Congenital basal encephaloceles involving functionally important structures

Close metadata

MATERIAL AND METHODS

We reviewed 43 articles (78 cases) devoted to basal encephaloceles involving functionally important structures. Analysis included transsphenoidal and sphenoethmoidal hernias.

RESULTS

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%.

CONCLUSION

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.

Keywords:

basal encephalocele

skull base

skull base surgery

sphenoethmoidal encephalocele

transsphenoidal encephalocele

Authors:

Chernikova N.A.

Burdenko Neurosurgical Center

ORCID: 0000-0002-4895-233X

Satanin L.A.

Burdenko Neurosurgical Center

ORCID: 0000-0003-2051-1855

Shelesko E.V.

Burdenko Neurosurgical Center

ORCID: 0000-0002-8249-9153

Danilov G.V.

Burdenko Neurosurgical Center

SPIN RSCI: 4140-8998
Scopus AuthorID: 56042615200
ResearcherID: P-6080-2016
ORCID: 0000-0003-1442-5993

Agrba S.B.

Burdenko Neurosurgical Center

ORCID: 0000-0003-1668-6409

Shkarubo A.N.

Burdenko Neurosurgical Center

ORCID: 0000-0003-3445-3115

Mazerkina N.A.

Burdenko Neurosurgical Center

ORCID: 0000-0002-0428-0498

Satanina M.L.

Pirogov Russian National Research Medical University

ORCID: 0009-0003-2747-8020

Received:

03.03.2024

Accepted:

21.06.2024

List of references:

Spacca B, Amasio ME, Giordano F, Mussa F, Busca G, Donati P, Genitori L. Surgical management of congenital median perisellar transsphenoidal encephaloceles with an extracranial approach: A series of 6 cases. Neurosurgery. 2009;65(6):1140-1145. https://doi.org/10.1227/01.NEU.0000351780.23357.F5
Modesti LM, Glasauer FE, Terplan KL. Sphenoethmoidal encephalocele. A case report with review of the literature. Childs Brain. 1977;3(3):140-153.
Pollock JA, Newton TH, Hoyt WF. Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology. 1968 Mar;90(3):442-453.
Kobets AJ, Redett RJ, Walsh JM, Lopez J, Guryildirim M, Cohen AR. Repair of giant anterior skull base encephalocele containing intralesional eloquent brain: Technical note Oper. Neurosurgery. 2021;21(2):73-80. https://doi.org/10.1093/ons/opab088
Formica F, Iannelli A, Paludetti G, Di Rocco C. Transsphenoidal meningoencephalocele. Child’s Nervous System. 2002;18(6-7):295-298. https://doi.org/10.1007/s00381-002-0578-z
Yang Z, Wang Z, Wang B, Liu P. Mechanism and surgical manage11ment of transsellar transsphenoidal encephalocele. Journal of Clinical Neuroscience. 2015;22(12):1916-1920. https://doi.org/10.1016/j.jocn.2015.03.066
Tsutsumi K, Asano T, Shigeno T, Matsui T, Ito S, Kaizu H. Transcranial approach for transsphenoidal encephalocele: Report of two cases. Surgical Neurology. 1999;51(3):252-257. https://doi.org/10.1016/s0090-3019(97)00221-8
Tan SH, Mun KS, Chandran PA, Manuel AM, Prepageran N, Waran V, Ganesan D. Combined transnasal and transoral endoscopic approach to a transsphenoidal encephalocele in an infant. Child’s Nervous System. 2015;31(7):1165-1169. https://doi.org/10.1007/s00381-015-2667-9
Saito K, Toda M, Sano K, Tomita T, Ogawa K, Yoshida K. Application of the endoscopic transsphenoidal approach to true type transsellar transsphenoidal meningoencephalocele in an adult: A case report and literature review. Acta Neurochirurgica. 2012;154(8):1511-1515. https://doi.org/10.1007/s00701-012-1346-9
Richardson S, Khandeparker RV, Raghuvaram AK, Mohan R. Modified two flap palatoplasty in asymptomatic transsphenoidal encephalocele: A case report. Journal of the Korean Association of Oral and Maxillofacial Surgeons. 2018;44(2):86-90. https://doi.org/10.5125/jkaoms.2018.44.2.86
Rice JF, Eggers DM. Basal transsphenoidal encephalocele: MR findings. Am J Neuroradiol. 1989;10(5 Suppl):S79.
Rathore YS, Sinha S, Mahapatra AK. Transsellar transsphenoidal encephalocele: A series of four cases. Neurology India. 2011;59(2):289-292. https://doi.org/10.4103/0028-3886.79157
Sharma RR, Mahapatra AK, Pawar SJ, Thomas C, Al-Ismaily M. Trans-sellar trans-sphenoidal encephaloceles: Report of two cases. Journal of Clinical Neuroscience. 2002;9(1):89-92. https://doi.org/10.1054/jocn.2001.0861
Pavanello M, Fiaschi P, Accogli A, Severino M, Tortora D, Piatelli G, Capra V. A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management. Neurological Sciences. 2021;42(12):5433-5439. https://doi.org/10.1007/s10072-021-05221-2
Ogiwara H, Morota N. Surgical treatment of transsphenoidal encephaloceles: Transpalatal versus combined transpalatal and transcranial approach. Clinical article. Journal of Neurosurgery: Pediatrics. 2013;11(5):505-510. PMID: 23495807. https://doi.org/10.3171/2013.2.PEDS12231
Nogueira JF, Stamm AC, Vellutini E, Santos FP. Endoscopic management of congenital meningo-encephalocele with nasal flaps. International Journal of Pediatric Otorhinolaryngology. 2009;73(1):133-137. https://doi.org/10.1016/j.ijporl.2008.09.013
Monteiro M, de Albuquerque AC, Nobre MC, Veloso AT, Mendes VA, de Souza Filho LD, da Silva de Magalhães MJ, Cardoso Bicalho GV. Meningoencefalocele transesfenoidal transpalatina. Arquivos de Neuro-Psiquiatria. 2006;64(3A):624-627. https://doi.org/10.1590/s0004-282x2006000400020
Acherman DS, Bosman DK, Horst CM. Sphenoethmoidal Encephalocele: A Case Report. The Cleft Palate Craniofacial Journal. 2003;40(3):329-333. PMID: 12733965. https://doi.org/10.1597/1545-1569_2003_040_0329_seacr_2.0.co_2
Smith DE, Murphy MJ, Hitchon PW, Babin RW, Abu-Yousef MM. Transsphenoidal encephaloceles. Surgical Neurology. 1983;20(6):471-480. https://doi.org/10.1016/0090-3019(83)90030-7
Lau BP, Newton TH. Sphenopharyngeal encephalo-meningoeoele. Radiologic Clinics. 1965;34(6):386-98.
Koerner JC, Sweeney J, Rheeman C, Kenning TJ. Delayed presentation of morning glory disc anomaly and transsphenoidal encephalocele: A management dilemma. Neuro-Ophthalmology. 2019;43(2):95-101. https://doi.org/10.1080/01658107.2018.1479434
Kai Y, Nagahiro S, Yoshioka S, Ushio Y. Application of the skull base technique to the repair of transsphenoidal meningoencephaloceles. Pediatric Neurosurgery. 1996;25(1):54-56. https://doi.org/10.1159/000121097
Kahyaoğlu O, Cavuşoğlu H, Müslüman AM, Kaya RA, Yilmaz A, Sahin Y, Dadaş B, Aydin Y. Transsellar transsphenoidal rhino-oral encephalocele: Case report. Turkish Neurosurgery. 2007;17(4):264-268.
Jabre A, Tabaddor R, Samaraweera R. Transsphenoidal meningoencephalocele in adults. Surgical Neurology. 2000;54(2):183-187. https://doi.org/10.1016/s0090-3019(00)00270-6
Hoff SR, Edwards MS, Bailey CM, Koltai PJ. The transpalatal approach to repair of congenital basal skull base cephaloceles. Journal of Neurological Surgery. 2014;75(2):96-103. https://doi.org/10.1055/s-0033-1358374
Faggin R, Pentimalli L, Grazzini M, Saetti R, Drigo P, d’Avella D. Combined endoscopic-microsurgical approach for transsphenoidal (sphenopalatine) encephalocele with an intralesional pituitary gland. Journal of Neurosurgery: Pediatrics. 2009;4(3):262-265. https://doi.org/10.3171/2009.4.PEDS0884
Eide JG, Walz P, Alden TD, Rastatter JC Pediatric skull base surgery: Encephaloceles and cerebrospinal fluid leaks. Operative Techniques in Otolaryngology — Head and Neck Surgery. 2019;30(1):30-36. https://doi.org/10.1016/j.otot.2019.01.012
Durham LH, Mackenzie IJ, Miles JB. Transphenoidal meningohydroencephalocoele. British Journal of Neurosurgery. 1988;2(3):407-409. https://doi.org/10.3109/02688698809001014
Coelho G, Rabelo NN, Varjão E, Marie T, Brito D, Del Massa EC, Zuppani H, Matos B, Yoshida M, Telles JPM, Teixeira MJ, Figueiredo EG. A hybrid simulation model for pre-operative planning of transsphenoidal encephalocele. Neurosurgical Review. 2021;44(3):1767-1774. https://doi.org/10.1007/s10143-020-01361-9
Chen G, Zhang Q, Ling F. An endoscopic endonasal approach for the surgical repair of transsphenoidal cephalocele in children. Journal of Clinical Neuroscience. 2011;18(5):723-724. https://doi.org/10.1016/j.jocn.2010.08.036
Brodsky MC, Hoyt WF, Hoyt CS, Miller NR, Lam BL. Atypical Retinochoroidal Coloboma in Patients With Dysplastic Optic Discs and Transsphenoidal Encephalocele: Report of Five Cases. Archives of ophthalmology. 1995;113(5):624-628. https://doi.org/10.1001/archopht.1995.01100050092036
Bayram Ö, Sebahat AY, Kadir A, Ali K. A rare cause of short stature: transsphenoidal encephalocele. European Journal of Pediatrics. 2014;173(12): 1611-1613. https://doi.org/10.1007/s00431-013-2175-4
Alshamrani A, Habalrih F, Altweijri I, Alsaleh S, Ajlan A. Endoscopic trans-nasal repair of basal encephalocele associated with morning glory syndrome. British Journal of Neurosurgery. 2022;36(1):108-110. https://doi.org/10.1080/02688697.2018.1494264
Abe T, Lüdecke DK, Wada A, Matsumoto K. Transsphenoidal cephaloceles in adults. A report of two cases and review of the literature. Acta Neurochirurgica. 2000;142(4):397-400. https://doi.org/10.1007/s007010050448
Barcelli OY, Durruti GP, Romero EP, Frías MM, Castellanos BR. Morning glory syndrome associated with transsphenoidal encephalocele and panhypopituitarism. Endocrinología y Nutrición. 2014;61(4):222-224. https://doi.org/10.1016/j.endonu.2013.11.003
Joy HM, Barker CS, Small JH, Armitage M. Trans-sphenoidal encephalocele in association with Dandy-Walker complex and cardiovascular anomalies. Neuroradiology. 2001;43(1):45-48. https://doi.org/10.1007/s002340000491
Chen CS, David D, Hanieh A. Morning glory syndrome and basal encephalocele. Child’s Nervous System. 2004;20(2):87-90. https://doi.org/10.1007/s00381-003-0869-z
Morioka M, Marubayashi T, Masumitsu T, Miura M, Ushio Y. Basal encephaloceles with morning glory syndrome, and progressive hormonal and visual disturbances: case report and review of the literature. Brain and Development. 1995;17(3):196-201. https://doi.org/10.1016/0387-7604(95)00021-3
Tuft SJ, Clemett RS. Dysplastic optic discs in association with transsphenoidal encephalocele and hypopituitary dwarfism: a case report. Australian and New Zealand Journal of Ophthalmology. 1983;11(4):309-313. https://doi.org/10.1111/j.1442-9071.1983.tb01098.x
Zeinalizadeh M, Sadrehosseini SM, Habibi Z, Nejat F, Silva HB, Singh H. Endonasal management of pediatric congenital transsphenoidal encephaloceles: Nuances of a modified reconstruction technique. Technical note and report of 3 cases. Journal of Neurosurgery: Pediatrics. 2017;19(3):312-318. https://doi.org/10.3171/2016.10.PEDS16270
Koral K, Geffner ME, Curran JG. Trans-sphenoidal and sphenoethmoidal encephalocele: Report of two cases and review of the literature. Australasian Radiology. 2000;44(2):220-224. https://doi.org/10.1046/j.1440-1673.2000.00788.x
Pradhan P, Phuyal S, Lamsal R, Agrawal P, Paudel R. Transsellar trans-sphenoidal encephalocele with cleft lip, cleft palate and agenesis of corpus callosum. Radiology Case Reports. 2021;16(8):2211-2213. https://doi.org/10.1016/j.radcr.2021.05.042
Mackel CE, Devaiah A, Holsapple J, Moore JM. Neural Crest Cell Failure as Embryogenesis for Fusiform Aneurysm of the Anterior Communicating Artery: Case Report and Review of the Literature. World Neurosurgery. 2019;129:232-236. https://doi.org/10.1016/j.wneu.2019.06.011
Koenig SB, Naidich TP, Lissner G. The Morning Glory Syndrome Associated with Sphenoidal Encephalocele. Ophthalmology. 1982;89(12):1368-1373. https://doi.org/10.1016/s0161-6420(82)34623-0

Close metadata