Circular configuration of Torcular Herophili presenting as childhood headache | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Circular configuration of Torcular Herophili presenting as childhood headache Debajyoti Datta, Arunkumar Sekar This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-2394561/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract The torcular herophili, also known as the confluence of the sinuses, has a highly variable anatomy and up to 9 different types have been reported in the literature. We describe a case of a circular configuration of the torcular herophili in a child who presented with headache in absence of other abnormalities. The torcular herophili embryologically develops from the anterior and middle dural plexus.The anterior dural plexus is joined posteriorly by the middle dural plexus forming the junctional area of the drainage of superior sagittal sinus, straight sinus and bilateral transverse sinus, thereby forming the primitive torcular herophili 2 . We propose that the circular configuration of the torcular herophili may cause episodes of headache due to raised intracranial pressure secondary to venous congestion caused by this configuration of the torcular. Childhood headache venous sinus anatomy confluence of sinuses Figures Figure 1 Introduction Torcular herophili, also known as the confluence of the sinuses, is present at the posterior end of the superior sagittal sinus. Bilateral transverse sinuses arise from it and the straight sinus and occipital sinus drains into it. Several anatomical variations of the torcular herophili have been noted and Kobayashi et al. classified variants of the torcular herophili into nine types with type 1 being the most common[ 1 ]. Tardieu et al. reported a previously unreported variant of the torcular herophili with a circular configuration along with absent right transverse sinus[ 2 ]. A second case was reported by Lake et al. in a cadaveric case[ 3 ] 3 . Herein, we report a case of circular torcula herophili presenting with headache. Case Report A middle school student presented with history of intermittent episodes of holocranial headache not associated with any history of vomiting for 4 months. There was no history of convulsion, loss of consciousness, limb weakness or any cranial nerve palsies. Neurological examination was unremarkable without any focal neurodeficit. Initial imaging showed mild enlargement of ventricles however fundoscopy did not show any papilledema. Magnetic resonance imaging of brain with MR venography brain showed the presence of circular configuration of torcular herophili with absent left transverse sinus without any other structural abnormality (Fig. 1 A). The straight sinus was draining into the left hemicircumference of the circular torcular herophili (Fig. 1 B). Aqueductal stroke volume was 69 microlitre. Lumbar puncture was done with the opening pressure being 16 mmHg. Although the child reported improvement after lumbar puncture and CSF drainage, we continued to keep the kid on conservative lines of management. On 6-month follow-up the kid remained asymptomatic without any further episodes of headache and resumed academics. Patient is continuing close follow-up. Discussion Headache in children have been classified into primary and secondary headache. The causes of headache in the pediatric population are multifactorial with the common causes of primary headache being migraine and migraine variants, tension type headaches and trigeminal autonomic cephalalgias. Common etiologies of secondary headache include post traumatic headache, headache due to intracranial space occupying lesions (SOLs), headache due to vascular lesions and those due to psychiatric disorders[ 4 ]. Clinically, headaches can also be classified as acute, episodic, chronic progressive or chronic non-progressive[ 5 ]. The American Academy of Neurology has recommended to obtain neuroimaging in children with headache who have abnormal findings in neurologic exam and/or concomitant seizures or in those children in whom there is history suggestive of recent onset severe headache, change in the type of headache or with associated neurologic dysfunction[ 6 ]. According to the classification system proposed by Kobayashi et al. type 1 torcular herophili, i.e., superior sagittal sinus and two transverse sinuses forming a single point, is the most common variant[ 1 ]. The superior sagittal sinus predominantly drains into the right transverse sinus. The embryological origin of the cranial venous system can be divided into the intracerebral venous system and the extracerebral venous system. The intracerebral venous system arises from the pericerebral capillary meshwork in the meninx primitiva. Subsequently, the intracerebral veins can be differentiated into i) deep medullary veins and dorsal nuclear veins which drain into the subependymal system, ii) cortical-subcortical medullary veins and the ventral nuclear veins which drain into the leptomeningeal system and iii) transcerebral veins which connect the surface venous network with the subependymal venous network[ 7 ]. The development of the extracerebral venous system is affected by the development of both the brain and the skull. Padget has divided the development of cranial venous system into eight stages [ 8 ]. In Padget’s venous stage 1, the capital venous plexuses and the capital vein is formed. In venous stage 2, three dural stems, the anterior, middle and posterior drain into the primary head sinus which is continuous with the anterior cardinal vein. In stages 3 and 4, the dural venous sinuses shift more laterally as the cerebral hemispheres expand. In stage 5, the head sinus is replaced by the sigmoid sinus. During stage 6, the external jugular system arises. In stage 7, the mature cerebral venous system becomes apparent and in stage 7a, the primitive torcular forms. The torcular herophili embryologically develops from the anterior and middle dural plexus. Developmentally the sagittal plexus arises from the caudally shifted anterior dural plexus due to the growth of the forebrain. The superior sagittal sinus arises from the sagittal plexus. The anterior dural plexus is joined posteriorly by the middle dural plexus forming the junctional area of the drainage of superior sagittal sinus, straight sinus and bilateral transverse sinus, thereby forming the primitive torcular herophili [ 2 ]. The present case has clinical similarity with that reported by Tardieu et al 2 . In both cases, headache was the presentation presumably due to raised intracranial pressure and had an unilateral absent transverse sinus. Tardieu et al. have proposed that circular configuration of the torcular herophili along with unilateral absence of transverse sinus may impair venous drainage causing cerebral oedema explaining the clinical symptom of the patient [ 2 ]. We propose that the association of circular torcular herophili with unilateral absent transverse sinus predisposes the patients to episodes of raised intracranial pressure and should be closely observed. Conclusion Circular configuration of the torcular herophili is an uncommon configuration of the torcular with only two previous reports in the literature to the best of our knowledge. This configuration may be an intriguing cause of otherwise unexplained headache, probably due to intermittent episodes of raised intracranial pressure due to venous congestion. Declarations Ethics approval and consent to participate – Institute ethical standards followed. Consent to participate was taken from individual patient. Consent for publication – Publication consent taken from the patient and the parent. Availability of data and material – all available date presented in the article Competing interests – There are no competing interests for any of the authors involved in the article. Funding – Not applicable. Authors' contributions – 1. Arunkumar Sekar – Patient care and preparation of the final draft. 2. Debajyoti Datta - First draft preparation, Review of literature and patient care. Acknowledgements – None. References Kobayashi K, Matsui O, Suzuki M, Ueda F (2006) Anatomical study of the confluence of the sinuses with contrast-enhanced magnetic resonance venography. Neuroradiology Tardieu GG, Oskouian RJ, Loukas M, Tubbs RS (2017) A previously undescribed variant of the confluence of sinuses. Folia Morphologica 76:316–8 Lake S, Altafulla JJ, Iwanaga J, et al (2018) A cadaveric case of a circular torcular Herophili. Cureus Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders. Cephalalgia [Internet] Cephalalgia 38:1–211 Kelly M, Strelzik J, Langdon R, DiSabella M (2018) Pediatric headache: overview. Current opinion in Pediatrics 1;30(6):748-54: Lewis DW, Ashwal S, Dahl G, et al (2002) Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 27;59(4):490–8: Raybaud C (2010) Normal and abnormal embryology and development of the intracranial vascular system. Neurosurgery Clinics 1;21(3):399-426: Padget DH (1956) The cranial venous system in man in reference to development, adult configuration, and relation to the arteries. Am J Anat 98:307–55 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. 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Also discoverable on Platform About In Review Editorial Policies Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-2394561","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":161872103,"identity":"50da6627-6a5f-4800-9484-6da608791876","order_by":0,"name":"Debajyoti Datta","email":"","orcid":"","institution":"All India Institute of Medical Sciences Bhubaneswar","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Debajyoti","middleName":"","lastName":"Datta","suffix":""},{"id":161872104,"identity":"eb32ed66-e466-4bd5-8d44-4af105ea011c","order_by":1,"name":"Arunkumar Sekar","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABAUlEQVRIiWNgGAWjYJACxgYQyd/YcOBDxQGwyIEHRGmROHzw4YwzBxh4QFoSiNLCkJZszNsG0cKATwv/7DNmD2e2bZM3bzhjJsE7746cvdjhh0Bb7OR0G7BrkTiXY264se224ZzDPWYSktueGfNIpxkAtSQbmx3AYc0ZHjPJh223GWcwAG0x3HY4sUc6AaTlQOI2HFrkoVrsZzDkmEkkzgFpSf+AV4sBSAvQYYkzgN43ONgA0pKD3xbDM2xlkjPO3U6eAQrkhmOHjXlu5xQcSDDA7Re5M8zbJHvKbtvOAEbl4T81h+XYZ6dv/vChwk4Op/dxAAPSlI+CUTAKRsEoQAUAuJppWH+Js60AAAAASUVORK5CYII=","orcid":"","institution":"All India Institute of Medical Sciences Bhubaneswar","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Arunkumar","middleName":"","lastName":"Sekar","suffix":""}],"badges":[],"createdAt":"2022-12-19 17:29:16","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-2394561/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-2394561/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":30905277,"identity":"c82f51f9-160d-47a6-9c4a-0af5844292d4","added_by":"auto","created_at":"2022-12-29 21:18:44","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":67215,"visible":true,"origin":"","legend":"\u003cp\u003eA – Magnetic resonance Venography showing the circular configuration of the torcular herophili with absent left transverse sinus (posterior view).\u003c/p\u003e\n\u003cp\u003eB - Magnetic resonance Venography showing the straight sinus (marked with arrow) draining into the left half of the circular torcular herophili (posterior view).\u003c/p\u003e","description":"","filename":"Figure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-2394561/v1/f34a787d3229f83da22f189d.jpg"},{"id":30957107,"identity":"55a19638-93eb-4ffe-b380-6d3fd2ddcf9d","added_by":"auto","created_at":"2023-01-01 11:14:26","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":206144,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-2394561/v1/939cb928-4eba-4f65-8894-6e34e062fc4c.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Circular configuration of Torcular Herophili presenting as childhood headache","fulltext":[{"header":"Introduction","content":"\u003cp\u003eTorcular herophili, also known as the confluence of the sinuses, is present at the posterior end of the superior sagittal sinus. Bilateral transverse sinuses arise from it and the straight sinus and occipital sinus drains into it. Several anatomical variations of the torcular herophili have been noted and Kobayashi \u003cem\u003eet al.\u003c/em\u003e classified variants of the torcular herophili into nine types with type 1 being the most common[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Tardieu \u003cem\u003eet al.\u003c/em\u003e reported a previously unreported variant of the torcular herophili with a circular configuration along with absent right transverse sinus[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. A second case was reported by Lake \u003cem\u003eet al.\u003c/em\u003e in a cadaveric case[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e] \u003csup\u003e3\u003c/sup\u003e. Herein, we report a case of circular torcula herophili presenting with headache.\u003c/p\u003e"},{"header":"Case Report","content":"\u003cp\u003eA middle school student presented with history of intermittent episodes of holocranial headache not associated with any history of vomiting for 4 months. There was no history of convulsion, loss of consciousness, limb weakness or any cranial nerve palsies. Neurological examination was unremarkable without any focal neurodeficit. Initial imaging showed mild enlargement of ventricles however fundoscopy did not show any papilledema. Magnetic resonance imaging of brain with MR venography brain showed the presence of circular configuration of torcular herophili with absent left transverse sinus without any other structural abnormality (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e1\u003c/span\u003eA). The straight sinus was draining into the left hemicircumference of the circular torcular herophili (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e1\u003c/span\u003eB). Aqueductal stroke volume was 69 microlitre. Lumbar puncture was done with the opening pressure being 16 mmHg. Although the child reported improvement after lumbar puncture and CSF drainage, we continued to keep the kid on conservative lines of management. On 6-month follow-up the kid remained asymptomatic without any further episodes of headache and resumed academics. Patient is continuing close follow-up.\u003c/p\u003e "},{"header":"Discussion","content":"\u003cp\u003eHeadache in children have been classified into primary and secondary headache. The causes of headache in the pediatric population are multifactorial with the common causes of primary headache being migraine and migraine variants, tension type headaches and trigeminal autonomic cephalalgias. Common etiologies of secondary headache include post traumatic headache, headache due to intracranial space occupying lesions (SOLs), headache due to vascular lesions and those due to psychiatric disorders[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. Clinically, headaches can also be classified as acute, episodic, chronic progressive or chronic non-progressive[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. The American Academy of Neurology has recommended to obtain neuroimaging in children with headache who have abnormal findings in neurologic exam and/or concomitant seizures or in those children in whom there is history suggestive of recent onset severe headache, change in the type of headache or with associated neurologic dysfunction[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eAccording to the classification system proposed by Kobayashi \u003cem\u003eet al.\u003c/em\u003e type 1 torcular herophili, i.e., superior sagittal sinus and two transverse sinuses forming a single point, is the most common variant[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. The superior sagittal sinus predominantly drains into the right transverse sinus. The embryological origin of the cranial venous system can be divided into the intracerebral venous system and the extracerebral venous system. The intracerebral venous system arises from the pericerebral capillary meshwork in the meninx primitiva. Subsequently, the intracerebral veins can be differentiated into i) deep medullary veins and dorsal nuclear veins which drain into the subependymal system, ii) cortical-subcortical medullary veins and the ventral nuclear veins which drain into the leptomeningeal system and iii) transcerebral veins which connect the surface venous network with the subependymal venous network[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe development of the extracerebral venous system is affected by the development of both the brain and the skull. Padget has divided the development of cranial venous system into eight stages [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. In Padget\u0026rsquo;s venous stage 1, the capital venous plexuses and the capital vein is formed. In venous stage 2, three dural stems, the anterior, middle and posterior drain into the primary head sinus which is continuous with the anterior cardinal vein. In stages 3 and 4, the dural venous sinuses shift more laterally as the cerebral hemispheres expand. In stage 5, the head sinus is replaced by the sigmoid sinus. During stage 6, the external jugular system arises. In stage 7, the mature cerebral venous system becomes apparent and in stage 7a, the primitive torcular forms. The torcular herophili embryologically develops from the anterior and middle dural plexus. Developmentally the sagittal plexus arises from the caudally shifted anterior dural plexus due to the growth of the forebrain. The superior sagittal sinus arises from the sagittal plexus. The anterior dural plexus is joined posteriorly by the middle dural plexus forming the junctional area of the drainage of superior sagittal sinus, straight sinus and bilateral transverse sinus, thereby forming the primitive torcular herophili [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eThe present case has clinical similarity with that reported by Tardieu \u003cem\u003eet al\u003c/em\u003e \u003csup\u003e2\u003c/sup\u003e. In both cases, headache was the presentation presumably due to raised intracranial pressure and had an unilateral absent transverse sinus. Tardieu \u003cem\u003eet al.\u003c/em\u003e have proposed that circular configuration of the torcular herophili along with unilateral absence of transverse sinus may impair venous drainage causing cerebral oedema explaining the clinical symptom of the patient [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. We propose that the association of circular torcular herophili with unilateral absent transverse sinus predisposes the patients to episodes of raised intracranial pressure and should be closely observed.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eCircular configuration of the torcular herophili is an uncommon configuration of the torcular with only two previous reports in the literature to the best of our knowledge. This configuration may be an intriguing cause of otherwise unexplained headache, probably due to intermittent episodes of raised intracranial pressure due to venous congestion.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e \u0026ndash; Institute ethical standards followed. Consent to participate was taken from individual patient.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e \u0026ndash; Publication consent taken from the patient and the parent.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and material\u003c/strong\u003e \u0026ndash; all available date presented in the article\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e \u0026ndash; There are no competing interests for any of the authors involved in the article.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e \u0026ndash; Not applicable.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions \u0026ndash;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e1. \u0026nbsp; \u0026nbsp;Arunkumar Sekar \u0026ndash; Patient care and preparation of the final draft.\u003c/p\u003e\n\u003cp\u003e2. \u0026nbsp; \u0026nbsp;Debajyoti Datta - First draft preparation, Review of literature and patient care.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u0026nbsp;\u003c/strong\u003e\u0026ndash; None.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eKobayashi K, Matsui O, Suzuki M, Ueda F (2006) Anatomical study of the confluence of the sinuses with contrast-enhanced magnetic resonance venography. Neuroradiology\u003c/li\u003e\n\u003cli\u003eTardieu GG, Oskouian RJ, Loukas M, Tubbs RS (2017) A previously undescribed variant of the confluence of sinuses. Folia Morphologica 76:316–8\u003c/li\u003e\n\u003cli\u003eLake S, Altafulla JJ, Iwanaga J, et al (2018) A cadaveric case of a circular torcular Herophili. Cureus\u003c/li\u003e\n\u003cli\u003eHeadache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders. Cephalalgia [Internet] Cephalalgia 38:1–211\u003c/li\u003e\n\u003cli\u003eKelly M, Strelzik J, Langdon R, DiSabella M (2018) Pediatric headache: overview. Current opinion in Pediatrics 1;30(6):748-54:\u003c/li\u003e\n\u003cli\u003eLewis DW, Ashwal S, Dahl G, et al (2002) Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 27;59(4):490–8:\u003c/li\u003e\n\u003cli\u003eRaybaud C (2010) Normal and abnormal embryology and development of the intracranial vascular system. Neurosurgery Clinics 1;21(3):399-426:\u003c/li\u003e\n\u003cli\u003ePadget DH (1956) The cranial venous system in man in reference to development, adult configuration, and relation to the arteries. Am J Anat 98:307–55\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"
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