[Modern diagnosis and treatment in children with congenital basal encephalocele]. / Sovremennye metody diagnostiki i lecheniia detei s vrozhdennymi bazal'nymi cherepno-mozgovymi gryzhami.

Basal encephalocele is a rare disease that predominantly occurs in children. Its most common symptoms include nasal liquorrhea, difficulty in nasal breathing, and deformity of the naso-orbital region. MATERIAL AND METHODS: The study group included 19 patients with basal encephalocele, aged 2 months to 18 years. Ten (59%) patients were operated on through a transnasal endoscopic approach; 3 (17.5%) patients were operated on through a transcranial approach; 4 (23.5%) patients were operated on using a combined approach: the patients underwent simultaneous elimination of a cranio-orbital region deformity using the basal transcranial approach as well as hernial sac resection and hernioplasty using the transnasal endoscopic approach. Two children had no surgery due to minimal symptoms and a lack of cerebrospinal fluid leak. RESULTS: Application of the algorithms for diagnosis and treatment of encephalocele, suggested by the authors, enabled making the timely diagnose, defining the optimal surgical tactics, and achieving good treatment results. CONCLUSION: A differentiated approach to the choice of a surgical technique for basal encephalocele, the use of auto-tissues for skull base reconstruction, intraoperative and postoperative lumbar drainage, and simultaneous elimination of deformity of the fronto-naso-orbital region enable avoiding complications and achieving good functional and aesthetic results.

[Transient enlargement of craniopharyngioma cysts after stereotactic radiotherapy and radiosurgery]. / Tranzitornoe uvelichenie ob''ema kist kraniofaringiom posle stereotaksicheskoi luchevoi terapii i radiokhirurgii.

Stereotactic radiotherapy/radiosurgery (RT/ES) is an effective technique for treating craniopharyngiomas (CPs). However, enlargement of the cystic part of the tumor occurs in some cases after irradiation. The enlargement may be transient and not require treatment or be a true relapse requiring treatment. MATERIAL AND METHODS: In this study, we performed a retrospective analysis of 79 pediatric patients who underwent stereotactic RT or RS after resection of craniopharyngioma. RESULTS: Five-year relapse-free survival after complex treatment of CP was 86%. In the early period after irradiation, 3.5 months (2.7-9.4) on average, enlargement of the cystic component of the tumor was detected in 10 (12.7%) patients; in 9 (11.4%) of them, the enlargement was transient and did not require treatment; in one case, the patient underwent surgery due to reduced visual acuity. In 8 (10.1%) patients, an increase in the residual tumor (a solid component of the tumor in 2 cases and a cystic component of the tumor in 6 cases) occurred in the long-term period after irradiation - after 26.3 months (16.6-48.9) and did not decrease during follow-up in none of the cases, i.e. continued growth of the tumor was diagnosed. A statistical analysis revealed that differences in the terms of transient enlargement and true continued growth were statistically significant (p<0.01). CONCLUSION: Enlargement of a craniopharyngioma cyst in the early period (up to 1 year) after RT/RS is usually transient and does not require surgical treatment (except cases where worsening of neurological symptoms occurs, or occlusive hydrocephalus develops).

[A giant hyperostotic parasagittal meningioma in a child with neurofibromatosis type II (a case report and literature review)]. / Gigantskaya giperostoticheskaya parasagittal'naya meningioma u rebenka s neirofibromatozom II tipa (sluchai iz praktiki i obzor literatury).

Large parasagittal meningiomas, in particular hyperostotic ones, in children are rare and problematic in the differential diagnosis. The literature reports only single clinical cases related to this issue; opinions about the indications, surgical treatment options, and prognosis are contradictory. This paper presents a clinical case of a hyperostotic parasagittal meningioma with intra-extracranial growth in a 10-year-old boy with neurofibromatosis type II significantly worsening the prognosis. We discuss the epidemiological and clinical features of childhood meningiomas and issues of their diagnosis, treatment, and prognosis.

[A follow-up of patients with anterior optic tract glioma concurrent with type 1 neurofibromatosis].

Fifty-nine patients with anterior optic tract (AOT) lesion concurrent with type neurofibromatosis (NF-1) were followed up for 1 to 36 years (median 5 years). Based on the study, the authors identified several grades of the disease: - stabilization without intervention; - stabilization after treatment; - insignificant negative changes as a slight tumor growth and a further stabilization of the process; - occasionally negative changes irrespective of treatment. The behavior of a tumor is unpredictable. The onset of progression in infancy is a poor predictor. Ophthalmological symptoms are not the criterion that can be used to make a prognosis. The prognosis is largely determined by neurovisualization techniques and primarily magnetic resonance imaging. A uniform slight thickening of AOT structures irrespective of the extent of the process along the AOT should be referred to as a good prognosis. There was no spontaneous tumor regression in any case. By taking into account the beneficial effect of radiotherapy, the authors consider it expedient to use it as monotherapy and in combination with surgery. Bypass surgery on the spinal fluid system frequently fails to entirely solve the problem intracranial hypertension.

[Specific features of manifestation of anterior optic tract gliomas in patients with type 1 neurofibromatosis: ophthalmological and neurovisualizational aspects].

Gliomas of the anterior optic tract (AOT) (optic nerves, chiasms, and visual tracts) are benign slowly growing tumors usually detectable in childhood. These are a rather heterogenic group of tumors. The pattern and course of the disease are frequently affected by the presence of type 1 neurofibromatosis (NF-1) in the patient. The ophthalmological symptoms of AOT lesion in the presence of NF-1 were analyzed in 80 patients aged 1 to 24 years (median 5 years). According to the ophthalmological symptoms and the pattern and extent of lesions to AOT structures, the authors identified 4 groups by the data of neurovisualization techniques (magnetic resonance imaging, computed tomography) and revealed that the ophthalmological pattern reflected the site and degree of lesion to AOT structures. At the same time it is shown that there is a group of silent tumors attending without visual disorders. These tumors have the similar neurovisualization pattern, such as a moderate thickening of AOT structures and they occur in 13.8% of cases, as shown by the authors.

[Neuronal heterotopy].

The diagnosis of most volumetric processes of the brain presents no difficulty today; however, there are a number of brain diseases whose clinical manifestations are similar to those of tumors. Despite the development of neurovisualization techniques (application of currently available computer and magnetic resonance tomographs and special programs, such as MR imaging and CT spectroscopy, diffusion and perfusion, etc.), some of these processes are difficult to differentiate from neoplasms noninvasively. It is evident that treatment policy should be quite different in these or those cases. Neuronal nodular heterotopy (ectopy) may be considered as one of such pseudotumorous processes. This case illustrates the rare abnormality that has the clinical and X-ray picture similar to that of neoplasms of the brain. Current noninvasive method do not always allow one to significantly verify the nature of brain tissue changes, which shows it necessary to use CT-stereotactic biopsy in some cases.

[Giant cavernous malformation of the cerebellum in a baby].

The paper presents a rare case of successful removal of giant cavernous malformation of the cerebellum in a baby. It describes the specific features of differential diagnosis of space-occupying lesions of the posterior cranial fossa in infants in detail.

[Concurrence of Fahr's disease with cerebellar tumors]. / Sochetanie bolezni Fara s opukholiami mozzhechka.

Fara's disease or idiopathic calcification of the basal ganglia is a rare disease that is characterized by multiple petrificates in the area of the basal ganglia, caudate nucleus, and dentate nuclei of the cerebellum. As of now, only two cases of a concurrence of Fara's disease and brain tumors have been described. The authors present two more cases. Both cases are unique since the tumors occurred in the presence of Fara's disease symmetrically, as in the mirror, in the cerebellar hemispheres at the periphery of petrificates. This may be confirmed by the fact that astrocytic proliferation and hyperplasia around the calcified vessels are a cause of neoplasms.

[Single-photon emission-computed tomography of the brain in craniopharyngiomas in the late postoperative period]. / Odnofotonnaia émissionnaia komp'iuternaia tomografiia golovnogo mozga pri kraniofaringiomakh v otdalennom posleoperatsionnom periode.

The paper analyzes tomographic scanning images of 32 patients with craniopharyngiomas in the late postoperative period. Computed tomographic data allowed the patients to be divided into 3 groups: 1) 10 patients without signs of tumor recurrence or hydrocephalus; 2) 14 patients with recurrent cystic craniopharyngiomas; 3) 8 patients with severe hydrocephalus. Single photon emission computed tomography (SPECT) of the brain indicated regional disturbances of brain tissue blood supply in the frontobasal or frontobasotemporal regions of the right hemisphere (the area of an surgical access and of the removed tumor) and revealed them in the distal areas (frontobasotemporal regions of the left hemisphere, parietal and occipital cortices of the cerebral hemisphere or cerebellar tissue). The compensatory reserves of cerebral circulation were assessed by the foci of relative physiological hyperemia of brain tissue (the cerebellum and the medial portions of the occipital regions of the brain). The findings provide evidence for that the vascular factor is involved in the late postoperative pathological picture in patients with craniopharyngiomas.

[Endoscopic fenestration of median supratentorial cerebrospinal fluid cysts]. / Endoskopicheskaia fenestratsiia sredinykh supratentorial'nykh likvornykh kist.

Mid-supratentorial liquor cysts are a relatively rare and generally congenital abnormality of the cerebral ventricles and subdural spaces. The data and views available in the literature on rational surgical policy is contradictory. The authors' experience in treating 16 patients was used to consider whether endoscopic techniques can be employed for invasive fenestration of the cysts. The goal of surgery was to remove the masses caused by cystic malformations and their local compression of the brain via fenestration of the walls of the cysts and via communication of their cavities with the ventricles and cisterns. There were solitary cysts in all cases (arachnoidal cysts of the interpedicular cistern and the third ventricle in 9; cysts of the ventricular septum in 4, ependicular cysts of the lateral ventricle in 2, and cysts of the celiac plexus of the third ventricle in other 2 cases, in 1 cases a liquor cyst was located in the midbrain thickness). The clinical picture was characterized by a combination of hypertensive, hydrocephalic and focal symptoms of damages to the hypothalamic and thalamic structures and the adjacent formations of the brain (pyramidal and extrapyramidal disorders, ataxia, chiasmal syndrome, metabolic and endocrine disorders, etc.). In 6 cases these symptoms were persistent despite preimplanted VP anastomosis. Rigid Storz endoscopes (Germany) with an external coat, 6 mm in diameter, and a Codman fibroendoscope (USA), 4 mm in diameter, were employed. Cystic ventriculostomy and cystic ventriculocisternostomies were made in 11 and 6 patients, respectively; one patient underwent endoscopic resection of the walls of an ependymal cyst. In one patient with signs of decreased liquor resorption, endoscopic fenestration was concurrently developed into a ventricle-peritoneal anastomosis. In other 4 anastomosis-dependent patients, the preimplanted mechanically consistent bypass system was left at its site. In 2 of these cases, cystic ventriculostomy was supplemented by ventricular septal fenestration and third-ventricular bottom perforation. Twelve patients were followed up for 6 to 36.5 months (mean 15 months). There has been no information about 6 patients since their discharge. In 12 (66.5%) surgery yielded expected results and the fenestration of cystic walls was followed by their retraction and a steady-state regression of local and/or hypertensive symptoms. In 5 (28%) patients, the complaints and clinical data remained unchanged despite although incomplete but objective cystic relaxation. This was most frequently noted in patients (n = 4) with arachnoidal cysts of the interpedicular cistern and the third ventricle who had endocrine disorders. In one case the operation was stopped due to bleeding. Totally, 5 patients were found to have complications (hemorrhage, ventriculitis). None patient died. Some aspects of indications for endoscopy and surgical techniques are considered. It is concluded that endoscopic internal bypass surgery in patients wit median cystic liquor malformations is the treatment of choice. When equipment is adjusted, fenestration of the membranous walls of these cysts by using an endoscope is reliable and safe. Such patients may be recommended endoscopic technology used as the method of choice.

[Colloid cysts of the third ventricle]. / Kolloidnye kisty III zheludochka.

The authors analyze the results of surgical treatment of 51 consecutive patients with colloid cysts of the third ventricle. Patients were operated by the senior author from 1981 to 1996. Provided are the clinical signs, CT and MRI diagnostic criteria. Surgical treatment of colloid cysts is discussed in every detail. The authors believe, transcallosal approach with cyst enucleation via foramen of Monroe is the most adequate technique. No complications related to surgery were observed, except for transitory fixational amnesia, which completely regressed by 2-3 weeks postoperatively. The conclusion is that transcallosal approach with subsequent removal of the colloid cyst via the foramen of Monroe is a safe and reliable technique bringing no surgical complications.

Surgical management of hematomas of the brain stem.

Nine patients with brain-stem hematoma were admitted to the authors' institute during the period from 1985 to 1988. Clinical symptoms and signs pointed to pontine involvement in most cases. Progressive clinical deterioration was quite common and usually led to a clinical diagnosis of brain-stem intra-axial tumor. Angiography was noncontributory; computerized tomography (CT) was the main diagnostic test. This gave evidence of different pathological characteristics, including masses showing highly increased density, nonhomogeneous hyperdense lesions, and isodense lesions with peripheral contrast enhancement. No clear correlation was found, however, between the presumed duration of the clinical picture and the CT characteristics of the lesion. In the last four cases, magnetic resonance imaging was performed using a 0.015-tesla resistive system. This examination usually confirmed the presence of a brain-stem mass already shown by previous CT scans. There were, however, no cases in which direct evidence of an intra-axial vascular malformation could be obtained. The patients were all treated surgically with an attempt at total removal of the lesion and thorough inspection of the hematoma cavity and biopsy. Evidence of "cryptic" arteriovenous malformation was obtained in six of the nine cases. There were minor transitory complications in three cases. All of the patients were able to resume their previous activity, and none suffered recurrence of the symptoms following the operation. It may be concluded that surgery is the treatment of choice for brain-stem hematoma.

[The efficacy of gamma teletherapy in the combined treatment of medulloblastomas in the posterior cranial fossa of children]. / Effektivnost' distantsionnoi gamma-terapii pri kombinirovannom lechenii meduloblastom zadnei cherepnoi iamki u detei.

The results of radiation therapy conducted after various schemes were compared in 77 patients operated on for medulloblastoma of the posterior cranial fossa: 1. irradiation of the posterior cranial fossa area; 2. irradiation of this area and the entire length of the spinal marrow; 3. total irradiation of the CNS. Stages in a metastatic process necessitate irradiation in stages, i.e. irradiation of the posterior cranial fossa and the length of the spinal marrow followed by irradiation of the cerebral hemispheres one year after the operation. Total irradiation of the CNS shortly after the operation is recommended only in the presence of early metastases.

Venous and cerebrospinal fluid outflow in patients with brain swelling and oedema.

67 patients with benign intracranial hypertension (BIH) and 44 with normal pressure hydrocephalus (NPH) were examined by employment of infusion tests. Brain swelling (decrease of ventricular size with normal or increased brain tissue density) was a characteristic feature of BIH. It may result from venous outflow disturbances leading to vascular engorgement. But later, the process appears to be independent from the increase of the dural sinus pressure. This was normal in patients with BIH and NPH. Despite absorption disturbances there was a strong positive correlation in NPH between cerebrospinal fluid- and dural sinus pressure, while in BIH such a correlation was absent. The data confirm a pathogenesis of brain swelling in BIH as an obstacle to venous outflow at the level of the bridging veins and venous lacunae, however, not at the level of the dural sinuses.

[Computed tomography in the diagnosis of brain abscesses in children]. / Komp'iuternaia tomografiia v diagnostike abstsessov golovnogo mozga u detei.

The findings of computed tomography in 27 children aged from 4 months to 15 years with abscesses of the brain are analysed. Computed tomography proved to be an informative diagnostic method in determining the exact topography, size, and shape of the abscess. It allows judgement of the efficacy of nonoperative and surgical management of abscesses.

[Clinical picture and roentgenoradiological diagnosis of suprasellar cerebrospinal fluid cysts in children]. / Klinika i rentgenoradiologicheskaia diagnostika supraselliarnykh likvornykh kist u detei.

Suprasellar cysts containing cerebrospinal fluid are considered a rare pathological condition. Characteristic symptom complexes in children are discussed on the basis of 12 cases. It is assumed that signs of affection of the chiasma and optic nerves in slowly progressive hydrocephalus, diencephalic disorders, and hyperkinesis suggest the presence of a suprasellar cyst. The authors present data on the use of diagnostic methods of examination and give their opinion on the informative value of these methods: craniography, gamma-topography and radionuclide cisterno- and ventriculography, computerized tomography, and computerized-tomographic cisterno- and ventriculography.