[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.

[Hemispherectomy in the treatment of pediatric symptomatic epilepsy of children]. / Gemisferektomiya v lechenii simptomaticheskoi epilepsii u detei.

INTRODUCTION AND PURPOSE: Hemispherectomy is a recognized option in the treatment of symptomatic forms of intractable focal epilepsy in patients with developmental brain malformations and some acquired lesions of one the hemispheres. The prognosis for an outcome of the technique is important in terms of the indications for surgical treatment. MATERIAL AND METHODS: We described the hemispherectomy technique and its variants and analyzed our own experience of surgery in 40 children. The most common (27 cases) brain pathology was extended unilateral cortical dysplasia with polymicro- or pachygyria and consequences of perinatal stroke. Six children had Rasmussen encephalitis; 6 patients had hemimegalencephaly; 1 child with Sturge-Weber syndrome had angiomatosis of the soft meninges. The patients' mean age was 3 years. Functional hemispherectomy (hemispherotomy) was used in most cases (37); 3 patients underwent anatomical hemispherectomy. RESULTS: At the time of discharge, seizures resolved in all patients; later, no seizure recurrence was observed in 25 out of 29 cases with known follow-up (the follow-up median was 2.5 years), which corresponded to class 1 outcomes on the ILAE scale (86%). Serious complications developed in 2 cases; 1 patient died; hydrocephalus and the need for bypass surgery occurred in other 2 children. These results are discussed along with the literature data, and the indications for hemispherectomy are provided. CONCLUSION: Hemispherectomy is a reliable and effective technique for treatment of symptomatic hemispheric forms of epilepsy in children. More than in 80% of patients with congenital or acquired pathology of one of the cerebral hemispheres, its deafferentation or resection leads to persistent elimination of seizures. Children with severe forms of intractable epilepsy should be promptly referred to dedicated centers to address the issue of advisability of surgical treatment.

[Blood loss in surgery of brain tumors in infants].

Functional immaturity of all organs and systems result in a high risk of complications in surgery of brain tumors in infants. One of the most serious complications is a massive blood loss and developing on its background severe disorders of hemostasis. In modern series of observations perioperative mortality in these children varies from 13 to 33%. The purpose of this paper--based on an analysis of topography, morphology and features of the operations to determine risk groups of blood loss in surgery of brain tumors in infants and suggest the best options for surgical tactics. When operating blood loss exceed 300% of the calculated blood volume persistent violations of coagulation homeostasis develop, which can lead to uncontrolled bleeding and death on the operating table, or post-operative bleeding. Intraoperative blood loss could be reduced by surgical techniques improvement, as well as by improving of anesthesia. First type includes preoperative embolization of the afferent vessels, careful planning of surgical approach, including using neuronavigation, deep stromal tumor coagulation during debulking, primary coagulation of main feeding blood vessels, two-stage surgery, and optimization of speed of tumor removal. All these methods has reduced the overall operational and post-operative mortality rate from 13 to 5%.

[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.

[Malignant hyperthermia in a child (case report)].

This case report describes the malignant hyperthermia (MH) accident in 12-year old boy undergoing maxillary-facial surgery: Crisis began 30 min after normal sevoflurane induction. Patient was successfully treated hence the MH exhibited abortive course. One should considerthis case as urgent reason for dantrolen registration in Russia, because it is not officially available yet. And it also demonstrates the necessity for more vigilant relation to the MH especially in pediatric anaesthesiology.

[Blood-sparing techniques in pediatric neuroanesthesiology].

The paper analyzes the clinical effectiveness of various blood-sparing techniques (hypervolemic hemodilution, isovolemic hemodilution, and instrumental infusion of washed autoerythrocytes) in neurosurgical interventions (n = 93) in 89 patients. Hypervolemic hemodilution that remains, however, to be the method of choice for young and low-weight children has proved to be the least clinically effective as a method for blood saving. The blood-sparing effectiveness of isovolmic hemodilution and instrumental reinfusion of washed autoerythrocytes is about the same, but the efficiency of reinfusion was higher with a blood loss volume of more than 100% of the proper volume of circulating complexes. A combination of hemodilution and instrumental reinfusion of autoerythrocytes provides a summation of blood-sparing effects for the mechanisms of blood saving vary with these techniques. The use of any blood-sparing technique accelerates and worsens hemostatic disorders.

[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.