Gamma Knife radiosurgery for arteriovenous malformations in pediatric patients.

OBJECTIVE: The authors present the results of Gamma Knife stereotactic radiosurgery performed in a series of children with arteriovenous malformations (AVMs). METHODS: Between June 2005 and January 2014, 75 patients 18 years old or younger received Gamma Knife radiosurgery for AVMs. Of these, 58 patients were eligible for further analysis. The median age of the population was 12 years; 41% presented with hemorrhage, 34% with neurological insult, and 24% patients were diagnosed incidentally. The median AVM volume was 3.5 cm(3). The median radiosurgery-based AVM score (RSBAVMS) was 0.86. The median follow-up period was 32 months. RESULTS: Single session Gamma Knife radiosurgery resulted in complete AVM obliteration in 40 (68.9%) patients. There were 35 (60.3%) excellent outcome (complete obliteration with no new deficits) in this series. During the follow-up period, nine (15.51%) patients experienced new deficits and three (5.1%) patients experienced intracranial hemorrhage. The annual rate of developing new deficits and hemorrhage was calculated as 5.45 and 1.8%, respectively. Volume, gender, RSBAVMS, and nidus type factor were factors associated with excellent outcome. CONCLUSIONS: Radiosurgery was successful in majority of patients with minimal morbidity. Gamma Knife radiosurgery for AVMs can be a safe and successful method in pediatric patients.

Spondylocostal dysostosis associated with type I split cord malformation and double nipple on one side: a case report.

Jarcho Levin syndrome is a rare genetic disorder characterized by multipl vertebral and costal anomalies at birth. Jarcho Levin syndrome includes two phenotypic groups: spondylothoracic dysostosis and spondylocostal dysostosis. The prognosis of spondylothoracic dysostosis has worse than spondylocostal dysostosis, because of respiratory complications. Associated malformations include those of the congenital heart disease, urogenital malformation, skeletal anomalies and neural tube defects. We present a patient with spondylocostal dysostosis, who also had type I split cord malformation, tethered cord, scoliosis and double nipple on the right. Although the association of spondylocostal dysostosis and type I split cord malformation is very rare, double nipples on one side is no previously reported.

The effect of temozolomide on the prevention of epidural fibrosis developing after lumbar laminectomy in rats.

AIM: Failed back surgery syndrome is observed in 15% of patients who have undergone surgery for lumbar disk hernia.Excess epidural fibrosis is the etiology in 24% of FBSS cases. This study was conducted with the belief that the antiproliferative effect of temozolomide can prevent epidural fibrosis. MATERIAL AND METHODS: 8 rats (Group I) underwent laminectomy and were then administered saline while 6 rats (Group II) were administered temozolomide at a dose of 18 mg/kg/day for 5 days after the surgery to make up a total of 14 male Wistar rats used. The pathology preparations of subjects sacrificed at the end of week 6 were histopathologically examined with the Hematoxylin-Eosin stain and Trichrome stain. The pathology preparations were assessed with the analysis parameters and scale generated by He et al. The results were analyzed with the Chisquare test. RESULTS: No significant difference was found between the two groups in terms of bone and cartilage regeneration, arachnoidal fibrosis, and inflammatory and fibroblast cell densities. Epidural fibrosis formation was significantly less and there was no grade III fibrosis in the Temozolomide group. This was found to be statistically significant (p=0.0302). No side effect of dural or intradural damage was observed. CONCLUSION: Temozolomide was found to be effective in preventing epidural fibrosis. However, further research is required to determine its effectiveness in local applications and the appropriate dose range.

Effects of different surgical techniques on cephalic index and intracranial volume in isolated bilateral coronal synostosis model.

BACKGROUND: Bilateral coronal synostosis (brachycephaly) is the most common single-suture synostosis that may lead to functional deficits such as mental retardation. This increases the importance of volume gain during surgery. This study was designed to understand the differences in volume gain, cranial index (CI), and aesthetic outcomes when additional osteotomies or rotations are applied on the frontoparietal segment. METHODS: Acrylic brachycephaly models were prepared. Frontoparietal osteotomy was standard in all models. Frontoparietal segment was fixed: to the same position in surgical control model, after 1.2-cm advancement in advancement model, after 180-degree rotation without advancement in rotation model, after 180-degree rotation plus a horizontal osteotomy and 1.2-cm advancement in rotation plus angled advancement model, and after a horizontal osteotomy without rotation and 1.2-cm advancement and in angled advancement model. RESULTS: Intracranial volume changes (in milliliters) and CIs were as follows between groups: control group, 828/94.1; surgical control group, 830/93.8; advancement model, 900/84.5; rotation model, 834/89.1; rotation plus angled advancement model, 897/82.7; angled advancement model, 902/81.8. CONCLUSIONS: Advancement of the frontoparietal segment is the keystone of surgery in brachycephaly treatment. Making an additional horizontal osteotomy can angle this segment and may supply additional volume gain. Rotation of the frontoparietal segment does not provide additional volume or CI gain but increase better aesthetic outcomes.

Institutional experience of paediatric high-grade central nervous system tumours: an analysis of 74 patients and review of the literature.

AIM OF THE STUDY: Although the survival for children with certain central nervous system (CNS) tumour types has improved through current surgical and adjuvant treatment modalities, the prognosis of many high-grade tumours remains poor despite aggressive treatment. The aim of this study is to analyse patients with high-grade brain tumours in our institution to determine the histopathology, clinical characteristics, treatment modalities, and survival. MATERIAL AND METHODS: A total of 74 patients with a diagnosis of high-grade brain tumour were analysed. There were a total of 31 patients with embryonal tumours, 27 patients with high-grade glial tumours, 12 patients with brain stem gliomas and 4 patients with other high-grade brain tumours. RESULTS: There were 48 (65%) boys and 26 (35%) girls (ratio: 1.85) with a median age of 99.7 months (range = 2-204 months). The median follow-up period was 19 months (range = 1-204 months). Tumour recurrence was observed in 38 patients (51.4%). The overall survival rate and event-free survival rate of our patients were 27% and 19.5%, respectively. CONCLUSIONS: Pediatric high-grade CNS tumours have a very aggressive behaviour and a significant number of children eventually succumb to disease despite multimodal treatment. There is a need of more effective therapeutic approaches for these tumours with poor prognosis. The future improvement in childhood high-grade brain tumour management depends on a better understanding of the molecular genetics and biology of brain tumours.

Effects of phenytoin sodium on dura mater healing in a rat model of CSF leakage.

AIM: Cerebro-spinal fluid (CSF) leakage caused by defects on the dura mater after trauma or some neurosurgical interventions is an important issue. In this study, we investigated the effects of local and systemic use of phenytoin sodium on dural healing. MATERIAL AND METHODS: Thirty-six male Wistar rats were divided into control, local phenytoin and systemic phenytoin groups with 12 rats in each. For each group, a dura defect was created at thoracic segment. Subjects were sacrificed at following 1st and 6th weeks and damaged segments were isolated. The results were compared histopathologically by Hematoxylin-Eosin and Masson-Trichrome staining. Criteria for the rate of collagen, neovascularization, and granulation formation were assessed semi quantitatively according to the histological assessment scale modified by Ozisik et al. RESULTS: Better healing was achieved in the systemic and local phenytoin groups than in the control group. The level of healing was significantly higher in the systemic group in both early and late periods than in other groups (p < 0.01). The level of healing in the late-local group was also statistically significantly higher than that in the control group. CONCLUSION: We observed that both systemic and local uses of phenytoin sodium (especially systemic) have positive effects on dura healing.

Fractionated gamma knife radiosurgery for optic nerve tumors: a technical report.

AIM: The main purpose of this report is to demonstrate the effects of fractionated radiosurgery regimens in the management of optic gliomas MATERIALS and METHODS: Two patients suffering from progressive loss of vision due to optic nerve gliomas were treated with Gamma Knife radiosurgery in three fractions within three consecutive days instead of a single dose regimen. Patients were followed for 42 and 39 months respectively. RESULTS: The treatment modality was well tolerated by the patients with radiological and neurological tumor control after 42 and 39 months of follow up. There were no additional complications. CONCLUSION: Fractionated regimens of Gamma Knife surgery offer a very good alternative for lesions such as optic glioma and optic nevre sheath meningioma.

The efficacy of vagal nerve stimulation in children with pharmacoresistant epilepsy: practical experience at a Turkish tertiary referral center.

BACKGROUND: Vagus nerve stimulation (VNS) is an effective therapy for pharmacoresistant epilepsy. Nevertheless, information regarding the long-term outcome of VNS in children is limited. AIM: To describe the long-term outcome of VNS in patients with pharmacoresistant epilepsy treated at the Gazi University Medical Faculty Epilepsy Center, Turkey. PATIENTS AND METHODS: The study included 24 patients - all younger than 18 years of age (mean age: 14.31 years). Median age at the time of VNS device implantation was 11 years. Median age at onset of epilepsy was 21 months and median duration of epilepsy was 126 months. All the patients' seizures were intractable with antiepileptic drug treatment and all had been treated with an average of 6+/-2 antiepileptic medications. In all, 12 patients had secondary generalized seizures and 12 had partial seizures. Because this was a retrospective open study, the number of seizures could not be enumerated in most of these cases. RESULTS: The only factor that was associated with seizure reduction was duration of follow-up. Age at seizure onset and age at VNS device implantation were not associated with seizure reduction. The difference in seizure reduction between patients >12 years of age and patients <12 years of age was not significant. Mean percentage of seizure reduction after 6 months-7 years of treatment was, respectively, 22.5% (n=24) (6th month), 32% (n=20) (1st year), 42% (n=16) (2nd year), 50.45% (n=11) (3rd year), 52% (n=10) (4th year), 60% (n=8) (5th year), 61.25% (n=8) (6th year), and 61.6% (n=6) (7th year). The positive effect of VNS persisted throughout the follow-up period. CONCLUSIONS: Although it is an expensive method, VNS is an effective treatment method. This series shows the necessity of long-term follow-up series for understanding the efficacy and advantages of VNS. Prospective, long-term double-blind studies with large samples are needed to confirm the present study's findings.

Iniencephaly and long-term survival: a rare case report.

INTRODUCTION: Iniencephaly, a neural tube defect involving occiput and inion and combined with rachischisis of the cervical, thoracic spine, and retroflexion position of the head is a very rare congenital abnormality of the fetus-newborn with a 0.1-10 of 10,000 prevalence. This abnormality's prognosis is thought to be dismal. This abnormality can be associated with other abnormalities such as anencephaly, encephalocele, hydrocephalus, cyclopia, absence of the mandible, cleft lip and palate, cardiovascular disorders, diaphragmatic hernia, renal abnormalities, overgrowth of the arms compared to the legs, and club food and gastrointestinal atresia. DISCUSSION: Most of the patients are dead born, and the others die in a few hours. There are only six previously documented long-term survivors. In our case, our patient with iniencephalic signs and findings is still living. She is 2 years old now. We think that this patient presents a mild form of iniencephaly.

Split-cord malformation and tethered cord associated with immature teratoma.

CASE REPORT: We report a case of a 12-month-old boy with split-cord malformation, tethered cord, and intradural immature teratoma containing immature nephroblastic tissue. He also had a horseshoe kidney. OUTCOME: Surgical removal of the teratoma and tethered cord resulted in functional improvement of the existing bladder dysfunction. DISCUSSION: To our knowledge, such a case has not been reported before.

Terminal syringomyelia: is it as innocent as it seems?--Case report.

A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases.

Topical mitomycin C-induced inhibition of postlaminectomy peridural fibrosis in rabbits.

OBJECT: The authors conducted a study to determine the effectiveness of mitomycin C in preventing postlaminectomy peridural fibrosis in rabbits. METHODS: Laminectomies were performed at L-4 in 12 rabbits. Color-coded cotton pads soaked either with 0.02% mitomycin C or saline were applied in a blinded fashion to the operative sites, with saline-treated laminectomy sites serving as controls. The rabbits were killed 30 days after surgery. The extent of peridural fibrosis was evaluated by histological analysis. The mitomycin C-treated sites showed significantly decreased peridural fibrosis. CONCLUSIONS: Peridural fibrosis can be a devastating condition that develops after laminectomy. Topical application of mitomycin C may be a successful method of preventing postlaminectomy peridural fibrosis.

Intravenous and local intraarterial tissue-plasminogen activator in a rabbit model of acute thromboembolic stroke: angiographic comparison.

The aim of early thrombolytic therapy in acute stroke is to obtain early recanalization of occluded cerebral arteries and prevent or reduce irreversible ischemic brain damage. In this study, Seldinger technique and digital subtraction angiography were used to verify thromboembolic occlusion of the common carotid artery by an autologous 12-hour-old thrombus in 28 rabbits. Thirty minutes after embolization, 2 mg/kg of recombinant tissue-plasminogen activator (rt-PA) was delivered intravenously by way of the femoral vein in group A (n = 8) and through a local intraarterial infusion in group B (n = 8). Twelve control animals received an equivalent volume of saline intravenously (group C, n = 6) or by local intra-arterial infusion (group D, n = 6). A comparison of posttreatment angiographs of the treatment groups and the control groups was used to demonstrate reperfusion induced by thrombolytic therapy. None of the control animals showed any evidence of recanalization. Both intravenously (group A) and intra-arterially (group B) treated animals achieved 100% recanalization, although the average time to recanalization was significantly longer in group A (P = .01; 123.7 vs 82.5 min). These results suggest that local intra-arterial administration of rt-PA within 30 minutes produces more rapid reperfusion compared with the intravenous route. Further studies and additional treatments are needed to widen the therapeutic window and decrease hemorrhagic side effects.