syndrome.

A 7-year-old, right-handed girl started to have seizures at age 1 year 4 months. She developed normally until age 4 when she had worsening of seizures with auditory verbal agnosia, complete aphasia, and a behavioral disorder fulfilling the diagnostic criteria of autism. Medical therapy failed. MRI revealed a right temporal tumor. Video/EEG monitoring at age 7 showed contralateral electrical status epilepticus in wakefulness and sleep and ipsilateral onset of seizures. Resection (ganglioglioma with excessive inflammation) resulted in seizure freedom and marked reduction of the autistic features. This case is unique for being, to our knowledge, (1) the first in which a lesion located in the right, rather than left, temporal lobe resulted in secondary falsely localizing left temporal lobe electrical status epilepticus with a clinical picture of Landau-Kleffner syndrome and autism, and (2) the fourth reported patient with lesional Landau-Kleffner syndrome to respond to resective surgery.

Intracranial hydatid cyst. Dilemma in diagnosis and management.

Intracranial hydatid disease is a parasitic infection that affects children in endemic areas such as the Mediterranean, South America, and Australia. In non-endemic areas, it is rare and may pose a diagnostic dilemma. We review a boy who presented with a right cerebral cyst. All investigations were negative, and hydatid cyst was encountered upon intra-operative exploration. We review the available diagnostic modalities, and the difficulty in reaching a preoperative diagnosis. We also review the surgical and medical treatment strategies. A cerebral hydatid cyst should be considered in children with cystic brain lesions even in non-endemic areas. Since serologic tests are frequently negative, the most reliable methods in reaching a diagnosis are radiological evaluation and histopathological examination. The cyst has a typical appearance on CT and MRI and this bears special importance on the treatment strategy where the cyst is best removed intact to avoid recurrence or anaphylaxis.

Pseudotumor cerebri: disordered cerebrospinal fluid hydrodynamics with extra-axial CSF collections.

Pseudotumor cerebri is uncommon in the pediatric age group and presents with clinical characteristics different from the adult form. We report a 5-year-old boy who presented with--seemingly spontaneous--alternating subdural CSF collections before progressing to develop pseudotumor cerebri. To our knowledge, this is the first report of such a presentation. In the prepubertal pediatric age group, pseudotumor cerebri may be associated with multiple etiologic factors (more than in the adult population). The exact common mechanism behind such a disorder is not known. Pseudotumor cerebri may be a disorder of CSF hydrodynamics. It may also be linked to other disorders of CSF hydrodynamics, as in our case. The exact link and mechanistic differences between these apparently related disorders deserve further analysis and investigation, possibly through dynamic MRI techniques.

Dorsal intramedullary dermoids.

We report a case of a 31-year-old woman with an intramedullary thoracic dermoid, who had progressive spastic paraparesis in her lower extremities over a period of three years, as well as loss of bowel and bladder control. She had been operated for "spina bifida" at three days of age but had no neurologic deficit. MRI of the thoracic spine showed an intramedullary hypointense lesion at T7-T8, associated with an intramedullary lipoma at a higher level. Intracapsular resection of an intramedullary dermoid cyst was performed. We review the literature on intramedullary dermoids differentiating them from the more common, but still rare, lumbosacral subdural juxtamedullary dermoids. We discuss the associated malformations with these lesions, as well as their pathophysiology and treatment. Although uncommon, dorsal intramedullary dermoids should be suspected and treated in a timely manner, especially in presence of other congenital anomalies.

Prevalence of neuropathic pain among patients suffering from chronic low back pain in Saudi Arabia.

OBJECTIVE: The aim of this study was to assess the prevalence of neuropathic pain among patients suffering from chronic low back pain using the Leeds assessment of neuropathic symptoms and signs (LANSS) pain scale. METHODS: This was a pilot study collected from 10 centers in the Middle East Region, with each center enrolling 10 patients between November 2004 and January 2004. In total, 100 patients with chronic low back pain were included in the study. The LANSS clinical assessment score was used to assess the presence of neuropathic pain. Patients with score >12 were considered to have neuropathic pain, while patients with score a < 12 were considered as having nociceptive pain. RESULTS: We found that 41% of the chronic low back pain patients had neuropathic pain and 59% had nociceptive pain. CONCLUSION: The ability to identify neuropathic pain mechanisms should lead to individualized treatment resulting in improved pain control in this group of patients with chronic low back pain.

Idiopathic spinal cord herniation: a new theory of pathogenesis.

BACKGROUND: Idiopathic spinal cord herniation is a rare entity that has been described more frequently over the past few years. Its pathophysiology remains obscure, however. METHODS: We report a case of spinal cord herniation and review the literature extensively. In view of our review, we try to determine the clinical features of the condition and the diagnostic measures used, with emphasis on the role of magnetic resonance (MR) phase-contrast CSF study. The factors affecting the outcome of the condition are also studied including time and type of presentation, as well as the surgical procedure performed. The pathophysiological mechanisms behind spontaneous herniation are discussed, and a new hypothesis is proposed. RESULTS: Idiopathic spinal cord herniation occurs in the middle-aged adult, with a preponderance of patients being female. Brown-Séquard syndrome is the most common clinical presentation and usually progresses to spastic paraparesis. MRI typically shows a ventral kink in the thoracic cord, with MR phase-contrast imaging proving an important addition to exclude an arachnoid cyst. Better outcomes were noted in the patients treated earlier, and in those with no spasticity. Widening the dural defect seems to afford better results compared to grafting of the defect. The prognosis is favorable after correction, though a vertebral body herniation variant may be associated with worse outcome. In view of the chronology of events and imaging studies in our patient, we hypothesize that herniation occurs as an acquired phenomenon where an inflammatory process results in adherence between the spinal cord and the dura, with erosion, formation of a dural defect, and then later herniation occurring with cerebral spinal fluid (CSF) pulsations. CONCLUSIONS: Idiopathic herniation of the spinal cord should be recognized and treated early to reach a favorable outcome. It seems to be an acquired condition likely caused by an inflammatory event, the nature of which is to be determined.

Prevalence of neuropathic pain among patients suffering from chronic low back pain in Saudi Arabia.

OBJECTIVE: The aim of this study was to assess the prevalence of neuropathic pain among patients suffering from chronic low back pain using the Leeds assessment of neuropathic symptoms and signs (LANSS) pain scale. METHODS: This was a pilot study collected from 10 centers in the Middle East Region, with each center enrolling 10 patients between November 2004 and January 2004. In total, 100 patients with chronic low back pain were included in the study. The LANSS clinical assessment score was used to assess the presence of neuropathic pain. Patients with score > or =12 were considered to have neuropathic pain, while patients with score a less than 12 were considered as having nociceptive pain. RESULTS: We found that 41% of the chronic low back pain patients had neuropathic pain and 59% had nociceptive pain. CONCLUSION: The ability to identify neuropathic pain mechanisms should lead to individualized treatment resulting in improved pain control in this group of patients with chronic low back pain.