BACKGROUND:Growing skull fracture (GSF) of the orbital roof is a rare complication of head injury in infancy and early childhood. Analysis of the Medline database between 1983 and 2023 identified 17 articles describing 63 children after surgical treatment of GSF of the orbital roof. MATERIAL AND METHODS:We present a 2-year-old child with moderate traumatic brain injury (TBI) complicated by GSF of the orbital roof. Neuroimaging and literature data are described. RESULTS:Severe ophthalmological symptoms in acute period of TBI subsequently partially regressed with persistent swelling of the upper eyelid and hypophthalmos. CT and MRI revealed a linear fracture of the frontal bone extending to superior orbital wall and intraorbital collection of cerebrospinal fluid (pseudomeningocele) mixed with blood. MR signs of GSF of the orbital roof with pseudomeningocele shrinkage were found in 5 weeks after injury. After 6 months, CT and MRI revealed signs of more severe GSF. Surgical treatment implied craniotomy, excision of scars, arachnoid membrane and abnormal brain matter, resection of deformed fracture edges, dura mater sealing and orbital roof reconstruction with bone autograft. Ophthalmological symptoms regressed after 6 months. The follow-up period was 6 years. There were no signs of fracture recurrence. CONCLUSION:In this case, acute intraorbital pseudomeningocele led to GSF of the orbital roof in 5 weeks after injury. Incomplete regression of ophthalmological symptoms, their aggravation or delayed appearance in children with orbital roof fracture requires a thorough examination including high-resolution CT and MRI, and orbital roof GSF should be considered in the differential diagnosis.

Primary intraocular lymphomas (PIOL) affecting the vitreoretinal complex is a rare nosology, and because of that PIOLs often cause diagnostic difficulties and/or lead to misdiagnosis. In the event of retinal lesions, in addition to routine ophthalmoscopy, optical coherence tomography (OCT) of the retina plays an important role in the diagnosis of the disease. PURPOSE Evaluation of the characteristic features of retinal lymphomas using OCT. MATERIAL AND METHODS The study included 6 patients (10 eyes) with retinal lymphomas associated with brain lesions of diffuse large B-cell lymphoma (DLBCL) who were treated at the N.N. Burdenko National Medical Research Center of Neurosurgery from 2017 to 2020; they were examined with OCT. RESULTS All patients with retinal lymphomas showed typical OCT signs in the form of hyperreflective subretinal infiltrates. CONCLUSION OCT is a modern non-invasive method that allows diagnosing retinal lymphomas based on clinical and instrumental signs with a high degree of reliability.

Papilledema (choked disk) is a sign of intracranial hypertension (ICH) - condition that presents danger not only for patient's vision, but also for their life. Despite the fact that ICH is usually a neurosurgical pathology, sometimes an ophthalmologist is the first doctor such patients visit, most often in a primary healthcare clinic. At the same time, as practice shows, not all ophthalmologists are well aware about in this pathology; difficulties occur in differential diagnosis of papilledema against similar changes of the optic nerve head seen during ophthalmoscopic examination. This article reviews scientific literature on ICH, including benign ICH, diagnosis and differential diagnosis of papilledema. The authors also share their decades-long experience of working in a neurosurgical facility.

Optic nerve sheath meningioma (ONSM) is a slow-growing benign tumor that tends to extend intracranially. The tumor is characterized by vision disturbance, optic nerve atrophy and edema. At present, radiotherapy is recommended as the treatment of choice for ONSM.PURPOSE:To assess the neuro-ophthalmic symptomatology of ONSM and how it changes in response to radiotherapy.MATERIAL AND METHODS:In the period from 2004 to 2018, 112 patients with ONSM aged 17 to 76 years underwent stereotactic radiotherapy. In 91 patients, visual functions varied from light perception to 1.0; in 21 patients the affected eye was blind. The prospective follow-up of 103 patients lasted 6 months to 10 years (mean follow-up duration was 57 months). Studied patients underwent either conventional fractionated radiotherapy with Novalis (n=88) or hypofractionation radiotherapy with CyberKnife (n=24).RESULTS:According to ophthalmological examination, in 60.5% of cases an improvement of visual functions was observed, 39.5% of patients had visual functions remain stable at the initial level. No vision impairment during irradiation was detected. Vision deterioration in the long-term follow-up was observed in 2 patients: extended tumor growth in one case and radio-induced retinopathy in the other.CONCLUSION:The study showed that this method of treatment for ONSM is effective and safe, allowing preservation of visual functions and in some cases - its improvement, while tumor growth remains under control.

Optic nerve glioma is a rather rare tumor. It predominantly arises in pediatric patients, including those with type I neurofibromatosis. This neoplasm is accompanied by decreased visual function and exophthalmos. Treatment strategy is individualized depending on age, volume and spread of tumor, as well as severity of clinical manifestations. Possible treatment options are surgical resection, chemotherapy, radiotherapy and their combination. Radiotherapy can be recommended for patients with intact visual functions, no severe proptosis and trophic lesions. Classic fractionation mode is used as a standard. Currently, the possibility of hypofractionated irradiation is being considered.OBJECTIVE:To evaluate safety and efficacy of hypofractionated radiotherapy in patients with optic nerve glioma.MATERIAL AND METHODS:Sixteen patients with optic nerve gliomas underwent hypofractionated stereotactic irradiation (CyberKnife) between May 2014 and October 2019. Single focal dose was 5.5 Gy. There were 5 fractions up to total focal dose of 27.5 Gy. The sample enrolled 14 children with a median age of 4 years (range 23 months - 13 years) and 2 adults aged 47 and 66 years, respectively. Median of tumor volume was 2.77 cm3 (range 1.69-10.01 cm3).RESULTS:Tumor growth control was achieved in all patients, partial remission was observed in 5 (32%) patients. None patient had deterioration of visual function. Improvement of visual acuity was noted in 3 (19%) cases. Visual field enlargement occurred in 4 (67%) out of 6 patients who were preoperatively examined. After irradiation, proptosis decreased by ≥ 1 mm in 9 (60%) out of 15 patients.

Работа посвящена изучению одной из самых сложных для лечения групп опухолей основания черепа — срединных менингиом передних отделов основания черепа, распространяющихся в полость носа и околоносовые пазухи, а также в ряде случаев в глазницу (срединные краниофациальные менингиомы). Вопрос об оптимальной тактике лечения таких опухолей остается предметом дискуссий. В современной литературе отсутствует сформулированная концепция подходов к хирургическому и комбинированному лечению срединных краниофациальных менингиом. ЦЕЛЬ ИССЛЕДОВАНИЯ Анализ результатов одноцентрового исследования, включающего пациентов с доброкачественными (WHO grade I/II) срединными краниофациальными менингиомами, которые прошли хирургическое или комбинированное лечение, а также разработка рекомендаций по тактике лечения на основании этого анализа. МАТЕРИАЛ И МЕТОДЫ Проанализированы данные 125 пациентов (89 женщин и 36 мужчин), прошедших хирургическое лечение. Катамнестические данные доступны у 90 пациентов. Из них хирургическое лечение было проведено у 53 пациентов, комбинированное — у 37. Для хирургического удаления применяли субфронтальные доступы и доступ через лобную пазуху (50,4%), эндоскопический эндоназальный (16,8%) и др. В большинстве случаев облучение проводили в режиме стереотаксической лучевой терапии со стандартным фракционированием. Выполнен анализ результатов и исходов лечения пациентов в раннем и отдаленном послеоперационном периодах. РЕЗУЛЬТАТЫ Всего было удалено радикально 34 (27,2%) опухоли, интракраниальный узел был удален у 29 (23,2%) пациентов, экстракраниальный — у 48 (38,4%), оба узла без резекции основания черепа — у 14 (11,2%) пациентов. Улучшение отмечено у 79 (63,2%) пациентов, изменений в состоянии у 29 (27,2%) не наблюдали, ухудшение было зафиксировано у 11 (8,8%) пациентов, один (0,8%) больной скончался. Послеоперационные осложнения (58 случаев) были зафиксированы у 32 (25,6%) пациентов. Контроль роста был достигнут в 65,5% наблюдений, прогрессирование опухоли — в 23,3%. Летальные исходы были зафиксированы в 10 (11,1%) случаях. Поздние осложнения (23 случая) развились у 15 (16,7%) пациентов. ЗАКЛЮЧЕНИЕ Результаты исследования показали, что анализируемая группа пациентов представляет особую сложность в связи с высокой частотой осложнений и рецидивирования, развивающихся после лечения у этой когорты. Все это обусловливает необходимость выявления факторов, влияющих на прогноз, и создания алгоритмов лечения на основе анализа полученных данных.

The authors report a patient with recurrent bifocal germinoma of the optical nerves and chiasm after previous combined treatment. The tumor resulted progressive visual acuity loss despite subsequent therapy (glucocorticoid therapy, chemo- and radiotherapy). Differential diagnosis between tumor progression and consequences of radiotherapy was complicated by MRI negative pattern. Subsequent development of the process, signs of anterior visual pathway damage and tumor spread throughout the ventricular system and subarachnoid spaces according to neuroimaging data indicated recurrence of disease.

Diencephalic cachexia (DC ) is a metabolic disorder characterized by a decrease in body weight. DC usually occurs in the presence of glioma brain tumors extended into the optic pathway. These tumors are very aggressive and have poor prognosis.Objective: to analyze the clinical course of optic pathway gliomas (OPG s) in patients with and without DC .Material and Methods. The study included 264 patients aged 0 to 18 years with an initial diagnosis of OPG s registered in the N.N. Burdenko National Medical Research Center of neurosurgery from 01/01/2003 to 12/31/2015. Patients were divided into two groups: without DC (204 people) and with DC (60 children). Results: neurofibromatosis type I (NFI) was much more common in children without DC , and pilomyxoid histology was much more prevalent in children with DC . Five-year overall survival (OS ) and event-free survival EFS were significantly lower in children with DC than in children without DC (82 ± 5 % and 96 ± 1 %, respectively versus 37 ± 7 % and 62 ± 3 %, respectively). It was found that in the DC group, the OS and EFS rates were significantly lower in girls, in children without NFI, in children without histological verification and in children with pilocytic astrocytomas. It was also found that in the DC group, OS rates were significantly lower in children under 1 year, and EFS rates were significantly lower in children aged more than 12 months. The number of patients without events were significantly higher in the group without DC (p=0.001). The number of deaths in the postoperative period was significantly higher in children with DC (p<0.001). Diabetes insipidus and hyponatremia were significantly more common in patients with diencephalic cachexia, and vision improvement after treatment was significantly more likely to occur in patients without DC .Conclusion. OPG s in patients with DC have a more aggressive clinical course, which requires more careful treatment and observation.

The study objective is to assess the outcomes after stereotactic radiosurgical treatment (SRS) in patients with pilocytic astrocytomas after non-radical surgery and after continued tumor growth.Materials and methods. The report includes 56 patients (37 males and 19 females) who have undergone SRS in N. N. Burdenko National Medical Research Center of Neurosurgery from March 2005 to January 2018.Results. The majority of patients (75 %) were children. Almost 43 % of patients underwent SRS as part of the primary treatment after biopsy or incomplete removal, other patients – in the event of continued tumor growth after non-total surgery. Tumors involved the cerebellum (41 %), brainstem (23 %), thalamus (19.6 %) and cerebral hemispheres (16.1 %). The median tumor volume was 1.9 cm3 (0.14–19.00 cm3), 23 % of patients had cysts in the tumor. The prescribed dose was 12 to 22 Gy (median 18 Gy) over 50–80 % isodose line. The follow-up was available for 54 (96.4 %) patients. The median follow-up was 67 months (3–151 months). All patients were alive at the end of the follow-up examination. In 14 (25 %) patients, the development of pseudo-progression (PSP) was noted. The median detection of PSP is 11 months (3–65 months). Of these, in 7 (50 %) patients PSP was accompanied by clinical deterioration: in 5 – an increase in general cerebral symptoms and in 1 patient – an episode and an increase in cerebellar symptoms. Eight (57 %) patients with PSP were reoperated: 4 underwent removal of the tumor, 4 – emptying of the cyst and placement of the Ommaya reservoir, which led to regression of the existing symptoms. No other toxicity was observed in patients. At the time of the completion of the follow-up examination (with a given median follow-up), no relapses were detected. There was no clinical deterioration after SRS.Conclusion. Radiosurgical treatment is an effective and safe method of radiation treatment for patients with primary pilocytic astrocytomas and recurrent pilocytic astrocytomas, providing control over tumor growth in all patients with a low risk of complications.

Glioma metastasis outside the central nervous system is a quite rare phenomenon. The disease in a young woman manifested itself as back pain and loss of vision in the left eye. Magnetic resonance imaging (MRI) revealed a tumor of the optic nerve; positron emission tomography showed multiple secondary bone changes. At the same time, MRI detected no signs of neoplasm in the midline brain structures (the brain stem and subcortical nuclei) and spinal cord. Two biopsies (superior iliac spine trephine biopsy and optic nerve tumor biopsy) were performed. There were similar histological tumors; the optic nerve tumor was found to have K27M mutation in the H3F3A gene, whereas the metastatic tumor lacked this mutation (possibly due to the quality and quantity of DNA isolated from the tumor cells). The interesting features of this case are the simultaneous detection of primary and metastatic tumors before receiving any treatment and the absence of the K27M mutation in the H3F3A gene in the metastasis.

OBJECTIVE The analyze the neuro-ophthalmic outcomes in patients with pineal and suprasellar germinoma after complex treatment. MATERIAL AND METHODS There were 125 patients (88 males, 37 females) with primary CNS germinoma for the period 2008-2017. All patients were divided into 3 groups depending on the tumor localization: pineal area - 62 patients, suprasellar neoplasm - 38 patients, bifocal germinoma - 25 patients. Treatment was performed in accordance with the «Germinoma-2008» protocol. Tumors were histologically patients (partial - 32 cases, subtotal - 16 cases). Surgery was followed by postoperative chemotherapy and stereotactic radiotherapy. The results were evaluated by neuro-ophthalmological examination at all stages of complex treatment. RESULTS Pineal germinoma resulted oculomotor and pupillary disorders (58 patients - 94%) and papilledema. The last one completely regressed under the treatment. Partial reduction of oculomotor and pupillary abnormalities occurred throughout chemotherapy (6 patients - 10%). More than half of patients (n=24, 63%) with suprasellar germinoma had visual impairment. Improvement of visual functions including severe visual disturbance was observed in 14 patients (58%). Predominantly, functional recovery occurred under chemotherapy (12 patients - 86%). Bifocal germinoma was followed by various combinations of oculomotor, pupillary and/or visual impairments. We observed regression of only mild visual disorders. Stereotactic radiotherapy did not ensure any changes in neuro-ophthalmic symptoms in all groups.

BACKGROUND:Optical coherence tomography (OCT) gives the opportunity to examine retrograde degeneration of visual pathway damaged at various levels.OBJECTIVE:To estimate OCT data on retrograde degeneration of visual pathway damaged at various levels.MATERIAL AND METHODS:Ganglion cell layer (GCL) thickness was measured by OCT in 79 patients with visual pathway damaged at various levels and known duration of visual disturbances. Twenty-One patients were diagnosed with traumatic lesions of the optic nerves and/or chiasma. Fifty-eight patients had retro-genicular visual pathway damage. Thirty-three patients were examined for postoperative homonymous hemianopia after surgery for drug-resistant temporal lobe epilepsy. Twenty-five patients were diagnosed with occipital lobe damage following stroke (12 patients), surgery for arteriovenous malformation (11 patients) and traumatic brain injury (2 patients). All patients underwent assessment of visual acuity, automatic static perimetry, MRI/CT of the brain. Retinal ganglion cell complex was analyzed during OCT.RESULTS:GCL thinning following anterior visual pathway damage was detected in 20 out of 21 patients after ≥22 days. In case of post-genicular visual pathway damage, GCL thinning was found in 25 out of 58 patients (9 out of 33 ones after surgery for temporal lobe epilepsy and 16 out of 25 patients with occipital lobe lesion). After surgery for temporal lobe epilepsy, minimum period until GCL thinning detection after previous visual pathway damage was 3 months, in case of occipital lobe lesion - 5 months.CONCLUSION:Retrograde visual pathway degeneration is followed by GCL thinning and depends on the level of visual pathway lesion.