Spinal Capillary Hemangioma with Intramedullary and Extramedullary Extension in a Child with Cutaneous Stigmata: A Rare Case

Spinal Capillary Hemangioma with Intramedullary and Extramedullary Extension in a Child with Cutaneous Stigmata: A Rare Case | 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 Spinal Capillary Hemangioma with Intramedullary and Extramedullary Extension in a Child with Cutaneous Stigmata: A Rare Case Ozan Başkurt, Mehmet Arda Inan, Tufan Hicdonmez This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6844072/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 16 Oct, 2025 Read the published version in Child's Nervous System → Version 1 posted 7 You are reading this latest preprint version Abstract We report a rare case of a pediatric spinal capillary hemangioma with both intramedullary and extramedullary components in association with a dorsal cutaneous lesion. The patient, an 13-month-old girl, initially presented with a soft tissue mass on her lower back. MRI revealed a lesion extending from the conus medullaris to L4, initially misinterpreted as a mature teratoma. Due to the emergence of new urodynamic abnormalities, surgical exploration revealed a reddish-brown, moderately firm tumor with tethering of the spinal cord, which was partially resected under intraoperative neuromonitoring. Histopathological findings confirmed capillary hemangioma. To date, only 13 pediatric cases have been reported, and our case represents the first with combined intra- and extramedullary involvement and cutaneous stigmata. This case highlights the importance of including vascular tumors in the differential diagnosis of spinal dysraphism with skin markers and underscores the value of early surgical intervention to prevent neurological decline. Capillary hemangioma Cutaneous stigmata Intramedullary lesion Pediatric spinal tumor Spinal dysraphism Tethered cord Figures Figure 1 Figure 2 Figure 3 Figure 4 Case Presentation We report the case of a female infant who presented to our clinic at 8 months of age with a soft tissue mass on her lower back, which had been previously treated with physical therapy. At admission, her head circumference was 45 cm (75th percentile), and the anterior fontanel was small but open. Neurological examination revealed full strength in the lower extremities, with no deformities of the legs or feet. On inspection, there was asymmetry in the mid-lumbar and upper sacral region, with a hyperemic soft tissue mass over the skin in the left paralumbar area ( Fig. 1 ). Lumbar MRI revealed findings consistent with dorsal lipomyelomeningocele and a thickened filum terminale, suggestive of a low conus medullaris terminating at the L2 level. A lesion extending distally from the conus medullaris measured approximately 11×11×61 mm. The superior portion appeared isointense on T1-weighted images and markedly hyperintense on T2-weighted sequences, with signal suppression on fat-saturated images. The inferior part of the lesion demonstrated heterogeneous internal architecture and showed irregular contrast enhancement, raising suspicion for a mature teratoma of the conus medullaris ( Fig. 2 ). Initial urodynamic evaluation was normal, and the patient was placed under follow-up. At 13 months, a control MRI using short tau inversion recovery (STIR) sequences was obtained. By this time, the patient had begun walking and her developmental milestones were age-appropriate. However, she exhibited right foot inversion and a positive pes varus deformity. Due to the emergence of new urodynamic abnormalities, surgical intervention was indicated. Under intraoperative neuromonitoring, laminoplasty was performed at L1–L5 levels, with osteoblastic laminar flaps retracted cranially. A partial spina bifida was noted at S1, with a soft tissue mass replacing the bony elements; laminectomy was performed at this level. A midline dural defect was identified and carefully opened bilaterally. Controlled cerebrospinal fluid drainage was achieved. A reddish-brown, moderately firm intradural tumor extending from the conus medullaris to the L4 level was identified, containing both intra- and extramedullary components with areas of vascularity ( Fig. 3 ). The lesion was carefully dissected from surrounding spinal nerve roots and confirmed with neuromonitoring at L2–S2 levels. First debulking was made via ultrasonic aspirator. At caudal part, an aberrant dorsal root adherent to the dura was identified; it was found to be non-functional on EMG and was sacrificed. Than a thickened filum terminale was transected to relieve cord tension. The tumor had a cranially displaced component tightly adherent to the conus; grosstotal resection was performed at this site to preserve function. The subcutaneous component, which appeared firm and fibrolipomatous, was gross-totally resected. The postoperative course was uneventful, and the child was discharged on the fifth day without new neurological deficits. Histopathology revealed features consistent with capillary hemangioma ( Fig. 4 ), including low mitotic activity and diffuse CD34 positivity. At 1-year follow-up, the patient remained asymptomatic. Discussion Spinal capillary hemangiomas are rare benign vascular tumors, particularly uncommon in the pediatric population [ 1 ]. While they most frequently occur in adults, pediatric presentations remain exceptional, with fewer than 15 cases reported to date [ 2 – 5 ]. Only 7 of these cases were associated with cutaneous stigmata, making our case the 14th pediatric spinal capillary hemangioma and the 8th to feature concurrent skin findings [ 6 ]. The classification of spinal capillary hemangiomas includes four subtypes, with the pediatric variant typically manifesting as an intradural-extramedullary mass connected to a cutaneous lesion via a sinus tract [ 2 ]. Most reported cases in children involve the cauda equina or conus medullaris and are extramedullary in location [ 7 ]. The uniqueness of our case lies in its combined intramedullary and extramedullary configuration, extending from the conus medullaris to L4, with an additional dorsal subcutaneous component. To the best of our knowledge, such topographical involvement in a child has not been previously reported. The patient initially presented with a dorsal skin lesion at birth but maintained intact neurological function until 13 months of age, when she developed gait disturbance and new-onset urodynamic abnormalities. MRI revealed a complex lesion with fat signal components, heterogeneous contrast enhancement, and caudal extension, initially raising suspicion for mature teratoma. This highlights the diagnostic challenge, as similar imaging characteristics can be seen in other entities such as lipomyelomeningocele, teratoma, or epidermoid tumors [ 3 , 7 ]. Surgical exploration under intraoperative neuromonitoring revealed a reddish-brown, moderately firm lesion adherent to the conus, with both intra- and extramedullary components. Gross-total resection was achieved except for a portion tightly adherent to the conus medullaris, which was left in place to preserve neurological function. Histopathological findings, including lobular capillary structures and diffuse CD34 positivity, confirmed the diagnosis of capillary hemangioma [ 3 ]. From an embryological perspective, the association of this lesion with a thickened filum terminale, partial spina bifida, and cutaneous hemangioma suggests a possible error during secondary neurulation. This theory has been proposed in similar cases where capillary hemangiomas were found within or adjacent to filar lipomas [ 8 ], indicating a metameric distribution of pluripotent progenitor cells [ 2 , 6 ]. Our case also resembles a rare presentation described by Steinberger et al., in which a capillary hemangioma extended across the epidural, intradural, and intramedullary compartments in an infant, underscoring the need for high suspicion and individualized surgical planning in pediatric patients with atypical spinal lesions [ 9 ]. Conclusion This case illustrates a rare instance of a pediatric spinal capillary hemangioma with both intramedullary and extramedullary components, associated with cutaneous stigmata and tethered cord syndrome. The coexistence of skin findings and a complex spinal mass should raise suspicion for vascular tumors, including capillary hemangioma, in the differential diagnosis—particularly when imaging reveals fat signal characteristics and heterogeneous enhancement [ 3 , 7 ]. Surgical resection remains the mainstay of treatment, with intraoperative neuromonitoring playing a crucial role in preserving neurological function during dissection of adherent components [ 9 ]. The embryological link between dorsal skin lesions, filar abnormalities, and intraspinal vascular tumors underscores the importance of early neuroimaging in patients presenting with cutaneous stigmata suggestive of occult spinal dysraphism [ 2 , 8 ]. To our knowledge, this is the first reported pediatric case of a spinal capillary hemangioma exhibiting both intramedullary and extramedullary components along with a dorsal cutaneous lesion. Early recognition and surgical management can prevent irreversible neurological deficits and contribute to a favorable long-term outcome. Declarations Acknowledgements: None. Declaration of Interests: The authors have no relevant financial or non-financial interests to disclose. Funding: The authors did not receive support from any organization for the submitted work. Consent: Written informed consent was obtained from the legally authorized representative of the patient for publication of this case report and any accompanying images. Ethical statement: The need for ethical approval was waived by Istinye University Ethics Committee Authors’ Contribution: Study conception and design: OB, MAI, TH. Data collection, surgery: OB, TH. Pathological evaluation: MAI. Analysis and interpretation of results: OB, MAI, TH. Draft manuscript preparation: OB, TH. Critical revision of the article: TH. Study supervision: TH. All authors (OB, MAI, TH) reviewed the results and approved the final version of the manuscript Data Availability: All data generated or analyzed during this study are included in this article. Further enquiries can be directed to the corresponding author. References Iannelli A, Lupi G, Castagna M et al (2005) Intramedullary capillary hemangioma associated with hydrocephalus in an infant. J Neurosurg 103:272–276. https://doi.org/10.3171/ped.2005.103.3.0272 Karikari IO, Selznick LA, Cummings TJ, George TM (2007) Spinal capillary hemangioma in infants: report of two cases and review of the literature. Pediatr Neurosurg 43:125–129. https://doi.org/10.1159/000098386 Ohadi M, Maroufi SF, Meybodi KT et al (2023) Perinatal Presentation of a Congenital Intramedullary Capillary Hemangioma in a Neonate Born with Hydrocephalus and Paraplegia. Turk Neurosurg 33:912–915. https://doi.org/10.5137/1019-5149.JTN.41154-22.2 Tunthanathip T, Rattanalert S, Oearsakul T, Kanjanapradit K (2017) Spinal capillary hemangiomas: Two cases reports and review of the literature. Asian J Neurosurg 12:556–562. https://doi.org/10.4103/1793-5482.148793 Lee JS, Lee JY, Park S-H et al (2024) Intramedullary spinal capillary hemangioma with secondary neurulation defect in children. Child’s Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 40:1287–1294. https://doi.org/10.1007/s00381-024-06276-0 Mawk JR, Leibrock LG, McComb RD, Trembath EJ (1987) Metameric capillary hemangioma producing complete myelographic block in an infant. Case report. J Neurosurg 67:456–459. https://doi.org/10.3171/jns.1987.67.3.0456 Zhu K, He D (2016) Intradural extramedullary capillary hemangioma: A case report and review of the literature. Oncol Lett 11:2896–2898. https://doi.org/10.3892/ol.2016.4320 Naruke Y, Horie H, Nagai Y, Ando R (2019) Capillary hemangioma involved in filar lipoma: A case report. Clin Neuropathol 38(1):33–37. https://doi.org/10.5414/NP301117 Steinberger J, Nistal DA, Ghatan S (2019) T3 infantile hemangioma: first case of a tumor involving epidural, intradural extramedullary, and intradural intramedullary spaces. J Neurosurg Pediatr 23(6):694–698. https://doi.org/10.3171/2018.12.PEDS18554 Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 16 Oct, 2025 Read the published version in Child's Nervous System → Version 1 posted Editorial decision: Accepted 10 Oct, 2025 Reviews received at journal 09 Oct, 2025 Reviewers agreed at journal 09 Oct, 2025 Reviewers invited by journal 13 Jul, 2025 Editor assigned by journal 11 Jun, 2025 Submission checks completed at journal 11 Jun, 2025 First submitted to journal 07 Jun, 2025 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. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. 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-6844072","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":484748182,"identity":"f08ecaad-f826-47ae-95e6-e3384e27f2f3","order_by":0,"name":"Ozan Başkurt","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA80lEQVRIiWNgGAWjYJACZiA2AGLGB0BCBsohSgszM0gpD4STQJwWNgmitPDzH372uKCmztjg9vljFT/32PAwsDdvk2D8cQ+nFsmGY+bGM44dNjM4l8x2s+dZGg8Dz7EyCYaEYpxaDA42mEnzsB2wMTjDzHaD58BhHgaJHDOgFtwuMzjM/k2a518dWEvhnwP/eRjk3xDQcozHTJq3jdkMpIWZ58ABoC08+LVI9vCUSc/sO2wseYbZWFrmQDIPG09asUVCGm4t/PzHt0kXfKsz7DvD+PDjmwN2cvzshzfe+GCDWwsmYAMRpGgYBaNgFIyCUYAJAHxWR8Ww09ERAAAAAElFTkSuQmCC","orcid":"","institution":"Istanbul Arel University, Memorial Bahcelievler Hospital","correspondingAuthor":true,"prefix":"","firstName":"Ozan","middleName":"","lastName":"Başkurt","suffix":""},{"id":484748183,"identity":"96c66990-b38e-4e14-8e6d-67076fb32e7d","order_by":1,"name":"Mehmet Arda Inan","email":"","orcid":"","institution":"Acıbadem University","correspondingAuthor":false,"prefix":"","firstName":"Mehmet","middleName":"Arda","lastName":"Inan","suffix":""},{"id":484748184,"identity":"e11c558f-98cf-43c6-b11e-b04c18196586","order_by":2,"name":"Tufan Hicdonmez","email":"","orcid":"","institution":"Istinye University Faculty of Medicine","correspondingAuthor":false,"prefix":"","firstName":"Tufan","middleName":"","lastName":"Hicdonmez","suffix":""}],"badges":[],"createdAt":"2025-06-07 17:08:15","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6844072/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6844072/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s00381-025-06997-w","type":"published","date":"2025-10-16T15:57:34+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":87033317,"identity":"316f6115-8531-4532-a863-340ba9fc4716","added_by":"auto","created_at":"2025-07-18 13:04:51","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":2271945,"visible":true,"origin":"","legend":"\u003cp\u003eSkin stigamata; cutaneous hemangioma\u003c/p\u003e","description":"","filename":"Fig1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6844072/v1/7a0cc1dd2ce0136d245e36fd.jpg"},{"id":87033319,"identity":"bc49d854-201d-4500-9d75-1feb5788b6bb","added_by":"auto","created_at":"2025-07-18 13:04:51","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1806440,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA.\u003c/strong\u003eSagittal T1-weighted, T2- weighted and short tau inversion recovery (STIR) sequences \u003cstrong\u003eB.\u003c/strong\u003e Axial Te-weighted secitons \u003cstrong\u003eC.\u003c/strong\u003e Coronal T2- STIR and T1-weighted images\u003c/p\u003e","description":"","filename":"Fig2page0001.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6844072/v1/09d6134e181a10100f174e86.jpg"},{"id":87033322,"identity":"70526c2c-9d8b-4c25-9006-b67060689f9f","added_by":"auto","created_at":"2025-07-18 13:04:51","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":3873859,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA. \u003c/strong\u003eAfter dural openning\u003cstrong\u003e B. \u003c/strong\u003eDebulking of the tumor\u003cstrong\u003e C. \u003c/strong\u003eUsage of ultrasonic aspiratör\u003cstrong\u003e D. \u003c/strong\u003eDissection from surrounding spinal nerve roots\u003cstrong\u003e E.\u003c/strong\u003eNon-funcitonal aberrant dorsal root\u003cstrong\u003e F. \u003c/strong\u003eMidline dural defect area, within fatty infiltration\u003cstrong\u003e G. \u003c/strong\u003eTransection of the thickened filum terminale \u003cstrong\u003eH. \u003c/strong\u003eCranial portion control via neuromonitozaiton probe\u003cstrong\u003e I. \u003c/strong\u003eClose relation of spinal rootlets and the tumor\u003cstrong\u003e J. \u003c/strong\u003eGross-total resection\u003c/p\u003e","description":"","filename":"Fig3page0001.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6844072/v1/16dd1d04bc35e1f9cf28ee76.jpg"},{"id":87033326,"identity":"b05046f8-a56d-4e0e-8ead-4366cf893d31","added_by":"auto","created_at":"2025-07-18 13:04:51","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":3264781,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA.\u003c/strong\u003e Low-power view of the well-circumscribed lesion surrounded by fat tissue. (Hematoxylin and Eosin stain, x50 magnification) \u003cstrong\u003eB.\u003c/strong\u003e High-power view showing hyalinized endothelial walls with a honeycomb appearance and prominent endothelial cell nuclei. Sparse erythrocytes are visible within the lumen. (Hematoxylin and Eosin stain, x400 magnification)\u003c/p\u003e","description":"","filename":"Fig4page0001.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6844072/v1/0b92cc1b9f755b28cc407219.jpg"},{"id":93956747,"identity":"b9bfed79-023c-4af8-9c23-d603ca4af530","added_by":"auto","created_at":"2025-10-20 16:12:05","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":27578735,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6844072/v1/b11680be-9c8e-43a2-8d6e-f209e07de76a.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Spinal Capillary Hemangioma with Intramedullary and Extramedullary Extension in a Child with Cutaneous Stigmata: A Rare Case","fulltext":[{"header":"Case Presentation","content":"\u003cp\u003eWe report the case of a female infant who presented to our clinic at 8 months of age with a soft tissue mass on her lower back, which had been previously treated with physical therapy. At admission, her head circumference was 45 cm (75th percentile), and the anterior fontanel was small but open. Neurological examination revealed full strength in the lower extremities, with no deformities of the legs or feet. On inspection, there was asymmetry in the mid-lumbar and upper sacral region, with a hyperemic soft tissue mass over the skin in the left paralumbar area (\u003cb\u003eFig.\u0026nbsp;1\u003c/b\u003e).\u003c/p\u003e\u003cp\u003eLumbar MRI revealed findings consistent with dorsal lipomyelomeningocele and a thickened filum terminale, suggestive of a low conus medullaris terminating at the L2 level. A lesion extending distally from the conus medullaris measured approximately 11\u0026times;11\u0026times;61 mm. The superior portion appeared isointense on T1-weighted images and markedly hyperintense on T2-weighted sequences, with signal suppression on fat-saturated images. The inferior part of the lesion demonstrated heterogeneous internal architecture and showed irregular contrast enhancement, raising suspicion for a mature teratoma of the conus medullaris (\u003cb\u003eFig.\u0026nbsp;2\u003c/b\u003e).\u003c/p\u003e\u003cp\u003eInitial urodynamic evaluation was normal, and the patient was placed under follow-up. At 13 months, a control MRI using short tau inversion recovery (STIR) sequences was obtained. By this time, the patient had begun walking and her developmental milestones were age-appropriate. However, she exhibited right foot inversion and a positive pes varus deformity. Due to the emergence of new urodynamic abnormalities, surgical intervention was indicated.\u003c/p\u003e\u003cp\u003eUnder intraoperative neuromonitoring, laminoplasty was performed at L1\u0026ndash;L5 levels, with osteoblastic laminar flaps retracted cranially. A partial spina bifida was noted at S1, with a soft tissue mass replacing the bony elements; laminectomy was performed at this level. A midline dural defect was identified and carefully opened bilaterally. Controlled cerebrospinal fluid drainage was achieved. A reddish-brown, moderately firm intradural tumor extending from the conus medullaris to the L4 level was identified, containing both intra- and extramedullary components with areas of vascularity (\u003cb\u003eFig.\u0026nbsp;3\u003c/b\u003e).\u003c/p\u003e\u003cp\u003eThe lesion was carefully dissected from surrounding spinal nerve roots and confirmed with neuromonitoring at L2\u0026ndash;S2 levels. First debulking was made via ultrasonic aspirator. At caudal part, an aberrant dorsal root adherent to the dura was identified; it was found to be non-functional on EMG and was sacrificed. Than a thickened filum terminale was transected to relieve cord tension. The tumor had a cranially displaced component tightly adherent to the conus; grosstotal resection was performed at this site to preserve function. The subcutaneous component, which appeared firm and fibrolipomatous, was gross-totally resected.\u003c/p\u003e\u003cp\u003eThe postoperative course was uneventful, and the child was discharged on the fifth day without new neurological deficits. Histopathology revealed features consistent with capillary hemangioma (\u003cb\u003eFig.\u0026nbsp;4\u003c/b\u003e), including low mitotic activity and diffuse CD34 positivity. At 1-year follow-up, the patient remained asymptomatic.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eSpinal capillary hemangiomas are rare benign vascular tumors, particularly uncommon in the pediatric population [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. While they most frequently occur in adults, pediatric presentations remain exceptional, with fewer than 15 cases reported to date [\u003cspan additionalcitationids=\"CR3 CR4\" citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. Only 7 of these cases were associated with cutaneous stigmata, making our case the 14th pediatric spinal capillary hemangioma and the 8th to feature concurrent skin findings [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eThe classification of spinal capillary hemangiomas includes four subtypes, with the pediatric variant typically manifesting as an intradural-extramedullary mass connected to a cutaneous lesion via a sinus tract [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Most reported cases in children involve the cauda equina or conus medullaris and are extramedullary in location [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. The uniqueness of our case lies in its combined intramedullary and extramedullary configuration, extending from the conus medullaris to L4, with an additional dorsal subcutaneous component. To the best of our knowledge, such topographical involvement in a child has not been previously reported.\u003c/p\u003e\u003cp\u003eThe patient initially presented with a dorsal skin lesion at birth but maintained intact neurological function until 13 months of age, when she developed gait disturbance and new-onset urodynamic abnormalities. MRI revealed a complex lesion with fat signal components, heterogeneous contrast enhancement, and caudal extension, initially raising suspicion for mature teratoma. This highlights the diagnostic challenge, as similar imaging characteristics can be seen in other entities such as lipomyelomeningocele, teratoma, or epidermoid tumors [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eSurgical exploration under intraoperative neuromonitoring revealed a reddish-brown, moderately firm lesion adherent to the conus, with both intra- and extramedullary components. Gross-total resection was achieved except for a portion tightly adherent to the conus medullaris, which was left in place to preserve neurological function. Histopathological findings, including lobular capillary structures and diffuse CD34 positivity, confirmed the diagnosis of capillary hemangioma [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eFrom an embryological perspective, the association of this lesion with a thickened filum terminale, partial spina bifida, and cutaneous hemangioma suggests a possible error during secondary neurulation. This theory has been proposed in similar cases where capillary hemangiomas were found within or adjacent to filar lipomas [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e], indicating a metameric distribution of pluripotent progenitor cells [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eOur case also resembles a rare presentation described by Steinberger et al., in which a capillary hemangioma extended across the epidural, intradural, and intramedullary compartments in an infant, underscoring the need for high suspicion and individualized surgical planning in pediatric patients with atypical spinal lesions [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e].\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThis case illustrates a rare instance of a pediatric spinal capillary hemangioma with both intramedullary and extramedullary components, associated with cutaneous stigmata and tethered cord syndrome. The coexistence of skin findings and a complex spinal mass should raise suspicion for vascular tumors, including capillary hemangioma, in the differential diagnosis\u0026mdash;particularly when imaging reveals fat signal characteristics and heterogeneous enhancement [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eSurgical resection remains the mainstay of treatment, with intraoperative neuromonitoring playing a crucial role in preserving neurological function during dissection of adherent components [\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. The embryological link between dorsal skin lesions, filar abnormalities, and intraspinal vascular tumors underscores the importance of early neuroimaging in patients presenting with cutaneous stigmata suggestive of occult spinal dysraphism [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eTo our knowledge, this is the first reported pediatric case of a spinal capillary hemangioma exhibiting both intramedullary and extramedullary components along with a dorsal cutaneous lesion. Early recognition and surgical management can prevent irreversible neurological deficits and contribute to a favorable long-term outcome.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements:\u0026nbsp;\u003c/strong\u003eNone.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eDeclaration of Interests:\u003c/strong\u003e The authors have no relevant financial or non-financial interests to disclose.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding:\u003c/strong\u003e The authors did not receive support from any organization for the submitted work.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent:\u003c/strong\u003e Written informed consent was obtained from the\u0026nbsp;legally authorized representative\u0026nbsp;of the patient for publication of this case report and any accompanying images.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical statement:\u0026nbsp;\u003c/strong\u003eThe need for\u0026nbsp;ethical\u0026nbsp;approval was\u0026nbsp;waived\u0026nbsp;by\u0026nbsp;Istinye University Ethics Committee\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026rsquo; Contribution:\u003c/strong\u003e Study conception and design: OB, MAI, TH. Data collection, surgery: OB, TH. Pathological evaluation: MAI. Analysis and interpretation of results: OB, MAI, TH. Draft manuscript preparation: OB, TH. Critical revision of the article: TH. Study supervision: TH. All authors (OB, MAI, TH) reviewed the results and approved the final version of the manuscript\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData Availability:\u0026nbsp;\u003c/strong\u003eAll data generated or analyzed during this study are included in this article. Further enquiries can be directed to the corresponding author.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eIannelli A, Lupi G, Castagna M et al (2005) Intramedullary capillary hemangioma associated with hydrocephalus in an infant. J Neurosurg 103:272\u0026ndash;276. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3171/ped.2005.103.3.0272\u003c/span\u003e\u003cspan address=\"10.3171/ped.2005.103.3.0272\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKarikari IO, Selznick LA, Cummings TJ, George TM (2007) Spinal capillary hemangioma in infants: report of two cases and review of the literature. Pediatr Neurosurg 43:125\u0026ndash;129. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1159/000098386\u003c/span\u003e\u003cspan address=\"10.1159/000098386\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eOhadi M, Maroufi SF, Meybodi KT et al (2023) Perinatal Presentation of a Congenital Intramedullary Capillary Hemangioma in a Neonate Born with Hydrocephalus and Paraplegia. Turk Neurosurg 33:912\u0026ndash;915. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.5137/1019-5149.JTN.41154-22.2\u003c/span\u003e\u003cspan address=\"10.5137/1019-5149.JTN.41154-22.2\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eTunthanathip T, Rattanalert S, Oearsakul T, Kanjanapradit K (2017) Spinal capillary hemangiomas: Two cases reports and review of the literature. Asian J Neurosurg 12:556\u0026ndash;562. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.4103/1793-5482.148793\u003c/span\u003e\u003cspan address=\"10.4103/1793-5482.148793\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eLee JS, Lee JY, Park S-H et al (2024) Intramedullary spinal capillary hemangioma with secondary neurulation defect in children. Child\u0026rsquo;s Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 40:1287\u0026ndash;1294. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.1007/s00381-024-06276-0\u003c/span\u003e\u003cspan address=\"10.1007/s00381-024-06276-0\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMawk JR, Leibrock LG, McComb RD, Trembath EJ (1987) Metameric capillary hemangioma producing complete myelographic block in an infant. Case report. J Neurosurg 67:456\u0026ndash;459. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3171/jns.1987.67.3.0456\u003c/span\u003e\u003cspan address=\"10.3171/jns.1987.67.3.0456\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eZhu K, He D (2016) Intradural extramedullary capillary hemangioma: A case report and review of the literature. Oncol Lett 11:2896\u0026ndash;2898. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3892/ol.2016.4320\u003c/span\u003e\u003cspan address=\"10.3892/ol.2016.4320\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eNaruke Y, Horie H, Nagai Y, Ando R (2019) Capillary hemangioma involved in filar lipoma: A case report. Clin Neuropathol 38(1):33\u0026ndash;37. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.5414/NP301117\u003c/span\u003e\u003cspan address=\"10.5414/NP301117\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSteinberger J, Nistal DA, Ghatan S (2019) T3 infantile hemangioma: first case of a tumor involving epidural, intradural extramedullary, and intradural intramedullary spaces. J Neurosurg Pediatr 23(6):694\u0026ndash;698. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003ehttps://doi.org/10.3171/2018.12.PEDS18554\u003c/span\u003e\u003cspan address=\"10.3171/2018.12.PEDS18554\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"childs-nervous-system","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"cnsy","sideBox":"Learn more about [Child's Nervous System](http://link.springer.com/journal/381)","snPcode":"381","submissionUrl":"https://submission.nature.com/new-submission/381/3","title":"Child's Nervous System","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Capillary hemangioma, Cutaneous stigmata, Intramedullary lesion, Pediatric spinal tumor, Spinal dysraphism, Tethered cord","lastPublishedDoi":"10.21203/rs.3.rs-6844072/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6844072/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eWe report a rare case of a pediatric spinal capillary hemangioma with both intramedullary and extramedullary components in association with a dorsal cutaneous lesion. The patient, an 13-month-old girl, initially presented with a soft tissue mass on her lower back. MRI revealed a lesion extending from the conus medullaris to L4, initially misinterpreted as a mature teratoma. Due to the emergence of new urodynamic abnormalities, surgical exploration revealed a reddish-brown, moderately firm tumor with tethering of the spinal cord, which was partially resected under intraoperative neuromonitoring. Histopathological findings confirmed capillary hemangioma. To date, only 13 pediatric cases have been reported, and our case represents the first with combined intra- and extramedullary involvement and cutaneous stigmata. This case highlights the importance of including vascular tumors in the differential diagnosis of spinal dysraphism with skin markers and underscores the value of early surgical intervention to prevent neurological decline.\u003c/p\u003e","manuscriptTitle":"Spinal Capillary Hemangioma with Intramedullary and Extramedullary Extension in a Child with Cutaneous Stigmata: A Rare Case","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-07-18 13:04:46","doi":"10.21203/rs.3.rs-6844072/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Accepted","date":"2025-10-10T08:53:39+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-10-09T15:16:33+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"64079867412767396964046532953512770731","date":"2025-10-09T15:01:49+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-07-13T20:23:03+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-06-11T04:27:35+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-06-11T04:27:01+00:00","index":"","fulltext":""},{"type":"submitted","content":"Child's Nervous System","date":"2025-06-07T17:00:00+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"childs-nervous-system","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"cnsy","sideBox":"Learn more about [Child's Nervous System](http://link.springer.com/journal/381)","snPcode":"381","submissionUrl":"https://submission.nature.com/new-submission/381/3","title":"Child's Nervous System","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"cd75fc2c-7ea2-4afc-9f12-e3a1d3c75cd6","owner":[],"postedDate":"July 18th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-10-20T16:08:44+00:00","versionOfRecord":{"articleIdentity":"rs-6844072","link":"https://doi.org/10.1007/s00381-025-06997-w","journal":{"identity":"childs-nervous-system","isVorOnly":false,"title":"Child's Nervous System"},"publishedOn":"2025-10-16 15:57:34","publishedOnDateReadable":"October 16th, 2025"},"versionCreatedAt":"2025-07-18 13:04:46","video":"","vorDoi":"10.1007/s00381-025-06997-w","vorDoiUrl":"https://doi.org/10.1007/s00381-025-06997-w","workflowStages":[]},"version":"v1","identity":"rs-6844072","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6844072","identity":"rs-6844072","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}