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Inadequate embryonic fusion between the posterior sphenoid and the occipital bones may result in a transclival meningocele. Given the absence of a formal classification system for clival meningoceles and clear guidelines for the most appropriate endoscopic skull base surgical approach, we combined an anatomical study and a systematic review to characterize anatomical patterns, propose a preliminary classification, and discuss transoral and endonasal surgical corridors according to each clival subtype. In addition, we report the first known case of a primary clival meningocele successfully treated via a transoral endoscopic approach. The study was registered with PROSPERO (CRD42024575282) and received approval from the Ethics Committee (82878724.1.0000.5259). The systematic review followed PRISMA guidelines. PubMed\/MEDLINE, Embase, and Cochrane were rigorously searched. Anatomical dissection of midsagittal skull base specimens was performed to assess surgical access to clival regions. Seventeen studies comprising 18 patients (mean age 30.7\u00a0years; 72.2% female) were included. Clival defects were classified as posterior transsphenoidal (n\u2009=\u200911), spheno-occipital (n\u2009=\u20091), and basioccipital (n\u2009=\u20096). The endoscopic endonasal approach appeared more appropriate for posterior transsphenoidal cases and the transoral route for basioccipital defects. Anatomical dissection studies confirmed that the endonasal corridor is more suitable for the upper third of the clivus and the transoral route for the lower third.<\/jats:p>","DOI":"10.1007\/s10143-026-04435-2","type":"journal-article","created":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T03:21:28Z","timestamp":1786072888000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Congenital\/primary clival meningocele: A systematic review and surgical approach based on a new proposed classification"],"prefix":"10.1007","volume":"49","author":[{"given":"Pedro Henrique Costa","family":"Ferreira-Pinto","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dom\u00eanica","family":"Baroni Coelho de Oliveira Ferreira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elington","family":"Lannes Sim\u00f5es","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Corr\u00eaa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Othavio","family":"Gomes Lopes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco Aur\u00e9lio","family":"Rodrigues da Fonseca Passos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fl\u00e1vio","family":"Nigri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,8,7]]},"reference":[{"issue":"2","key":"4435_CR1","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1111\/j.1552-6569.2007.00187.x","volume":"18","author":"M Akyuz","year":"2008","unstructured":"Akyuz M, Arslan G, Gurkanlar D, Tuncer R (2008) CSF rhinorrhea from a transclival meningocele: a case report. 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The cadaveric specimens were obtained from the Department of Anatomy of the Instituto de Biologia Roberto Alc\u00e2ntara Gomes (IBRAG), in accordance with Article 2 of Brazilian Law No. 8,501 of November 30, 1992, which regulates the use of unclaimed bodies for scientific and educational purposes. The study protocol was reviewed and approved by the Research Ethics Committee of the Universidade do Estado do Rio de Janeiro (UERJ) via Plataforma Brasil (No: 82878724.1.0000.5259, date: September 16th 2024). All procedures complied with the principles of respect and dignity for human remains. Clinical trial number is not applicable.","order":1,"name":"Ethics","label":"Ethics approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Patient anonymity is preserved and written informed consent from the patient's legal guardian has been obtained.","order":2,"name":"Ethics","label":"Consent to participate","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors confirm that the patient\u2019s legal guardian provided informed consent for the publication of the case and the images in Figs.\u00a0\n                      \n                      and\n                      \n                      .","order":3,"name":"Ethics","label":"Consent to publish","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"None.","order":4,"name":"Ethics","label":"Competing interests","group":{"name":"EthicsHeading","label":"Declarations"}}],"article-number":"518"}}