neurosurgeryНейрохирургияRussian journal of neurosurgery1683-32952587-7569Издательский дом "МедИНК"10.17650/1683-3295-2024-26-1-99-109neurosurgery-1505Research ArticleНАБЛЮДЕНИЕ ИЗ ПРАКТИКИFROM PRACTICEПричины неудач хирургического лечения пациентов с врожденными базальными энцефалоцелеReasons for failure of surgical treatment of patients with congenital basal encephaloceleshttps://orcid.org/0000-0002-4895-233XЧерниковаН. А.ChernikovaN. A.

Надежда Алексеевна Черникова 

125047 Москва, ул. 4-я Тверская-Ямская, 16

Chernikhope@gmail.comhttps://orcid.org/0000-0003-2051-1855СатанинЛ. А.SataninL. A.

125047 Москва, ул. 4-я Тверская-Ямская, 16

https://orcid.org/0000-0002-8249-9153ШелескоЕ. В.SheleskoE. V.

125047 Москва, ул. 4-я Тверская-Ямская, 16

https://orcid.org/0000-0001-8079-8523ШугайС. В.ShugaiS. V.

125047 Москва, ул. 4-я Тверская-Ямская, 16

https://orcid.org/0000-0002-6520-4296КутинМ. А.KutinM.  A.

125047 Москва, ул. 4-я Тверская-Ямская, 16

https://orcid.org/0000-0002-9600-940XГоловинН. И.GolovinN. I.

125047 Москва, ул. 4-я Тверская-Ямская, 16

https://orcid.org/0000-0002-3243-129XСахаровА. В.SakharovA. V. 

125047 Москва, ул. 4-я Тверская-Ямская, 16

ФГАУ «Национальный медицинский исследовательский центр нейрохирургии им. акад. Н. Н. Бурденко» Минздрава РоссииРоссияN. N. Burdenko National Medical Research Center of Neurosurgery, Ministry of Health of RussiaRussian Federation20240504202426199‑109Copyright © Черникова Н.А., Сатанин Л.А., Шелеско Е.В., Шугай С.В., Кутин М.А., Головин Н.И., Сахаров А.В., 20242024Черникова Н.А., Сатанин Л.А., Шелеско Е.В., Шугай С.В., Кутин М.А., Головин Н.И., Сахаров А.В.Chernikova N.A., Satanin L.A., Shelesko E.V., Shugai S.V., Kutin M.A., Golovin N.I., Sakharov A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.therjn.com/jour/article/view/1505

Энцефалоцеле, или черепно‑мозговая грыжа, представляет собой заболевание, при котором происходит пролабирование мозговых оболочек и структур головного мозга через дефект черепа. Клинические проявления включают нарушение носового дыхания, деформацию назоэтмоидальной области, назальную ликворею. На фоне персистирования грыжи могут возникать различные воспалительные осложнения (менингит, менингоэнцефалит, вентрикулит, абсцесс головного мозга), летальность при этом составляет 8–10 %.

Базальное энцефалоцеле – редкая патология, требующая комплексного подхода к лечению в условиях специализированного стационара с применением высокотехнологичного оборудования. Лечебная тактика и риски определяются индивидуально с учетом возраста пациента, имеющихся симптомов, размеров полости носа, локализации и размеров костного дефекта. При отсутствии назальной ликвореи возможна отсрочка хирургического лечения для получения возможности использования аутокости свода черепа, забора надкостничного лоскута большего размера, выполнения операции комбинированным доступом, сведения к минимуму операционных осложнений. При эндоназальном эндоскопическом доступе необходимо отделить энцефалоцеле от окружающих тканей, полностью удалить грыжевой мешок и визуализировать костные края дефекта, затем выполнить его пластическое закрытие. Несмотря на то что существующие лечебные методы, как правило, эффективны, в ряде случаев не удается достичь желаемого результата. В статье представлены 2 редких клинических наблюдения, когда пациентам с базальным энцефалоцеле потребовалась повторная операция для устранения грыжи и пластики дефекта основания черепа.

Encephalocele or craniocerebral hernia is a disease in which there is a prolapse of the meninges and structures of the brain through a skull defect. Clinically, they are manifested by a violation of nasal breathing, deformation of the naso‑ethmoid region, and nasal liquorrhea. Various inflammatory complications (meningitis, meningoencephalitis, ventriculitis, brain abscess) can occur against the background of persistent hernia, while mortality is 8–10 %.

Basal encephalocele is a rare pathology that requires an integrated approach in a specialized hospital using high‑tech equipment. Therapeutic tactics and risks are determined individually based on the patient’s age, current symptoms, size of the nasal cavity, location and size of the skull base defect. In the absence of nasal liquorrhea, it is possible to delay surgical treatment in order to be able to use an autologous bone of the calvarium, to collect a larger periosteal flap, to perform the operation using a combined approach and to minimize surgical complications. With endonasal endoscopic access, it is necessary to separate the encephalocele from the surrounding tissues, completely remove the hernial sac and visualize the bone edges of the defect, and then perform its plastic closure.

Despite the fact that in most cases the existing methods of treatment are very effective, in a number of cases it is not possible to achieve the desired result. This article presents two rare clinical cases in which patients with basal encephalocele required reoperation for herniation and skull base defect repair.

базальное энцефалоцелечерепно‑мозговая грыжаназальная ликвореяполость носахирургическое лечениетрансплантатпластика костного дефектаbasal encephalocelecranial hernianasal liquorrheanasal cavitysurgical treatmentgraftbone defect repairReferencesLenze N.R., Gossett K.A., Farquhar D.R. et al. Outcomes of endoscopic versus open skull base surgery in pediatric patients. Laryngoscope 2021;131(5):996–1001. DOI: 10.1002/lary.29127Lenze N.R., Gossett K.A., Farquhar D.R. et al. Outcomes of endoscopic versus open skull base surgery in pediatric patients. Laryngoscope 2021;131(5):996–1001. DOI: 10.1002/lary.29127Suwanwela C., Suwanwela N. A morphological classification of sincipital encephalomeningoceles. J Neurosurg 1972;36(2):201– 11. DOI: 10.3171/jns.1972.36.2.0201Suwanwela C., Suwanwela N. A morphological classification of sincipital encephalomeningoceles. J Neurosurg 1972;36(2):201– 11. DOI: 10.3171/jns.1972.36.2.0201Suwanwela C. Geographical distribution of fronto-ethmoidal encephalomeningocele. Br J Prev Soc Med 1972;26(3):193–8. DOI: 10.1136/jech.26.3.193Suwanwela C. Geographical distribution of fronto-ethmoidal encephalomeningocele. Br J Prev Soc Med 1972;26(3):193–8. DOI: 10.1136/jech.26.3.193Okano S., Tanaka R., Okayama A. et al. Congenital basal meningoceles with different outcomes: a case series. J Med Case Rep 2017;11(1):359. DOI: 10.1186/s13256-017-1497-7Okano S., Tanaka R., Okayama A. et al. Congenital basal meningoceles with different outcomes: a case series. J Med Case Rep 2017;11(1):359. DOI: 10.1186/s13256-017-1497-7Bernal-Sprekelsen M., Alobid I., Mullol J. et al. Closure of cerebrospinal fluid leaks prevents ascending bacterial meningitis. Rhinology 2005;43(4):277–81. DOI: 10.1016/s0194-5998(03)01254-3Bernal-Sprekelsen M., Alobid I., Mullol J. et al. Closure of cerebrospinal fluid leaks prevents ascending bacterial meningitis. Rhinology 2005;43(4):277–81. DOI: 10.1016/s0194-5998(03)01254-3Черникова Н.А., Шелеско Е.В., Сатанин Л.А. и др. Эндоскопический эндоназальный метод в лечении детей с врожденными и приобретенными базальными менингоэнцефалоцеле. Фармакология & Фармакотерапия 2022;(спецвыпуск): 64–9. DOI: 10.46393/27132129_2022_S_64Chernikova N.A., Shelesko E.V., Satanin L.A. et al. Endoscopic endonasal method in the treatment of children with congenital and acquired basal meningoencephaloceles. Pharmacologiya & Pharmacoterapiya = Pharmacology & Pharmacotherapy 2022;(spesial issue):64–9. DOI: 10.46393/27132129_2022_S_64 (In Russ.).Adil E., Robson C., Perez-Atayde A. et al. Congenital nasal neuroglial heterotopia and encephaloceles: an update on current evaluation and management. Laryngoscope 2016;126(9):2161–7. DOI: 10.1002/lary.25864Adil E., Robson C., Perez-Atayde A. et al. Congenital nasal neuroglial heterotopia and encephaloceles: an update on current evaluation and management. Laryngoscope 2016;126(9):2161–7. DOI: 10.1002/lary.25864Parisi C., Saetti R., Petitto L.L. et al. Transethmoidal encephalocele endoscopically treated in a newborn: a case report. Childs Nerv Syst 2021;37(4):1333–7. DOI: 10.1007/s00381-020-04669-5Parisi C., Saetti R., Petitto L.L. et al. Transethmoidal encephalocele endoscopically treated in a newborn: a case report. Childs Nerv Syst 2021;37(4):1333–7. DOI: 10.1007/s00381-020-04669-5Koutourousiou M., Gardner P.A., Fernandez-Miranda J.C. et al. Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients. J Neurosurg 2013;119(5):1194–207. DOI: 10.3171/2013.6.JNS122259Koutourousiou M., Gardner P.A., Fernandez-Miranda J.C. et al. Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients. J Neurosurg 2013;119(5):1194–207. DOI: 10.3171/2013.6.JNS122259Schwartz T.H., Fraser J.F., Brown S. et al. Endoscopic cranial base surgery: сlassification of operative approaches. Neurosurgery 2008;62(5):991–1002. DOI: 10.1227/01.neu.0000325861.06832.06Schwartz T.H., Fraser J.F., Brown S. et al. Endoscopic cranial base surgery: сlassification of operative approaches. Neurosurgery 2008;62(5):991–1002. DOI: 10.1227/01.neu.0000325861.06832.06Higgins T.S., Thorp B., Rawlings B.A. et al. Outcome results of endoscopic vs craniofacial resection of sinonasal malignancies: a systematic review and pooled-data analysis. Int Forum Allergy Rhinol 2011;1(4):255–61. DOI: 10.1002/alr.20051Higgins T.S., Thorp B., Rawlings B.A. et al. Outcome results of endoscopic vs craniofacial resection of sinonasal malignancies: a systematic review and pooled-data analysis. Int Forum Allergy Rhinol 2011;1(4):255–61. DOI: 10.1002/alr.20051Peng A., Li Y., Xiao Z. et al. Exploration of endonasal endoscopic repair of pediatric cerebrospinal fluid rhinorrhea. Int J Pediatr Otorhinolaryngol 2011;75(3):308–15. DOI: 10.1016/j.ijporl.2010.11.016Peng A., Li Y., Xiao Z. et al. Exploration of endonasal endoscopic repair of pediatric cerebrospinal fluid rhinorrhea. Int J Pediatr Otorhinolaryngol 2011;75(3):308–15. DOI: 10.1016/j.ijporl.2010.11.016Keshri A.K., Shah S.R., Patadia S.D. et al. Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci 2016;11(1):42–5. DOI: 10.4103/1817-1745.181249Keshri A.K., Shah S.R., Patadia S.D. et al. Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci 2016;11(1):42–5. DOI: 10.4103/1817-1745.181249Di Rocco F., Couloigner V., Dastoli P. et al. Treatment of anterior skull base defects by a transnasal endoscopic approach in children: clinical article. J Neurosurg Pediatr 2010;6(5):459–63. DOI: 10.3171/2010.8.PEDS09325Di Rocco F., Couloigner V., Dastoli P. et al. Treatment of anterior skull base defects by a transnasal endoscopic approach in children: clinical article. J Neurosurg Pediatr 2010;6(5):459–63. DOI: 10.3171/2010.8.PEDS09325Lee J.A., Byun Y.J., Nguyen S.A. et al. Endonasal endoscopic surgery for pediatric anterior cranial fossa encephaloceles: a systematic review. Int J Pediatr Otorhinolaryngol 2020;132:109919. DOI: 10.1016/j.ijporl.2020.109919Lee J.A., Byun Y.J., Nguyen S.A. et al. Endonasal endoscopic surgery for pediatric anterior cranial fossa encephaloceles: a systematic review. Int J Pediatr Otorhinolaryngol 2020;132:109919. DOI: 10.1016/j.ijporl.2020.109919Tirumandas M., Sharma A., Gbenimacho I. et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst 2013;29(5):739–44. DOI: 10.1007/s00381-012-1998-zTirumandas M., Sharma A., Gbenimacho I. et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst 2013;29(5):739–44. DOI: 10.1007/s00381-012-1998-zElluru R.G. Congenital and acquired malformations of the nose and nasopharynx. Cummings Pediatric Otolaryngology 2021;64–76. DOI: 10.1016/b978-0-323-69618-0.00005-6Elluru R.G. Congenital and acquired malformations of the nose and nasopharynx. Cummings Pediatric Otolaryngology 2021;64–76. DOI: 10.1016/b978-0-323-69618-0.00005-6Xue L., Gehong D., Ying W. et al. Nasal meningoencephalocele: a retrospective study of clinicopathological features and diagnosis of 16 patients. Ann Diagn Pathol 2020;49:151594. DOI: 10.1016/j.anndiagpath.2020.151594Xue L., Gehong D., Ying W. et al. Nasal meningoencephalocele: a retrospective study of clinicopathological features and diagnosis of 16 patients. Ann Diagn Pathol 2020;49:151594. DOI: 10.1016/j.anndiagpath.2020.151594Makary C.A., Zalzal H.G., Ramadan J. et al. Endoscopic endonasal CSF rhinorrhea repair in children: systematic review with meta-analysis. Int J Pediatr Otorhinolaryngol 2020;134:110044. DOI: 10.1016/j.ijporl.2020.110044Makary C.A., Zalzal H.G., Ramadan J. et al. Endoscopic endonasal CSF rhinorrhea repair in children: systematic review with meta-analysis. Int J Pediatr Otorhinolaryngol 2020;134:110044. DOI: 10.1016/j.ijporl.2020.110044Ma J., Huang Q., Li X. et al. Endoscopic transnasal repair of cerebrospinal fluid leaks with and without an encephalocele in pediatric patients: from infants to children. Childs Nerv Syst 2015;31(9):1493–8. DOI: 10.1007/s00381-015-2746-yMa J., Huang Q., Li X. et al. Endoscopic transnasal repair of cerebrospinal fluid leaks with and without an encephalocele in pediatric patients: from infants to children. Childs Nerv Syst 2015;31(9):1493–8. DOI: 10.1007/s00381-015-2746-yThompson H.M., Schlosser R.J., McCarty Walsh E. et al. Current management of congenital anterior cranial base encephaloceles. Int J Pediatr Otorhinolaryngol 2020;131:109868. DOI: 10.1016/j.ijporl.2020.109868Thompson H.M., Schlosser R.J., McCarty Walsh E. et al. Current management of congenital anterior cranial base encephaloceles. Int J Pediatr Otorhinolaryngol 2020;131:109868. DOI: 10.1016/j.ijporl.2020.109868Mohindra Satyawati, Mohindra Sandeep, Mahendru S., Patil N.R. Endoscopic management of congenital nasal meningoencephaloceles: a short series of 15 paediatric cases. Childs Nerv Syst 2020;36(12):3059–62. DOI: 10.1007/s00381-020-04649-9Mohindra Satyawati, Mohindra Sandeep, Mahendru S., Patil N.R. Endoscopic management of congenital nasal meningoencephaloceles: a short series of 15 paediatric cases. Childs Nerv Syst 2020;36(12):3059–62. DOI: 10.1007/s00381-020-04649-9Gun R., Tosun F., Durmaz A. et al. Predictors of surgical approaches for the repair of anterior cranial base encephaloceles. Eur Arch Otorhinolaryngol 2013;270(4):1299–305. DOI: 10.1007/s00405-012-2174-5Gun R., Tosun F., Durmaz A. et al. Predictors of surgical approaches for the repair of anterior cranial base encephaloceles. Eur Arch Otorhinolaryngol 2013;270(4):1299–305. DOI: 10.1007/s00405-012-2174-5Woodworth B.A., Schlosser R.J., Faust R.A. et al. Evolutions in the management of congenital intranasal skull base defects. Arch Otolaryngol Head Neck Surg 2004;130(11):1283–8. DOI: 10.1001/archotol.130.11.1283Woodworth B.A., Schlosser R.J., Faust R.A. et al. Evolutions in the management of congenital intranasal skull base defects. Arch Otolaryngol Head Neck Surg 2004;130(11):1283–8. DOI: 10.1001/archotol.130.11.1283Sasani M., Ozer A.F., Aydin A.L. Endoscopic treatment of transsellar trans-sphenoidal encephalocele associated with morning glory syndrome presenting with non-traumatic cerebrospinal fluid rhinorrhea. J Neurosurg Sci 2009;53(1):31–5.Sasani M., Ozer A.F., Aydin A.L. Endoscopic treatment of transsellar trans-sphenoidal encephalocele associated with morning glory syndrome presenting with non-traumatic cerebrospinal fluid rhinorrhea. J Neurosurg Sci 2009;53(1):31–5.Oucheng N., Lauwers F., Gollogly J. et al. Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases – clinical article. J Neurosurg Pediatr 2010;6(6):541–9. DOI: 10.3171/2010.9.PEDS1043Oucheng N., Lauwers F., Gollogly J. et al. Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases – clinical article. J Neurosurg Pediatr 2010;6(6):541–9. DOI: 10.3171/2010.9.PEDS1043Сахаров А.В., Рогинский В.В., Капитанов Д.Н. и др. Современные методы диагностики и лечения детей с врожденными базальными черепно-мозговыми грыжами. Журнал «Вопросы нейрохирургии им. Н.Н. Бурденко» 2017;81(3):30–8. DOI: 10.17116/neiro201781330-38Sakharov A.V., Roginskiy V.V., Kapitanov D.N. et al. Modern diagnosis and treatment in children with congenital basal encephalocele. Zhurnal Voprosy neirokhirurgii im. N.N. Burden - ko = Burdenko’s Journal of Neurosurgery 2017;81(3):30–8. (In Russ.). DOI: 10.17116/neiro201781330-38Batra P.S., Kanowitz S.J., Citardi M.J. Clinical utility of intraoperative volume computed tomography scanner for endoscopic sinonasal and skull base procedures. Am J Rhinol 2008;22(5):511–5. DOI: 10.2500/ajr.2008.22.3216Batra P.S., Kanowitz S.J., Citardi M.J. Clinical utility of intraoperative volume computed tomography scanner for endoscopic sinonasal and skull base procedures. Am J Rhinol 2008;22(5):511–5. DOI: 10.2500/ajr.2008.22.3216Iida E., Anzai Y. Imaging of paranasal sinuses and anterior skull base and relevant anatomic aariations. Radiol Clin North Am 2017;55(1):31–52. DOI: 10.1016/j.rcl.2016.08.009Iida E., Anzai Y. Imaging of paranasal sinuses and anterior skull base and relevant anatomic aariations. Radiol Clin North Am 2017;55(1):31–52. DOI: 10.1016/j.rcl.2016.08.009Deopujari C.E., Shah N.J., Shaikh S.T. et al. Endonasal endoscopic skull base surgery in children. Childs Nerv Syst 2019;35(11): 2091–8. DOI: 10.1007/s00381-019-04167-3Deopujari C.E., Shah N.J., Shaikh S.T. et al. Endonasal endoscopic skull base surgery in children. Childs Nerv Syst 2019;35(11): 2091–8. DOI: 10.1007/s00381-019-04167-3Nogueira J.F. Jr, Stamm A.C., Vellutini E. et al. Endoscopic management of congenital meningo-encephalocele with nasal flaps. Int J Pediatr Otorhinolaryngol 2009;73(1):133–7. DOI: 10.1016/j.ijporl.2008.09.013Nogueira J.F. Jr, Stamm A.C., Vellutini E. et al. Endoscopic management of congenital meningo-encephalocele with nasal flaps. Int J Pediatr Otorhinolaryngol 2009;73(1):133–7. DOI: 10.1016/j.ijporl.2008.09.013Shah R.N., Surowitz J.B., Patel M.R. et al. Endoscopic pedicled nasoseptal flap reconstruction for pediatric skull base defects. Laryngoscope 2009;119(6):1067–75. DOI: 10.1002/lary.20216Shah R.N., Surowitz J.B., Patel M.R. et al. Endoscopic pedicled nasoseptal flap reconstruction for pediatric skull base defects. Laryngoscope 2009;119(6):1067–75. DOI: 10.1002/lary.20216Bassett E., Farag A., Iloreta A. et al. The extended nasoseptal flap for coverage of large cranial base defects. Int Forum Allergy Rhinol 2016;6(11):1113–6. DOI: 10.1002/alr.21778Bassett E., Farag A., Iloreta A. et al. The extended nasoseptal flap for coverage of large cranial base defects. Int Forum Allergy Rhinol 2016;6(11):1113–6. DOI: 10.1002/alr.21778Zanation A.M., Snyderman C.H., Carrau R.L. et al. Minimally invasive endoscopic pericranial flap: a new method for endonasal skull base reconstruction. Laryngoscope 2009;119(1): 13–8. DOI: 10.1002/lary.20022Zanation A.M., Snyderman C.H., Carrau R.L. et al. Minimally invasive endoscopic pericranial flap: a new method for endonasal skull base reconstruction. Laryngoscope 2009;119(1): 13–8. DOI: 10.1002/lary.20022Boetto J., Labidi M., Watanabe K. et al. Combined nasoseptal and inferior turbinate flap for reconstruction of large skull base defect after expanded endonasal approach: operative technique. Oper Neurosurg 2019;16(1):45–52. DOI: 10.1093/ons/opy046Boetto J., Labidi M., Watanabe K. et al. Combined nasoseptal and inferior turbinate flap for reconstruction of large skull base defect after expanded endonasal approach: operative technique. Oper Neurosurg 2019;16(1):45–52. DOI: 10.1093/ons/opy046

The authors declare that there are no conflicts of interest present.