An infant with intradural extramedullary synovial sarcoma: the youngest case in the literature.

Yalçin K, Tüysüz G, Kazan S, Gürer EI, Karaali K, Küpesiz A, Güler E. An infant with intradural extramedullary synovial sarcoma: the youngest case in the literature. Turk J Pediatr 2019; 61: 765-770. Spinal cord involvement of synovial sarcoma is extremely rare. So far only two cases have been reported. Herein we describe the youngest case in the literature. She is 14-month-old and first presented with difficulty in walking ongoing for a week. Imagining showed a spinal cord mass at C5-T3 levels. The patient had gone under Decompressive surgery and histopathologic examination of the specimen revealed the presence of synovial sarcoma. Although the tumor regressed after chemotherapy, she was lost due to viral pneumonia. Synovial sarcoma should be kept in mind while evaluating spinal tumors even in infantile group.

Evaluation of Cortical Brain Parenchyma by Diffusion and Perfusion MRI Before and After Chronic Subdural Hematoma Surgery.

AIM: To evaluate microcirculatory changes in neighboring parenchyma as a result of pressure due to chronic subdural hematoma (CSDH) in early and late periods after hematoma drainage. MATERIAL AND METHODS: The subject group consisted of 25 patients who underwent CSDH drainage. Brain diffusion and perfusion magnetic resonance images (MRIs) were obtained preoperatively, and at 48 hours (early period) and 2 months (late period) postoperatively. Measurements were performed on 1 cm2 regions of interest (ROI) in the neighboring parenchymal tissue. RESULTS: The early postoperative diffusion values showed improvement compared to the preoperative values. The late postoperative values showed improvement compared to the preoperative and early postoperative values. The early postoperative perfusion values showed slight decline compared to the preoperative values. However, the late postoperative values showed improvement compared to the preoperative and early postoperative values. CONCLUSION: The fact that there was an increase in diffusion values from early to late postoperative periods, compared with the preoperative period, indicates that the beneficial effects of surgery increase over time. Brain perfusion was found to be slightly decreased in early postoperative period. Following CSDH drainage, neurological deteriorations are observed in some patients in the early postoperative periods; a slight impairment in perfusion may account for this. However, during the late postoperative period, perfusion was seen to recover prominently.

Spontaneous Shrinkage of a Suprasellar Arachnoid Cyst Diagnosed with Prenatal Sonography and Fetal Magnetic Resonance Imaging: Case Report and Review of the Literature.

Arachnoid cysts (AC's) are congenital anomalies that occur within the cerebrospinal fluid (CSF) cisterns and major cerebral fissures. Suprasellar AC's comprise 5-12.5% of all the lesions. Despite being commonly reported, their etiology and pathogenesis still remain unclear. In this report, we presented an unusual case of spontaneous shrinkage of a suprasellar AC that had been incidentally diagnosed during a routine prenatal sonographic examination. To our knowledge, only three cases of spontaneous shrinkage of suprasellar AC have been reported in the literature. In detail of the manuscript, fetal and postnatal radiological images were presented and the possible mechanisms were discussed with a review of the literature.

The effect of amifostine on pathophysiological changes in vasogenic brain edema induced by an experimental cold injury model.

AIM: To investigate the effects of amifostine, a cytoprotective agent, on pathophysiological changes in vasogenic brain edema induced by an experimental cold injury model and to compare these changes with dexamethasone. MATERIAL AND METHODS: A total of 138 rats divided into 6 groups. Brain water content (BWC), malondialdehyde (MDA) concentration and myeloperoxidase (MPO) activity in brain tissue were calculated to evaluate the pathophysiological changes following experimental cold injury. In addition, effects of cold injury on cell structure were assessed with direct light and transmission electron microscopy (TEM). RESULTS: Extent of edema, MDA and MPO levels were significantly higher in cold injury groups than in controls. Although a decrease was noted in these parameters in both the amifostine and dexamethasone groups, the differences were significant only for MDA concentration in dexamethasone group, and for MPO activity in both groups. In addition, there was a significant difference between the group in which amifostine was administered prior to cold injury and dexamethasone group for MPO activity. Histopathologically, positive effects were observed in treatment groups. CONCLUSION: Despite several positive effects of amifostine, its superiority to dexamethasone could not be clearly demonstrated. Further experimental and clinical studies are warranted to better delineate the neuroprotective effects of amifostine.

Effects of decompressive surgery in patients with severe traumatic brain injury and bilateral non-reactive dilated pupils.

BACKGROUND: We investigated Glasgow Coma Scale (GCS) scores, intracranial pressure (ICP) and cerebral perfusion pressure (CPP) changes, and long-term clinical outcomes in patients with severe traumatic brain injury (STBI) associated with bilateral non-reactive dilated pupils (BNDP) who underwent decompressive surgery (DS). METHODS: The study group consisted of 28 patients (11 females, 17 males) with BNDP from among 147 patients who underwent DS due to STBI in our department. RESULTS: The mean GCS score was 4.96±1.20 at admission and 4 preoperatively. Mean ICP in non-surviving patients after DS was higher (p<0.05). ICP decrease after DS was also higher in surviving patients than in non-surviving patients (p<0.05). The overall mortality rate was 61.02%. A GCS motor score >2 at admission was associated with lower mortality (p<0.05). Four of the surviving patients (14.28%) had a functional outcome (Glasgow Outcome Score: 4 and 5) at one year after hospital discharge. CONCLUSION: Outcome in patients with BNDP after STBI may not always be fatal or poor. Rapid DS may increase the chance of functional survival, especially in patients with admission GCS score of 6 or 7.

Sutural diastasis caused by pseudotumor cerebri.

The diagnosis of pseudotumor cerebri is based on the triad of: (1) papilloedema, (2) elevated intracranial pressure with a normal cerebrospinal constituency and (3) normal central nervous system imaging studies. However, the diagnosis of pseudotumor cerebri is not always straightforward. We report a 19-year-old morbidly obese girl who developed pseudotumor cerebri associated with sutural diastasis of the cranium based on the direct radiographies. Following a ventriculo-peritoneal shunt operation, we demonstrated the closing and perisuturally sclerosis on all major cranial sutures. A careful examination of direct radiographies might be critical for diagnosis of pseudotumor cerebri. Prompt recognition, evaluation, and treatment are needed to prevent permanent visual loss.

Outcomes of intrathecal baclofen (ITB) therapy in spacticity.

AIM: Spasticity is associated with various neurological conditions. Intrathecal baclofen (ITB) is one of the popular treatments for severe spasticity. In this paper we present our experience in treating 30 patients with both spinal and supraspinal spasticity with chronic infusion of Baclofen to evaluate the long term efficacy of this treatment on spasticity, disability and pain, and to evaluate the side effects of intrathecal Baclofen. MATERIAL AND METHODS: The medical records of 30 patients who underwent baclofen pump placement from 2000 to 2010 under Department of Neurosurgery at the University of Akdeniz at Antalya/Turkey, were reviewed. All study subjects had diffuse chronic, severe, and generalized spasticity (Ashworth score ≥ 3), and had shown inadequate response to various oral antispastic drugs including baclofen. All patients were evaluated by means of the Ashworth score, spasm frequency, Barthel index, Rankin scales and VAS. RESULTS: Spasticity and spasm frequency and pain scores were clinically and statistically decreased in all patients. CONCLUSION: ITB therapy increases the quality of lifestyle and functional independence by reducing not only cerebral but also spinal related spasticity in appropriately selected cases.

Effect of early bilateral decompressive craniectomy on outcome for severe traumatic brain injury.

AIM: Debate continues as to whether decompressive craniectomy (DC) is an effective treatment for severe traumatic brain injury (STBI). DC is mostly used as a second tier treatment option. The aim of this study was determined whether early bilateral DC is effective as a first tier treatment option in patients with STBI. MATERIAL AND METHODS: The study compared two groups. Group 1 comprised 36 STBI patients for whom control of intracranial pressure (ICP) was not achieved with conservative treatment methods according to radiological and neurological findings. These patients underwent bilateral or unilateral DC as a second tier treatment. Group 2 comprised 40 STBI patients who underwent early bilateral DC as a first tier treatment. RESULTS: Group 2 patients had a mean better outcome than Group 1 patients especially for patients with a GCS 6-8. Postoperative ICP was lower in Group 2 patients than Group 1 patients. CONCLUSION: This study indicates that early bilateral DC can be effective for controlling ICP in STBI patients. It is likely the favorable outcome results for Group 2 patients reflects the relatively short time between trauma and surgery. Therefore, these data indicate early bilateral DC can be considered as a first tier treatment in STBI patients.

Multilevel primary intraspinal PNETs in an infant associated with hydrocephalus.

PNETs of the spinal cord are aggressive and local recurrence and/or leptomeningeal spread is common. Primary spinal PNETs are extremely rare and most cases involving the spinal cord are drop metastases from primary intracranial tumors by cerebrospinal fluid. Herewith, we present a 40-day-old infant with multilevel primary spinal PNET at Th12-L1 and L5-S1 levels associated with hydrocephalus occurring nearly 15 days after the operation. According to our knowledge this is probably the first case harboring all these pathologies. Multilevel primary intraspinal PNET in an infant is even rarer and can be associated with hydrocephalus that occurs during the postoperative period.

Spontaneous resolution of a large chronic subdural hematoma: a case report and review of the literature.

Spontaneous resolution of a chronic subdural hematoma has been reported rarely in the literature. We present herein the case of a 35-year-old patient with spontaneous resolution of a large chronic subdural hematoma, the volume of which was measured as approximately 76.5 ml on magnetic resonance imaging. No pathology was determined on neurological examination. Neither cerebral angiography nor EEG showed any abnormality. We discuss the possible relation between mechanisms of physio-pathogenesis and spontaneous resolution of chronic subdural hematomas, and also present the patient characteristics together with the other literature data.

Primary atypical teratoid/rhabdoid tumor of the clival region. Case report.

An atypical teratoid/rhabdoid tumor of the central nervous system (CNS) is a rare, aggressive neoplasm found in infants and children that has similar characteristics to CNS primitive neuroectodermal tumors/medulloblastomas. The authors present the case of a patient with an atypical teratoid/rhabdoid tumor and discuss the imaging, histopathological, immunohistochemical, and cytogenetic findings. Tumor cells displayed positive reactions for vimentin, epithelial membrane antigen, and cytokeratin, and they displayed no reaction for glial fibrillary acidic protein, desmin, and actin. The karyotype was 46, XY. The phenotype of an atypical teratoid/rhabdoid tumor appears heterogeneous when examined by histological, immunohistochemical, and genetic analysis. The authors describe the case of a 4-year-old boy who harbored an atypical teratoid/rhabdoid tumor in the clivus, which appeared as a chordoma on neuroimages. To their knowledge, this location of an atypical teratoid/rhabdoid tumor has not been described in the literature.

Modified experimental mild traumatic brain injury model.

BACKGROUND: Experimental models of traumatic brain injury (TBI), using a variety of techniques and species, have been devised with the aim of producing repeatable lesions resembling those found in head injuries. There are various TBI models mentioned in the literature. In experimental head trauma models, emphasis has been placed on the severe head injuries. There are only a few models developed to study mild traumatic brain injury (MTBI). In fact, MTBI is as important a problem as severe head injuries for neurosurgeons. METHODS: Fifty-six male Sprague-Dawley rats were subjected to MTBI with a weight-drop device, which was described by Marmarou et al. The said model was used in its original form as well as in modified forms by employing different weights dropped from the same height. Animals were divided into four groups of 14 rats as follows: Group I (n=14), head injury was induced using 450 g-1 m weight-height impact; Group II (n=14), head injury was induced using 350 g-l m weight-height impact; Group III (n=14), head injury was induced using 300 g-1 m weight-height impact; Group IV (n=14), control group, no injury was applied. Animals were evaluated neurologically, physiologically, electrophysiologically, and histopathologically. RESULTS: Group I and II animals (450 and 350 g-1m weight-height impact, respectively) showed the symptoms of severe head injury, whereas Group III animals (300 g-l m) showed more MTBI symptoms. CONCLUSION: We recommend the application of the modified MTBI model used for group III (300 g-l m weight-height impact) as the most appropriate and the simplest model for future MTBI studies.

Hydrocephalus after intraventricular hemorrhage in preterm and low-birth weight infants: analysis of associated risk factors for ventriculoperitoneal shunting.

BACKGROUND: Intraventricular hemorrhage and posthemorrhagic hydrocephalus are common causes of neonatal morbidity and mortality among preterm and low-birth weight infants (PT-LBWIs). Clinical management of posthemorrhagic hydrocephalus (PHH) is difficult and not well standardized. In this study, we aimed to determine the incidence of hydrocephalus after intraventricular hemorrhage (IVH) and the associated risk factors for ventriculoperitoneal (V-P) shunting in PT-LBWIs. We also aimed to identify the medical-care practices for these babies. METHODS: We reviewed the medical records of 42 babies with IVH diagnosed by cranial ultrasonography (classification of Papile et al, J Pediatr 1978;92:529-34). We compared 11 babies who required a V-P shunt with the 31 control subjects who did not require a V-P shunt or who died before discharge with respect to risk factors involved in V-P shunting. Maternal, perinatal, and neonatal risk factors, and therapies for IVH and PHH were studied as the V-P shunt-associated risk factors. RESULTS: The mean gestational age studied was 28.9 +/- 2.7 weeks, and the mean birth weight was 1164 +/- 391 g. This study revealed an incidence of 26% of PHH in PT-LBWIs with IVH. The most important risk factor for V-P shunt was found to be the severity of IVH (P < .05). Late gestational age and the time of IVH were found to be significant as well (P < .05). The length of hospitalization was found to be longer in patients with V-P shunt (P < .05). Therapies used for IVH and/or PHH were not significant as a risk factor for V-P shunt. In addition, the mortality rate was found to be 38% for all patients with IVH. CONCLUSION: Intraventricular hemorrhage in PT-LBWIs remains a significant problem, particularly when it is associated with PHH leading to long-term neurological impairment and decreased survival rate.

Role of decompressive surgery in the management of severe head injuries: prognostic factors and patient selection.

Decompressive surgery or craniectomy (DC) is a treatment option, which should be considered when the intracranial pressure (ICP) cannot be treated by conservative methods. The purpose of this study was to evaluate the benefits of decompressive craniectomy in patients with intractable posttraumatic intracranial hypertension and to evaluate the patient selection criteria for this management protocol. In this study, 100 patients with severe head injuries were involved. All patients were treated according to the European Brain Injury Consortium (EBIC) guidelines for severe head injuries and were assessed based on individual initial Glasgow Coma Scores (GCS), age, Glasgow Outcome Score (GOS), presence of systemic injury, changes in ICP, presence of mass lesion and the right timing for DC. All patients presented with a GCS of 8 or below. Based on their initial GCS, the patients were divided in two groups of 60 (group I with GCS 4-5) and 40 (group II with GCS 6-8) in each, respectively. Prognosis was evaluated according to the (GOS). After treatment with DC, 84 of the patients (84%) showed unfavorable and 16 (16%) showed favorable outcomes. In group I, 58 patients (96.6%) showed unfavorable and two (3.4%) showed favorable outcomes. In group II, 26 (65%) patients showed unfavorable and 14 (25%) showed favorable outcomes. The importance of initial GCS and age in patient outcomes were statistically significant. The presence of systemic injuries or mass lesions in outcomes were not statistically significant. Based on our findings, we conclude that patients with Glasgow Coma Scores of 6-8 are the best candidates for DC treatment.