Trans-sellar encephalocele is a rare subtype of basal encephalocele characterized by herniation of neural tissue through a defect in the sellar floor, extending into the sphenoid sinus or nasopharynx. Due to its deep anatomical location and proximity to critical neurovascular and endocrine structures, trans-sellar encephalocele presents significant diagnostic and surgical challenges. We report two adult cases of symptomatic trans-sellar encephalocele with progressive visual impairment, both successfully treated via endoscopic endonasal surgery. In addition, to contextualize these cases, we conducted a comprehensive literature review of 47 previously reported symptomatic trans-sellar encephalocele that was surgically treated in the neurosurgical field over the past quarter century, resulting in a total 49 patients (33 males, 16 females). Clinical characteristics, imaging findings, surgical approaches, and outcomes were analyzed. Of the 49 patients, the common symptoms included nasal obstruction (59.2%), endocrine dysfunction (46.9%), cerebrospinal fluid leakage (30.6%), and visual impairment (24.5%). The majority (69.4%) were diagnosed at age six or younger. Surgery was performed primarily via transnasal approaches (49.4%). Outcomes were available in 44 cases; 81.8% experienced symptom resolution or improvement. In our two cases, postoperative imaging confirmed the reduction of the trans-sellar encephalocele and cessation of cerebrospinal fluid inflow. Visual symptoms improved without recurrence or complications. Early diagnosis and tailored surgical intervention are essential for optimizing outcomes in trans-sellar encephalocele. The endoscopic endonasal approach enables safe and effective reduction of the trans-sellar encephalocele while preserving critical structures. Modifying of cerebrospinal fluid dynamics appears to play a key role in halting symptom progression. Long-term follow-up and further studies are warranted to establish standardized management strategies.

The combination of basal encephalocele with craniosynostosis is a rare clinical situation. Few observations of these two pathologies combination are described in the literature. Objective: to analyze own sample of patients to determine possible causes of encephalocele formation and surgical treatment strategy. Medical records of 4 patients with combination of encephalocele and craniosynostosis were analyzed. Data on complaints, medical history, results of medical specialists' examinations were studied. Data of CT and MRI, craniometric analysis were assessed. Obtained results were compared with literature data. Encephalocele development in patients with craniosynostosis was secondary and related to various causes such as traumas, iatrogenic injury, intracranial hypertension. The most significant factor in occurrence and development of nasal liquorrhea relapse is increased cerebrospinal fluid pressure. The treatment strategy for patients with basal encephalocele can be determined by the presence of intracranial hypertension and its increase after nasal liquorrhea elimination. One of the possible signs of long-lasting intracranial hypertension in children is a symptom of increased convolutional markings pattern according to the CT. If this sign is detected, it is justified to perform transcranial plastic surgery of cerebrospinal fluid-venous fistula with simultaneous reconstruction of the skull bones. Сочетание базального энцефалоцеле с краниосиностозом является редкой клинической ситуацией. В литературе описаны единичные наблюдения сочетания этих двух патологий. Анализ собственной серии пациентов для выяснения возможных причин формирования энцефалоцеле и определения тактики хирургического лечения. Был проведен анализ историй болезни 4 пациентов, у которых энцефалоцеле сочеталось с краниосиностозом. Были проанализированы данные о жалобах, анамнезе, результаты осмотров врачей-специалистов. Проводилась оценка данных КТ- и МРТ-исследований, краниометрический анализ. Полученные результаты сравнивались с данными литературы. Развитие энцефалоцеле у пациентов с краниосиностозами носило вторичный характер и было связано с различными причинами, такими как травмы, ятрогенное повреждение, внутричерепная гипертензия. Наиболее значимым фактором возникновения и развития рецидива назальной ликвореи является повышенное ликворное давление. Тактика лечения пациентов с базальным энцефалоцеле может определяться наличием внутричерепной гипертензии и ее усилением после устранения назальной ликвореи. Одним из возможных признаков длительно персистирующей внутричерепной гипертензии у детей является симптом усиления рисунка пальцевых вдавлений по данным КТ-исследования, при выявлении которого представляется оправданным выполнение транскраниальной пластики ликворной фистулы с одновременной реконструкцией костей черепа.

In rare cases, basal encephaloceles can include important brain structures (pituitary gland, chiasm, third ventricle walls, cerebral vessels). We reviewed 43 articles (78 cases) devoted to basal encephaloceles involving functionally important structures. Analysis included transsphenoidal and sphenoethmoidal hernias. Nasal breathing disorders were found in 76.9% of cases, decreased visual acuity - 53.8%, endocrine disorders - 44.9%, nasal CSF leakage - 14.1%, meningitis - 10.3%. Concomitant malformations occurred in 85.9%. Severe respiratory disorders (52.6%, p<0.05) and multiple concomitant malformations (71.7%, p<0.05) were more common among patients with sphenoethmoidal encephalocele. Among 78 patients, 73.1% underwent surgery while follow-up was preferable in 26.9% of cases. There was progression of endocrine disorders (9.5%), visual impairment (4.8%) and nasal breathing disorders (4.8%) in follow-up period. Surgical treatment also has many risks. Transcranial, transnasal, transoral and combined approaches are currently used. It was not possible to establish the most effective surgical approach due to heterogeneous clinical data. Postoperative complications occurred in 47.4% of cases (endocrine disorders - 33.3%, CSF leakage - 8.8%, visual impairment - 1.8%, infectious complications - 14%). Mortality rate was 3.5%. Congenital basal encephalocele involving functionally important structures is an extremely rare developmental defect. Such patients often have concomitant anomalies. Comprehensive examination is necessary to estimate the structures included in hernial sac and concomitant diseases. Treatment strategy should be determined individually after thorough examination and comparison of all risks. Surgical treatment is associated with high risk of complications due to damage to the structures comprising encephalocele. В редких случаях в состав базальных энцефалоцеле могут входить важные структуры головного мозга — гипофиз, хиазма, стенки III желудочка, сосуды головного мозга. Выполнен обзор 43 публикаций (78 наблюдений), посвященных базальным энцефалоцеле, включающим функционально важные структуры. В анализ вошли транссфеноидальные и сфеноэтмоидальные грыжи. В клинической картине отмечались нарушения носового дыхания — в 76,9% случаев, снижение остроты зрения — в 53,8% случаев, эндокринные нарушения — в 44,9% случаев, ликворея — в 14,1% случаев, менингиты — в 10,3% случаев. Сопутствующие пороки развития встречались в 85,9% случаев. При сфеноэтмоидальных энцефалоцеле чаще зафиксированы выраженные расстройства дыхания (52,6%, p<0,05), чаще отмечались множественные сопутствующие пороки развития (71,7%, p<0,05). Из 78 пациентов у 73,1% было выполнено хирургическое вмешательство, у 26,9% выбрана тактика динамического наблюдения. При динамическом наблюдении отмечались прогрессирование эндокринных нарушений (9,5%), нарушения зрения (4,8%), нарушения носового дыхания (4,8%). Хирургическое лечение также имеет множество рисков и является сложной задачей. На сегодняшний день применяются транскраниальный, трансназальный, трансоральный, комбинированные доступы. Не удалось установить наиболее эффективный хирургический доступ вследствие неоднородности клинических данных. Послеоперационные осложнения зарегистрированы в 47,4% наблюдений. В 33,3% случаев зафиксированы эндокринные нарушения, в 8,8% случаев — ликворея, в 1,8% случаев — нарушение зрения, в 14% случаев — инфекционные осложнения, которые в 3,5% случаев привели к летальному исходу. Врожденное базальное энцефалоцеле с включением функционально важных структур является крайне редким пороком развития. У таких пациентов часто имеются сопутствующие аномалии развития. Необходимо детальное обследование пациентов для определения структур, входящих в грыжевой мешок, и сопутствующей патологии. Вопрос о тактике лечения необходимо решать индивидуально после тщательного обследования и сопоставления всех рисков. Хирургическое лечение связано с высоким риском осложнений вследствие повреждения структур, входящих в состав энцефалоцеле.

Basal encephaloceles are the result of a concomitant cranial and dural defect that allows for inferior displacement of cerebral tissue, meninges, and cerebrospinal fluid into the paranasal sinuses and outside the cranial vault. This work illustrates a step-by-step surgical approach of a successful single-stage, endoscopic repair of a congenital basal encephalocele in a 10-year-old child, using a free mucosal middle turbinate graft that provided effective results without utilization of traditional open reconstructive techniques or vascularized flaps. A previously healthy 10-year-old male with a history of unilateral clear rhinorrhea was admitted as an inpatient because of an acute episode of nausea, vomiting, and confusion, accompanied by fever, diplopia, and bilateral abducens nerve palsies. Preoperative imaging revealed a 2-cm right-sided intranasal mass accompanied by a subcentimeter skull base defect spanning the lateral lamella. After completing a course of intravenous antibiotic therapy for 1 week after a negative lumbar puncture to ensure clearance of intracranial infection, the decision was made to proceed with definitive endoscopic skull base repair to obviate recurrent bacterial meningitis episodes and potential neurological complications. This study demonstrates technical feasibility of a single-stage endoscopic endonasal approach for pediatric basal encephalocele resection and repair which minimizes craniofacial morbidity associated with traditional open approaches and sinonasal morbidity associated with local pedicle-based flaps for small cranial base defects in this unique patient population.

To review surgical techniques used in the endoscopic transnasal repair of pediatric basal meningoencephaloceles and compare perioperative outcomes in children <2 and ≥2 years old. MEDLINE, EMBASE, and CENTRAL. Data sources were searched from inception to August 22, 2022, using search terms relevant to endoscopic transnasal meningoencephalocele repair in children. Reviews and Meta-analyses were excluded. Primary outcomes were the incidence of intraoperative and postoperative complications, including cerebrospinal fluid leak, recurrence, and reintervention. Quality assessments were performed using Newcastle-Ottawa Scale, ROBIN-I, and NIH. Overall, 217 patients across 61 studies were identified. The median age at surgery was 4 years (0-18 years). Fifty percent were female; 31% were <2 years. Most defects were meningoencephaloceles (56%), located transethmoidal (80%), and of congenital origin (83%). Seventy-five percent of repairs were multilayered. Children ≥2 years underwent multilayer repairs more frequently than those <2 years (P = 0.004). Children <2 years more frequently experienced postoperative cerebrospinal fluid leaks (P = 0.02), meningoencephalocele recurrence (P < 0.0001), and surgical reintervention (P = 0.005). Following multilayer repair, children <2 years were more likely to experience recurrence (P = 0.0001) and reintervention (P = 0.006). Younger children with basal meningoencephaloceles appear to be at greater risk of postoperative complications following endoscopic endonasal repair, although the quality of available evidence is weakened by incomplete reporting. In the absence of preoperative cerebrospinal fluid leak or meningitis, it may be preferable to delay surgery as access is more conducive to successful repair in older children.