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      Pediatric Nondysraphic Intramedullary Lipoma : Report of Two Cases and Review of the Literature

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          Abstract

          Pediatric nondysraphic intramedullary lipoma is very rare, and only limited cases have been reported. In the present case, we present two infant patients with these pathologies who were surgically treated. Previous literature on 20 patients with these diseases who had undergone surgical treatments was analyzed. Surgical treatment should be considered in most symptomatic patients, and laminoplastic laminotomy and internal debulking of the lipoma under intraoperative neurophysiological monitoring are mostly recommended.

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          Most cited references18

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          Intramedullary spinal cord lipomas.

          Spinal cord lipomas are rare lesions, accounting for approximately 1% of all spinal cord tumors. True intramedullary spinal cord lipomas are extremely rare and are represented in the literature as scattered, single case reports. The authors present a series of six patients with intramedullary spinal cord lipomas managed at our institution from July, 1985 to July, 1993. The patients' ages ranged from 8 to 45 years. Four patients presented with newly diagnosed tumors and two had undergone previous surgery. Patients usually presented with long histories of disability followed by rapid progression of their symptoms. Most patients were in poor neurological condition on presentation. Presenting symptoms included spinal pain, dysesthetic sensory changes, gait difficulties, weakness, and incontinence. Three patients had cervical tumors, two had cervicothoracic tumors, and one patient had a thoracic tumor. Diagnostic studies, including magnetic resonance imaging, were obtained in all patients. No patient exhibited any form of spinal dysraphism or had a dural defect. All patients underwent decompressive, subtotal resections of 40% to 70% of their lesions. Follow-up times ranged from 12 to 96 months. All patients had resolution of their pain, but they generally showed no neurological improvement. As of their most recent follow-up visit, none of the patients was neurologically normal; three can function independently, although with neurological deficits. The other three patients cannot function independently and have severe neurological deficits. The authors conclude that patients with intramedullary spinal cord lipoma who present with significant neurological compromise have a very poor prognosis with regard to neurological function and generally show no improvement with surgical resection.
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            Asymptomatic lumbosacral lipomas--a natural history study.

            Inevitable deterioration due to mechanical tethering is perceived as the natural history for complex congenital spinal lipomas of the conus medullaris region, even if asymptomatic at presentation. The conventional wisdom that prophylactic surgical untethering improves outcome has been challenged recently [1, 2]. This study examines the natural history of asymptomatic un-operated children with lumbosacral lipomas (LSL) and investigates whether predictive factors herald deterioration.
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              Lipomyelomeningocele: pathology, treatment, and outcomes.

              Lipomyelomeningocele represents a rare but complex neurological disorder that may present with neurological deterioration secondary to an inherent tethered spinal cord. Radiological testing is beneficial in determining the morphology of the malformation. Specialized testing such as urodynamic studies and neurophysiological testing may be beneficial in assessing for neurological dysfunction secondary to the lipomyelomeningocele. Early surgical intervention may be beneficial in preventing further neurological decline.
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                Author and article information

                Journal
                J Korean Neurosurg Soc
                J Korean Neurosurg Soc
                JKNS
                Journal of Korean Neurosurgical Society
                Korean Neurosurgical Society
                2005-3711
                1598-7876
                March 2023
                8 September 2022
                : 66
                : 2
                : 211-218
                Affiliations
                [1 ]Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
                [2 ]Department of Pediatrics, Pusan National University Hospital, Pusan National University School of Medicine and Biochemical Research Institute, Busan, Korea
                [3 ]Department of Pediatrics, Chung-Ang University Hospital, College of Medicine, Chung-Ang University, Seoul, Korea
                Author notes
                Address for correspondence : Ji Hoon Phi Division of Pediatric Neurosurgery, Seoul National University Children’s Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea Tel : +82-2-2072-3639, Fax : +82-2-744-8459, E-mail : phi.jihoon@ 123456gmail.com
                Author information
                http://orcid.org/0000-0003-1423-2681
                http://orcid.org/0000-0002-4689-8974
                http://orcid.org/0000-0002-9825-8194
                http://orcid.org/0000-0002-0039-0083
                http://orcid.org/0000-0002-9603-5843
                Article
                jkns-2022-0156
                10.3340/jkns.2022.0156
                10009248
                36071566
                8d2e960a-3fe8-45db-8f65-51c3e8c99166
                Copyright © 2023 The Korean Neurosurgical Society

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 1 July 2022
                : 28 August 2022
                : 6 September 2022
                Categories
                Case Report

                Surgery
                spinal cord neoplasms,lipoma,neurologic manifestations,laminoplasty
                Surgery
                spinal cord neoplasms, lipoma, neurologic manifestations, laminoplasty

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