[Epidemiology of pediatric intramedullary spinal cord tumors].

UNLABELLED: Intramedullary spinal cord tumors (IMSCTs) are a rare neurosurgical disease. There are a lot of controversies in their epidemiology. Most references were published in the 80-90ss. The authors describe and analyze the largest contemporary clinical series of IMSCTs treated by one surgeon (YK). MATERIAL AND METHODS: 201 patients (aged 2 months - 18 years) with IMSCT operated on during the past 12 years. RESULTS: A slight predominance of male patients was discovered. Astrocytomas account for 74% of all pediatric IMSCTs. Classic ependymomas are rare (5,5%). Pediatric spinal cord hemangioblastomas (HABs) are highly suspicious for von Hippel-Lindau disease. CONCLUSION: Benign histological variants of astrocytomas are the most common type of pediatric IMCTs. No gender or age differences in the incidence of most tumors (except for HABs) have been observed. Patients with intramedullary HAB should be routinely screened for von Hippel-Lindau disease. There are still a lot of uncertainties in the published data on epidemiology of IMSCTs.

[Primary and metastatic Ewing sarcoma of the skull base - case reports and comparative analysis].

UNLABELLED: The aim of the present study was to evaluate and compare diagnostic/and treatment modalities of primary and metastatic Ewing sarcoma (ES) of the skull base. MATERIAL AND METHODS: We analyzed nine cases of the skull base ES patients operated in Burdenko Neurosurgical Institute from 2003 to 2011. Among them there were five cases of primary ES, the other four were of metastatic origin. Clinical history, neuroimaging and pathology data together with IHC are presented. Treatment options and results are discussed. Seven patients were operated transcranially, in the other two cases endoscopic endonsnasal operations/were performed. Mean follow up was 11-92 months. RESULTS: We did not reveal arW pathological or IHC differences between primary and metastatic tumors. The labeling index Ki-67 was insignificantly higher in the metastatic tumors gro'Vip. In one case the patient developed relapse of the metastatic tumour in the temporal bone; he underwent second surgery and died 7 months after the operation. CONCLUSION: Despite both metastatic and primary ES of the skull base are of maMignant behavior, the long-term relatively good prognosis can be achieved when combined treatment, including surgery, chemotherapy and radiation is applied.

[Are the medulloepithelioma, ependymoblastoma and embryonal tumor with multilayered rosettes the same entity?].

Medulloepithelioma is a rare malignant tumor arising in cerebral hemispheres. Microscopically, medulloepithelioma is characterized by epithelial structures that mimic the embryonic neural tube. Immunohistochemical analysis revealed that tumor cells are immunopositive for LIN28A and fluorescence in situ hybridization showed an amplification of a miRNA cluster at 19q13.42. Presence of these both aberrations suggesting that medulloepithelioma, ependymoblastoma and embryonal tumor with multilayered rosettes are the same entity.

[A new entity in WHO classification of tumors of the central nervous system--embryonic tumor with abundant neuropil and true rosettes: case report and review of literature].

Embryonic tumor with abundant neuropil and true rosettes (ETANTR) is a very aggressive rare tumor with unique histologic and molecular features occurring in very young children. At present approximately 80 cases of ETANTR have been documented in the literature since first description in 2000. We report seven patients with ETANTR below 4 years of age who underwent surgical resection in the Burdenko Neurosurgery Institute between 2005 and 2010. Four children have received different modality chemotherapy and radiotherapy and two patients were treated by chemotherapy alone. One child did not receive any adjuvant treatment. All children had local relapses, two of them were operated twice. A 2 year old girl underwent subtotal resection thrice. Histological examination showed that all tumors were composed of true multilayered rosettes admixed with large areas of paucicellular neuropil. By analysis of recurrences we have found that large areas of neuropil and number of true rosettes were lost and tumors acquired a resemblance to central nervous system primitive neuroectodermal tumors. In four cases frozen tumor material was available for array-based comparative genomic hybridization, which discovered trisomy of chromosome 2 and amplification at the 19q13.42 chromosome locus. Fluorescence in situ hybridization revealed amplification at the 19q13.42 chromosome locus in all cases.

[Tethered cord syndrome in different types of spina bifida in children].

BACKGROUND: Tethered cord syndrome (TCS) occurs in 10-75% of children after correction of myelomeningocele and lipomyelomeningocele. Early diagnosis and surgical treatment of TCS after correction of different types of spina bifida remains a challenging problem of modern neurosurgery. MATERIALS AND METHODS: Since January till December 2008 in Burdenko Neurosurgical Institute 15 children were prospectively examined and operated. All of them had TCS after surgical treatment of spina bifida: myelomeningocele--7 (47%), lipomyelomeningocele--4 (27%), meningoradiculocele--1 (7%), meningocele--2 (13%), diastematomyelia--1 (7%). In the analyzed series peak of TCS manifestation was within 4 and 12 years. RESULTS: Clinical presentation of the disease consisted of neurological, urological, proctological and orthopaedic syndromes. In the postoperative period positive results were observed in most cases: regression of pain syndrome in 7 cases (87.5%), increase of muscle strength--in 4 (57%), improvement of sensory function--in 2 (33.3%), full or partial improvement of bladder and rectal function--in 4 (33.3%) and 5 (41.7%), respectively. Surgical complications occurred in 7 patients (46%): would CSF leak in 3 (20%), pseudomeningocele in 1 (6.7%), transient urinary retention in 2 (13.3%), wound insufficiency due to CSF leak in 1 (6.7%), motor deterioration in 1 (6.7%). CONCLUSION: Early surgical treatment of TCS in most cases leads to stabilization or improvement of neurological status. Risk of surgical complications associated with CSF leak indicates that optimal ways of their prevention should be developed.

[Intramedullary abscess of spinal cord as a complication of existing dermal sinus].

The paper describes a rare case of intramedullary spinal cord abscess in an infant after dermal sinus. In discussion with literature review the contemporary approach to patients with this pathology is highlighted.

[Effective therapy of acute cardiovascular and respiratory failure developed after removal of cervical spinal cord tumor in a child].

Development of hemodynamic and respiratory disturbances in cervical spinal cord injury is a known phenomenon. Its degree depends on severity of injury. Hemodynamic impairments include bradycardia and other arrhythmias, arterial hypotension due to decreased vascular tone and cardiac output and they may result in shock and asystolia. Pathogenesis of respiratory insufficiency is explained by decrease of vital and residual pulmonary volume, dysregulation of respiratory function, development of athelectases, changes in permeability of pulmonary capillaries, pneumonia. This leads to respiratory failure due to acute damage to lungs.

[Glomus tumors of the middle ear in children].

Glomus tumor (GT) is a rare tumor of the middle ear in children. In spite of cytologically benign nature of this tumor, its course can be characterized as malignant because of GT infiltrative growth, destruction of the adjacent bone tissue, possible intracranial involvement. Because of scare symptoms, early GT diagnosis is problematic. At admission to hospital principal complaints of the children are poor hearing, discharge from the ear. These often underlie misdiagnosis -- acute otitis media. GT of the middle ear are detected with application of computed tomography which is able to show changes in the soft tissue and bone destruction, and angiography. Combination of the above two methods allows assessment of location and advance of the pathological process. GT treatment planning depends on the examination data and can include radiotherapy, endovascular occlusion of the vessels carrying blood to the tumor, surgical treatment and combination of the methods. Modern approaches to GT diagnosis and treatment are described. A case of GT of the temporal bone with involvement of the middle cranial fossa in a 4-year-old child is reported.

[Laminoplasty for removal of disseminated intramedullar tumors in children].

The paper deals with the new application of the well-known surgical technique laminoplasty that is not commonly used to remove giant intramedullary tumors in pediatric patients. The basic stages of the operations and the nuances of their performance are described in the step-by-step fashion. Real photos of each stage are designed to furnish insights into the specific features of surgical techniques.

[Radical surgical tactics in the treatment of patients with intramedullary neoplasms inducing severe neurological deficit. What can we find in this?].

BACKGROUND: Radical surgery for intramedullary spinal cord tumors (IMSCT) is currently accepted as a first-line treatment. At the same time most papers concerning this problem show that surgical results depend on the preoperative neurological status of a patient. Many authors consider that the functional status of a patient with severe neurological deficit does not change for the better. MATERIALS AND METHODS: In 2002-2006, at the Academician N. N. Burdenko Research Institute of Neurosurgery, the author operated on 63 patients diagnosed as having IMSCT as evidenced by magnetic resonance imaging (MRI). Among them, there were 29 patients with severe preoperative neurological deficit (3-4 scores by the McCormick scale (MCS)). Thirteen (45%) tumors were totally removed; in the remaining patients with diffuse tumors, tumor removal was subtotal (more than 80% of a tumor being removed, as confirmed by MRI scans). There were neither neurological status deteriorations, nor fatal outcomes. Nineteen patients were found to have functional improvement by 1-3 MCS scores. Out of them, 15 (52%) patients became completely independent in their everyday activities due to surgery. CONCLUSIONS: In this series of patients with IMSCT and severe preoperative neurological deficit, tumor removal led to functional improvement in the patient and his independence in everyday life in 52% of cases. Radical surgery for localized IMSCT does not deteriorate the patients' functional status.

[Dorsal extradural arachnoid cyst at the Th(VII)-Th(X) level, causing chronic progressive myelopathy].

The paper describes a rare case of an extradural spinal arachnoid cyst in a 15-year-old boy, illustrates surgical treatment policy in detail, and reviews the current literature on this pathology.

[Osteoplastic laminotomy]. / Osteoplasticheskaia laminotomiia edinym blokom.

The paper describes the surgical technique of laminotomy. The basic stages of the operation and nuances of its performance are provided so that the readers can easily understand. Real photos of each surgical stage are useful for neurosurgeons dealing with intramedullary tumors.

[Dislocation and impaction of the brain after purgetive enema]. / Dislokatsiia i vklinenie mozga posle ochistitel'noi klizmy.

The paper describes a case of dislocation and impaction of the brain in a child with a large brain tumor, which developed after purgetive enema. It also discusses the management of patients with this complication and possible ways of its prevention.

[Correction of scaphocephaly by pi-plasty]. / Korrektsiia skafotsefalii metodom pi-plastiki.

The article contains a vivid description of a modern surgical technique used to correct the scaphocephalic cranial deformations, i.e. pi-plasty method. The surgery is described, in an easy-to-understand manner ("the way I do it"), from positioning the patient on the surgical table and making an incision to sewing up the skin. The real photos of each surgical stage facilitate, for the reader, the perception of surgical peculiarities.

[Transcutaneous external ventriculostomy (surgical technique)]. / Punktsionnaia naruzhnaia ventrikulostomiia (khirurgicheskaia tekhnika).

The paper deals with the placement of external ventricular drainage, one of the most common mini-invasive surgical techniques. The operation is described in the stepwise fashion. Photos of each of the surgical stages allow the reader to gain a deep insight into the specific features of the surgical technique. This technique can be useful particularly for emergency neurosurgeons.

[Use of the navigation system Stealth Station(TM) for removal of brain neoplasm]. / Ispol'zovanie navigatsionnoi sistemy Stealth Station(TM) dlia udaleniia opukholei golovnogo mozga.

The paper analyzes the authors' initial experience with the Stealth Station navigation system (Medtronic-Sofamor Danek, USA) during neurosurgery for intracranial tumors and outlines its techniques. Twenty eight patients with various brain tumors were operated on. Thirteen patients had tumors of the skull base (meningiomas, chordomas, paragangliomas, fibrous dysplasia). Twelve patients showed various intracerebral tumor-forming processes (gliomas, cavernous angiomas, and metastizing carcinomas). The navigation system ensured resection of these tumors. It was used in 2 other patients with gliomas and in 1 patient with cystic craniopharyngioma for their stereotactic puncture, biopsy, and drainage. The Stealth Station proved to be an effective tool for preoperative planning of cranial neurosurgical operations and their intraoperative guidance. Strict observance of the protocols of preoperative preparation ensures a high accuracy and facilitates search for and identification of anatomic guides. Only in 1 patient with surface glioma involving the central gyri, its resection was followed by hemiparesis that subsequently progressed. In the remaining 27 cases, the operation ran smoothly and caused no new neurological defect. The brain-shift phenomenon is the principal limitation of accuracy of the intraoperative system. This problem is less evident in skull-base surgery where the system was effectively used for critical structural location. Also, it can be safely employed for elimination of slight deep lesions to protect the brain and to minimize its injury via approach to a tumor through the brain parenchyma.