Neuroendoscopic Management for Right Epileptogenic Hypothalamic Hamartoma

Neuroendoscopic Management for Right Epileptogenic Hypothalamic Hamartoma | 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 Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Neuroendoscopic Management for Right Epileptogenic Hypothalamic Hamartoma Sergio Cavalheiro, José Antonio Rangel Quiróz, Patricia Alessandra Dastoli, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-5908672/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 Hypothalamic hamartomas are congenital hypothalamus lesions that usually cause refractory seizures, endocrinological disturbances, cognitive decline, and psychiatric symptoms. Magnetic resonance imaging of the brain with and without contrast agent is the gold standard for diagnosis. Surgery is generally indicated when medication-refractory seizures occur. We present the case of a 4-year-old boy with epilepsy refractory to anticonvulsant medications where magnetic resonance imaging revealed a lesion at the level of the right mammillary body. We present endoscopic resection of the right hamartoma, which improved the patient’s seizures (Engel I) without neurological complications in his postoperative images and without complications in his postoperative images. Neurosurgery Pediatrics Epilepsy Disconnection Tumor Mammillary body Figures Figure 1 Figure 2 Figure 3 Introduction Hypothalamic hamartomas are congenital hypothalamus lesions that usually cause refractory seizures, endocrinological disturbances, cognitive decline, and psychiatric symptoms[1]. Magnetic resonance imaging of the brain with and without contrast agent is the gold standard for diagnosis.[2] Among the treatments are observation, surgery, antiepileptic medication, radiosurgery, stereotaxic thermoablation, and hormonal restitution[3]. Surgery is generally indicated when medication-refractory seizures occur[3]. Among the surgical approaches, Stereotaxic Thermoablation (Magnetic Resonance-Guided Laser Interstitial Thermal Therapy) is the first-line method.[4] However, the endoscopic approach can also be used depending on the location of the hamartoma.[3]The neuroendoscopic approach has been implemented to disconnect and excise the lesion.[5], [6]. At the time of the approach, it is advisable to make a contralateral entry to the injury for better visualization[6]. Clinical presentation The most common symptoms at presentation are gelastic seizures and central precocious puberty.[7], [8]The age of onset of epilepsy ranged from 1 postnatal day to 27 years (mean 2.5 years, median 1 year). The initial seizure pattern is usually a gelastic seizure with a brief attack of mechanical laughter starting during early infancy, and the patient can experience another type of seizure: atypical absence, drop attacks, motor seizures, and tonic‒clonic seizures. In patients with epilepsy onset during early childhood, cognitive dysfunction and behavioral problems are commonly progressive. Pubertal manifestations include deepening of the voice and enlarged testes and penis in males, breast development and menses in females, excessive skeletal and muscular development, pubic hair, and adolescent personality in both sexes.[7] Diagnosis Hypothalamic hamartomas are isodense on cerebral tomography.[9] . Magnetic resonance imaging typically reveals a collar button-like lesion between the infundibular stalk and the mammillary bodies. This lesion does not enhance but appears slightly hyperintense compared with gray matter on T2-weighted imaging. The immediate differential diagnosis is a hypothalamic glioma that is usually strongly and uniformly enhanced following contrast.[10] Surgical Management Different types of surgical treatment are used: ventricular endoscopic, transcallosal, and skull base approaches. No single approach is the best approach or is appropriate in all cases. Adequate treatment requires individualizing the approach on the basis of a patient’s age and condition, the anatomy of the HH, and the surgeon’s experience[11]. The transcallosal, interseptal, and interfornical approach is preferred for large hypothalamic hamartomas with a significant intraventricular component located superior to the level of the optic tract. This approach can be used alone to treat large type II lesions. Many Type III and IV lesions require a staged approach.[11] The skull-based approach is used for types I, III, and IV hamartomas. Depending on the surgeon's expertise, an orbit zygomatic approach, pterional approach, supraorbital eyebrow approach, or subtemporal approach can be used.[11] The endoscopic approach is used for both resection and disconnection. When this method is used, an intraoperative image guidance system is recommended to identify the best entry point and trajectory for the transcortical, transventricular, and foramen of Monroe approaches. A contralateral approach to the lesion is also crucial for good visualization of the plane between the hypothalamus and the hamartoma.[6] Factors favoring the transventricular endoscopic approach for the resection of hypothalamic hamartomas (HHs) include the following: Small lesions : The endoscopic approach is ideal for smaller HHs. Unilaterality of attachment : Lesions with unilateral attachment to the hypothalamus are best suited for this approach. Generously sized ventricles : Adequately sized ventricles facilitate the use of an endoscope. In particular, Type II lesions with unilateral attachment are ideal for an endoscopic approach from the contralateral ventricle[11]. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has emerged as a minimally invasive method for treating hypothalamic hamartoma. Many epilepsy centers have begun to adopt it as a first-line treatment option for this condition. In addition to its minimally invasive nature, MRgLITT is associated with low rates of postoperative complications. However, relatively few studies have described surgical outcomes for patients undergoing this treatment or identified prognostic indicators related to procedure success in sufficiently large patient groups.[4] Prognostic In general, most patients who undergo surgery via the transventricular endoscopic approach experience improvements in the control of their seizures.[11] Diabetes insipidus is commonly encountered as an acute and usually transient postoperative complication in 15–55% of HH patients[12] We have reported that increased appetite and increased risk of obesity are the single most common long-term postoperative complications after HH resection, occurring in up to 20% of patients.[12] Overall, surgical treatment for HHs can significantly improve seizure control and quality of life for many patients, although the risk of complications and the need for multiple procedures should be considered . Outcome Approximately 50% of patients who use the transventricular endoscopic technique achieve Engel I results, whereas the other 50% improve their seizures.[7] The mean postoperative stay was shorter for patients who underwent endoscopy than for those who underwent transcallosal resection. The main complications of endoscopy include 15–55% transient diabetes insipidus, permanent short-term memory loss in 5% of patients, and small thalamic infarcts in 7% of patients, the majority of whom are asymptomatic.[11] For the transcallosal approach, 54% of patients were completely seizure free, and 35% had at least a 90% improvement in seizure frequency. Transient postoperative memory disturbances occurred in 58% of patients who underwent surgery via the transcallosal approach, and persistent memory disturbances were noted in 8% of patients. Persistent endocrine disturbances requiring hormone replacement therapy were observed in 8% of patients who underwent transcallosal approaches (diabetes insipidus and hypothyroidism).[11] For orbitozygomatic craniotomy, 40% of patients were seizure free, and another 40% had a greater than 50% reduction in seizures[11] The complications associated with the orbitozygomatic approach for treating hypothalamic hamartomas (HHs) include the following: endocrine disturbances (diabetes insipidus, poikilothermia) and neurological deficits (visual field, hemiparesis). For magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) for hypothalamic hamartoma (HH), 72.3% of patients remained gelastic seizure free after the procedure. The overall Engel class I rate was 68.1%. [4] The long-term postoperative complications of magnetic resonance-guided laser interstitial thermal T include memory disturbances, weight gain, and endocrine dysfunction. [4] Exemplary case description A 4-year-old patient has been experiencing seizures since the first month of life and is currently not responding to anticonvulsant medication at therapeutic doses. The patient is presently experiencing 50 seizures per day. Historical background: Gelastic seizures Neonatal medical history: No relevant issues Current medications: Lamotrigine, Cannabidiol, Levetiracetam, Trileptal Magnetic resonance imaging revealed a hypointense lesion in T1 that was not reinforced with gadolinium (Fig. 1, Fig. 2) and appeared slightly hyperintense compared with the gray matter on T2(Fig. 3). We present endoscopic resection of the right hamartoma, which improved the patient’s seizures (Engel I) without neurological complications in his postoperative images and without complications in his postoperative images. (“Online Resource 1”) Conclusion - Hypothalamic hamartomas are rare congenital malformations that often cause gelastic/dacrystic seizures that can progress to pharmacoresistant epilepsy, leading to cognitive and behavioral problems, especially in children. - Unilateral hypothalamic hamartoma (Delalande class B) and a shorter history of epilepsy were identified as predictive factors for better seizure control outcomes. - The endoscopic approach is minimally invasive, with a short operative time and a short hospital stay, allowing for better preservation of short-term memory and hypothalamic function, which is a good option in place that does not include magnetic resonance-guided laser interstitial thermal therapy. -The use of one or more surgical approaches in appropriately selected patients with symptomatic HHs can provide excellent results. Curing or controlling seizures and improving quality of life are seen in a large majority of patients. Complications occur at an acceptable rate and can be minimized by a thorough understanding of the complex regional anatomy and anatomical considerations specific to each approach. Declarations Ethics committee approval was not required to perform the surgical procedure on the patient. The patient's parents gave informed consent for the surgery and gave verbal consent for the publication of their video surgery. References J. Parvizi et al. , “Gelastic epilepsy and hypothalamic hamartomas: Neuroanatomical analysis of brain lesions in 100 patients,” Brain , vol. 134, no. 10, pp. 2960–2968, 2011, doi: 10.1093/brain/awr235. J. L. Freeman et al. , “MR Imaging and Spectroscopic Study of Epileptogenic Hypothalamic Hamartomas: Analysis of 72 Cases.” C. M. Carballo Cuello and O. De Jesus, “Hypothalamic Hamartoma,” Stat Pearls , 2024. Y. Yao et al. , “Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Hypothalamic Hamartoma: Surgical Approach and Treatment Outcomes,” J Clin Med , vol. 11, no. 21, Nov. 2022, doi: 10.3390/jcm11216579. M. Budke, M. Á. Pérez-Jiménez, and J. H. Mena-Bernal, “A simple technique for endoscopic hypothalamic hamartoma disconnection in refractory epilepsy: 2-dimensional operative video,” Aug. 01, 2020, Oxford University Press . doi: 10.1093/ons/opaa055. S. Chibbaro et al. , “Pure endoscopic management of epileptogenic hypothalamic hamartomas,” Neurosurg Rev , vol. 40, no. 4, pp. 647–653, Oct. 2017, doi: 10.1007/s10143-017-0822-3. K. Arita, K. Kurisu, Y. Kiura, K. Iida, and H. Otsubo, “Hypothalamic Hamartoma,” 2005. J. Scholly et al. , “Hypothalamic hamartoma: Epileptogenesis beyond the lesion?,” Epilepsia , vol. 58, pp. 32–40, Jun. 2017, doi: 10.1111/epi.13755. R. Grech, S. Looby, J. Thornton, and P. Brennan, “Hypothalamic hamartoma,” 2013, doi: 10.1136/bcr-2012. “454”. S. D. Wait, A. A. Abla, B. D. Killory, P. Nakaji, and H. L. Rekate, “Surgical approaches to hypothalamic hamartomas,” Neurosurg Focus , vol. 30, no. 2, Feb. 2011, doi: 10.3171/2010.11.FOCUS10250. V. S. Harrison, O. Oatman, and J. F. Kerrigan, “Hypothalamic hamartoma with epilepsy: Review of endocrine comorbidity,” Epilepsia , vol. 58, pp. 50–59, Jun. 2017, doi: 10.1111/epi.13756. Additional Declarations The authors declare no competing interests. Supplementary Files NMREHH.mp4 Neuroendoscopic Management for Right Epileptogenic Hypothalamic Hamartoma 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. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board 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-5908672","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":407383214,"identity":"f4f89e03-b85d-47d7-a3f4-0966e32082f7","order_by":0,"name":"Sergio Cavalheiro","email":"","orcid":"","institution":"University Federal of São Paulo, Brazil","correspondingAuthor":false,"prefix":"","firstName":"Sergio","middleName":"","lastName":"Cavalheiro","suffix":""},{"id":407383215,"identity":"dc3db9a7-f080-4ca2-9c0d-db9008982b33","order_by":1,"name":"José Antonio Rangel 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version.\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-5908672/v1/c5a8091a17f058fc3a7fa41f.png"},{"id":75190281,"identity":"6e261ee6-3182-4ede-836e-eed07521441d","added_by":"auto","created_at":"2025-01-31 18:07:14","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":558431,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-5908672/v1/72a96443-2d4a-49a9-9287-130b669061db.pdf"},{"id":75187770,"identity":"a26315f9-16a2-4dc0-8bfc-9323f94ba19d","added_by":"auto","created_at":"2025-01-31 17:59:14","extension":"mp4","order_by":1,"title":"","display":"","copyAsset":false,"role":"supplement","size":252997378,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cu\u003e\u003cstrong\u003eNeuroendoscopic Management for Right Epileptogenic Hypothalamic Hamartoma\u003c/strong\u003e\u003c/u\u003e\u003c/p\u003e","description":"","filename":"NMREHH.mp4","url":"https://assets-eu.researchsquare.com/files/rs-5908672/v1/043e063de06c4ca92077ddf2.mp4"}],"financialInterests":"The authors declare no competing interests.","formattedTitle":"\u003cp\u003e\u003cstrong\u003eNeuroendoscopic Management for Right Epileptogenic Hypothalamic Hamartoma\u003c/strong\u003e\u003c/p\u003e","fulltext":[{"header":"Introduction","content":"\u003cp\u003eHypothalamic hamartomas are congenital hypothalamus lesions that usually cause refractory seizures, endocrinological disturbances, cognitive decline, and psychiatric symptoms[1].\u003c/p\u003e\n\u003cp\u003eMagnetic resonance imaging of the brain with and without contrast agent is the gold standard for diagnosis.[2]\u003c/p\u003e\n\u003cp\u003eAmong the treatments are observation, surgery, antiepileptic medication, radiosurgery, stereotaxic thermoablation, and hormonal restitution[3].\u003c/p\u003e\n\u003cp\u003eSurgery is generally indicated when medication-refractory seizures occur[3].\u003c/p\u003e\n\u003cp\u003eAmong the surgical approaches, Stereotaxic Thermoablation (Magnetic Resonance-Guided Laser Interstitial Thermal Therapy) is the first-line method.[4] However, the endoscopic approach can also be used depending on the location of the hamartoma.[3]The neuroendoscopic approach has been implemented to disconnect and excise the lesion.[5], [6]. At the time of the approach, it is advisable to make a contralateral entry to the injury for better visualization[6].\u003c/p\u003e"},{"header":"Clinical presentation","content":"\u003cp\u003eThe most common symptoms at presentation are gelastic seizures and central precocious puberty.[7], [8]The age of onset of epilepsy ranged from 1 postnatal day to 27 years (mean 2.5 years, median 1 year). The initial seizure pattern is usually a gelastic seizure with a brief attack of mechanical laughter starting during early infancy, and the patient can experience another type of seizure: atypical absence, drop attacks, motor seizures, and tonic‒clonic seizures.\u003c/p\u003e\n\u003cp\u003eIn patients with epilepsy onset during early childhood, cognitive dysfunction and behavioral problems are commonly progressive.\u003c/p\u003e\n\u003cp\u003ePubertal manifestations include deepening of the voice and enlarged testes and penis in males, breast development and menses in females, excessive skeletal and muscular development, pubic hair, and adolescent personality in both sexes.[7]\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cu\u003eDiagnosis\u003c/u\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eHypothalamic hamartomas are isodense on cerebral tomography.[9]\u003csup\u003e.\u0026nbsp;\u003c/sup\u003eMagnetic resonance imaging typically reveals a collar button-like lesion between the infundibular stalk and the mammillary bodies. This lesion does not enhance but appears slightly hyperintense compared with gray matter on T2-weighted imaging. The immediate differential diagnosis is a hypothalamic glioma that is usually strongly and uniformly enhanced following contrast.[10]\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cu\u003eSurgical Management\u003c/u\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eDifferent types of surgical treatment are used: ventricular endoscopic, transcallosal, and skull base approaches. No single approach is the best approach or is appropriate in all cases. Adequate treatment requires individualizing the approach on the basis of a patient\u0026rsquo;s age and condition, the anatomy of the HH, and the surgeon\u0026rsquo;s experience[11].\u003c/p\u003e\n\u003cp\u003eThe transcallosal, interseptal, and interfornical approach is preferred for large hypothalamic hamartomas with a significant intraventricular component located superior to the level of the optic tract. This approach can be used alone to treat large type II lesions. Many Type III and IV lesions require a staged approach.[11]\u003c/p\u003e\n\u003cp\u003eThe skull-based approach is used for types I, III, and IV hamartomas. Depending on the surgeon\u0026apos;s expertise, an orbit zygomatic approach, pterional approach, supraorbital eyebrow approach, or subtemporal approach can be used.[11]\u003c/p\u003e\n\u003cp\u003eThe endoscopic approach is used for both resection and disconnection. When this method is used, an intraoperative image guidance system is recommended to identify the best entry point and trajectory for the transcortical, transventricular, and foramen of Monroe approaches. A contralateral approach to the lesion is also crucial for good visualization of the plane between the hypothalamus and the hamartoma.[6]\u003c/p\u003e\n\u003cp\u003eFactors favoring the transventricular endoscopic approach for the resection of hypothalamic hamartomas (HHs) include the following:\u003c/p\u003e\n\u003cul\u003e\n \u003cli\u003e\u003cem\u003eSmall lesions\u003c/em\u003e: The endoscopic approach is ideal for smaller HHs.\u003c/li\u003e\n \u003cli\u003e\u003cem\u003eUnilaterality of attachment\u003c/em\u003e: Lesions with unilateral attachment to the hypothalamus are best suited for this approach.\u003c/li\u003e\n \u003cli\u003e\u003cem\u003eGenerously sized ventricles\u003c/em\u003e: Adequately sized ventricles facilitate the use of an endoscope.\u003c/li\u003e\n\u003c/ul\u003e\n\u003cp\u003eIn particular, Type II lesions with unilateral attachment are ideal for an endoscopic approach from the contralateral ventricle[11].\u003c/p\u003e\n\u003cp\u003eMagnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has emerged as a minimally invasive method for treating hypothalamic hamartoma. Many epilepsy centers have begun to adopt it as a first-line treatment option for this condition. In addition to its minimally invasive nature, MRgLITT is associated with low rates of postoperative complications. However, relatively few studies have described surgical outcomes for patients undergoing this treatment or identified prognostic indicators related to procedure success in sufficiently large patient groups.[4]\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePrognostic\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eIn general, most patients who undergo surgery via the transventricular endoscopic approach experience improvements in the control of their seizures.[11]\u003c/p\u003e\n\u003cp\u003eDiabetes insipidus is commonly encountered as an acute and usually transient postoperative complication in 15\u0026ndash;55% of HH patients[12]\u003c/p\u003e\n\u003cp\u003eWe have reported that increased appetite and increased risk of obesity are the single most common long-term postoperative complications after HH resection, occurring in up to 20% of patients.[12]\u003c/p\u003e\n\u003cp\u003eOverall, surgical treatment for HHs can significantly improve seizure control and quality of life for many patients, although the risk of complications and the need for multiple procedures should be considered\u003cstrong\u003e.\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eOutcome\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eApproximately 50% of patients who use the transventricular endoscopic technique achieve Engel I results, whereas the other 50% improve their seizures.[7]\u003c/p\u003e\n\u003cp\u003eThe mean postoperative stay was shorter for patients who underwent endoscopy than for those who underwent transcallosal resection.\u003c/p\u003e\n\u003cp\u003eThe main complications of endoscopy include 15\u0026ndash;55% transient diabetes insipidus, permanent short-term memory loss in 5% of patients, and small thalamic infarcts in 7% of patients, the majority of whom are asymptomatic.[11]\u003c/p\u003e\n\u003cp\u003eFor the transcallosal approach, 54% of patients were completely seizure free, and 35% had at least a 90% improvement in seizure frequency.\u003c/p\u003e\n\u003cp\u003eTransient postoperative memory disturbances occurred in 58% of patients who underwent surgery via the transcallosal approach, and persistent memory disturbances were noted in 8% of patients. Persistent endocrine disturbances requiring hormone replacement therapy were observed in 8% of patients who underwent transcallosal approaches (diabetes insipidus and hypothyroidism).[11]\u003c/p\u003e\n\u003cp\u003eFor orbitozygomatic craniotomy, 40% of patients were seizure free, and another 40% had a greater than 50% reduction in seizures[11]\u003c/p\u003e\n\u003cp\u003eThe complications associated with the orbitozygomatic approach for treating hypothalamic hamartomas (HHs) include the following: endocrine disturbances (diabetes insipidus, poikilothermia) and neurological deficits (visual field, hemiparesis).\u003c/p\u003e\n\u003cp\u003eFor magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) for hypothalamic hamartoma (HH), 72.3% of patients remained gelastic seizure free after the procedure. \u0026nbsp;The overall Engel class I rate was 68.1%. [4]\u003c/p\u003e\n\u003cp\u003eThe long-term postoperative complications of magnetic resonance-guided laser interstitial thermal T include memory disturbances, weight gain, and endocrine dysfunction. [4]\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eExemplary case description\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eA 4-year-old patient has been experiencing seizures since the first month of life and is currently not responding to anticonvulsant medication at therapeutic doses. The patient is presently experiencing 50 seizures per day.\u003c/p\u003e\n\u003cp\u003eHistorical background: Gelastic seizures\u003c/p\u003e\n\u003cp\u003eNeonatal medical history: No relevant issues\u003c/p\u003e\n\u003cp\u003eCurrent medications: Lamotrigine, Cannabidiol, Levetiracetam, Trileptal\u003c/p\u003e\n\u003cp\u003eMagnetic resonance imaging revealed a hypointense lesion in T1 that was not reinforced with gadolinium (Fig. 1, Fig. 2) and appeared slightly hyperintense compared with the gray matter on T2(Fig. 3).\u003c/p\u003e\n\u003cp\u003eWe present endoscopic resection of the right hamartoma, which improved the patient\u0026rsquo;s seizures (Engel I) without neurological complications in his postoperative images and without complications in his postoperative images. (\u0026ldquo;Online Resource 1\u0026rdquo;)\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003e- Hypothalamic hamartomas are rare congenital malformations that often cause gelastic/dacrystic seizures that can progress to pharmacoresistant epilepsy, leading to cognitive and behavioral problems, especially in children.\u003c/p\u003e\n\u003cp\u003e- Unilateral hypothalamic hamartoma (Delalande class B) and a shorter history of epilepsy were identified as predictive factors for better seizure control outcomes.\u003c/p\u003e\n\u003cp\u003e- The endoscopic approach is minimally invasive, with a short operative time and a short hospital stay, allowing for better preservation of short-term memory and hypothalamic function, which is a good option in place that does not include magnetic resonance-guided laser interstitial thermal therapy.\u003c/p\u003e\n\u003cp\u003e-The use of one or more surgical approaches in appropriately selected patients with symptomatic HHs can provide excellent results. \u0026nbsp;Curing or controlling seizures and improving quality of life are seen in a large majority of patients. \u0026nbsp;Complications occur at an acceptable rate and can be minimized by a thorough understanding of the complex regional anatomy and anatomical considerations specific to each approach.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eEthics committee approval was not required to perform the surgical procedure on the patient. The patient\u0026apos;s parents gave informed consent for the surgery and gave verbal consent for the publication of their video surgery.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eJ. Parvizi \u003cem\u003eet al.\u003c/em\u003e, \u0026ldquo;Gelastic epilepsy and hypothalamic hamartomas: Neuroanatomical analysis of brain lesions in 100 patients,\u0026rdquo; \u003cem\u003eBrain\u003c/em\u003e, vol. 134, no. 10, pp. 2960\u0026ndash;2968, 2011, doi: 10.1093/brain/awr235.\u003c/li\u003e\n\u003cli\u003eJ. L. Freeman \u003cem\u003eet al.\u003c/em\u003e, \u0026ldquo;MR Imaging and Spectroscopic Study of Epileptogenic Hypothalamic Hamartomas: Analysis of 72 Cases.\u0026rdquo;\u003c/li\u003e\n\u003cli\u003eC. M. Carballo Cuello and O. De Jesus, \u0026ldquo;Hypothalamic Hamartoma,\u0026rdquo; \u003cem\u003eStat Pearls\u003c/em\u003e, 2024.\u003c/li\u003e\n\u003cli\u003eY. Yao \u003cem\u003eet al.\u003c/em\u003e, \u0026ldquo;Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Hypothalamic Hamartoma: Surgical Approach and Treatment Outcomes,\u0026rdquo; \u003cem\u003eJ Clin Med\u003c/em\u003e, vol. 11, no. 21, Nov. 2022, doi: 10.3390/jcm11216579.\u003c/li\u003e\n\u003cli\u003eM. Budke, M. \u0026Aacute;. P\u0026eacute;rez-Jim\u0026eacute;nez, and J. H. Mena-Bernal, \u0026ldquo;A simple technique for endoscopic hypothalamic hamartoma disconnection in refractory epilepsy: 2-dimensional operative video,\u0026rdquo; Aug. 01, 2020, \u003cem\u003eOxford University Press\u003c/em\u003e. doi: 10.1093/ons/opaa055.\u003c/li\u003e\n\u003cli\u003eS. Chibbaro \u003cem\u003eet al.\u003c/em\u003e, \u0026ldquo;Pure endoscopic management of epileptogenic hypothalamic hamartomas,\u0026rdquo; \u003cem\u003eNeurosurg Rev\u003c/em\u003e, vol. 40, no. 4, pp. 647\u0026ndash;653, Oct. 2017, doi: 10.1007/s10143-017-0822-3.\u003c/li\u003e\n\u003cli\u003eK. Arita, K. Kurisu, Y. Kiura, K. Iida, and H. Otsubo, \u0026ldquo;Hypothalamic Hamartoma,\u0026rdquo; 2005.\u003c/li\u003e\n\u003cli\u003eJ. Scholly \u003cem\u003eet al.\u003c/em\u003e, \u0026ldquo;Hypothalamic hamartoma: Epileptogenesis beyond the lesion?,\u0026rdquo; \u003cem\u003eEpilepsia\u003c/em\u003e, vol. 58, pp. 32\u0026ndash;40, Jun. 2017, doi: 10.1111/epi.13755.\u003c/li\u003e\n\u003cli\u003eR. Grech, S. Looby, J. Thornton, and P. Brennan, \u0026ldquo;Hypothalamic hamartoma,\u0026rdquo; 2013, doi: 10.1136/bcr-2012.\u003c/li\u003e\n\u003cli\u003e\u0026ldquo;454\u0026rdquo;.\u003c/li\u003e\n\u003cli\u003eS. D. Wait, A. A. Abla, B. D. Killory, P. Nakaji, and H. L. Rekate, \u0026ldquo;Surgical approaches to hypothalamic hamartomas,\u0026rdquo; \u003cem\u003eNeurosurg Focus\u003c/em\u003e, vol. 30, no. 2, Feb. 2011, doi: 10.3171/2010.11.FOCUS10250.\u003c/li\u003e\n\u003cli\u003eV. S. Harrison, O. Oatman, and J. F. Kerrigan, \u0026ldquo;Hypothalamic hamartoma with epilepsy: Review of endocrine comorbidity,\u0026rdquo; \u003cem\u003eEpilepsia\u003c/em\u003e, vol. 58, pp. 50\u0026ndash;59, Jun. 2017, doi: 10.1111/epi.13756.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"University Federal of Sao Paulo Brasil","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Epilepsy, Disconnection, Tumor, Mammillary body","lastPublishedDoi":"10.21203/rs.3.rs-5908672/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-5908672/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eHypothalamic hamartomas are congenital hypothalamus lesions that usually cause refractory seizures, endocrinological disturbances, cognitive decline, and psychiatric symptoms.\u003c/p\u003e\n\u003cp\u003eMagnetic resonance imaging of the brain with and without contrast agent is the gold standard for diagnosis.\u003c/p\u003e\n\u003cp\u003eSurgery is generally indicated when medication-refractory seizures occur.\u003c/p\u003e\n\u003cp\u003eWe present the case of a 4-year-old boy with epilepsy refractory to anticonvulsant medications where magnetic resonance imaging revealed a lesion at the level of the right mammillary body.\u003c/p\u003e\n\u003cp\u003eWe present endoscopic resection of the right hamartoma, which improved the patient’s seizures (Engel I) without neurological complications in his postoperative images and without complications in his postoperative images.\u003c/p\u003e","manuscriptTitle":"Neuroendoscopic Management for Right Epileptogenic Hypothalamic Hamartoma","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-01-31 17:59:05","doi":"10.21203/rs.3.rs-5908672/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"6ea54b33-4b18-44e8-81d7-dc2b47e54a3d","owner":[],"postedDate":"January 31st, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":43655552,"name":"Neurosurgery"},{"id":43655553,"name":"Pediatrics"}],"tags":[],"updatedAt":"2025-01-31T17:59:05+00:00","versionOfRecord":[],"versionCreatedAt":"2025-01-31 17:59:05","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-5908672","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-5908672","identity":"rs-5908672","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}