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      Spontaneous Lateral Sphenoid Cephalocele in Association with Idiopathic Intracranial Hypertension: A Case Report Translated title: 특발성 두개 내 고혈압과 관련된 자발성 측면 접형골형 뇌류: 증례 보고

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          Abstract

          Spontaneous lateral sphenoid cephalocele (SLSC) is the herniation of intracranial contents through a bony defect in the lateral sphenoid, without predisposing factors. SLSC pathogenesis is associated with idiopathic intracranial hypertension (IIH); however, the relationship between IIH and SLSC is not fully understood due to the limited number of published case reports. Here, we report a unique case of SLSC in a 39-year-old female who presented with a combination of a lateral sphenoid cephalocele and multiple radiologic findings indicative of IIH, some of which have never been described in previously published case reports. This case highlights the possibility that chronically elevated intracranial pressure may be a determining factor in SLSC development.

          Translated abstract

          자발성 측면 접형골형 뇌류는 어떠한 선행 요인 없이 측면 접형골의 결손을 통하여 두개 내 구조물이 탈출하는 것을 의미한다. 자발성 측면 접형골형 뇌류의 발병 원인은 특발성 두개 내 고혈압이 동반되어 발생하는 것으로 여겨지고 있다. 그러나, 이에 대한 연구는 제한적으로, 추가적인 이해와 연구가 필요하다. 이에 우리는 39세 여성에서 발생한 특이한 자발성 측면 접형골형 뇌류 증례를 보고하고자 한다. 이 증례 환자의 뇌 영상 사진에서 측면 접형골형 뇌류와 특발성 두개 내 고혈압을 시사하는 여러 영상학적 소견들의 조합을 확인할 수 있으며, 이 중 일부는 이전에 보고된 다른 증례에서는 함께 기술된 적이 없는 소견이다. 이는 만성적으로 상승한 두개 내 압력이 자발적 측면 접형골형 뇌류의 발생에 결정적인 영향을 준다는 가설을 더욱 지지해 준다.

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          Most cited references10

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          Imaging of skull base cerebrospinal fluid leaks in adults.

          Cerebrospinal fluid (CSF) leak occurs when there is an osseous and dural defect at the skull base, with direct communication of the subarachnoid space to the extracranial space, usually a paranasal sinus. Recognition of the leak site and source and appropriate treatment are necessary to avoid rhinorrhea or otorrhea, low-pressure headaches, and meningitis, known complications of CSF leak. The imaging evaluation has evolved over the past several decades. Description of current techniques available to direct treatment options, including multidetector thin-section computed tomography, and imaging recommendations are presented. RSNA, 2008
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            Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension.

            Previous reports indicate that elevated intracranial pressure (ICP) may cause spontaneous cerebrospinal fluid (CSF) leaks. In this study we examined the clinical diagnosis of benign intracranial hypertension (BIH) in this population using the modified Dandy criteria. We performed a retrospective review of clinical data and measurements of ICPs after surgical repair. Sixteen patients with spontaneous CSF leaks were surgically treated from 1996 to 2002. In 11 patients with CSF pressure measurements, strict adherence to the modified Dandy criteria definitively confirmed a diagnosis of BIH in 8 patients (72%) and a likely diagnosis in the 3 remaining patients. The mean ICP was 31.1 cm H20 (range, 17.3 to 52 cm H2O), and 81% of the patients were obese middle-aged women. Clinically, all patients had signs and/or symptoms of elevated ICP, such as headache (91%), pulsatile tinnitus (45%), hypertension (45%), balance problems (27%), and visual complaints (18%). Surgical repair was 100% successful in leak cessation over a mean follow-up of 14.1 months. Most patients with spontaneous CSF leaks fulfill the modified Dandy criteria; thus, this disorder appears to be a variant of BIH. Further investigation is needed to determine the exact cause of elevated CSF pressures in this group and whether medical or surgical treatments to correct the intracranial hypertension are warranted.
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              Spontaneous CSF leaks: a paradigm for definitive repair and management of intracranial hypertension.

              To report our outcomes with the repair of spontaneous cerebrospinal fluid (CSF) leaks and to demonstrate how management of underlying intracranial hypertension improves outcomes. Retrospective review of spontaneous CSF leaks treated at the University of Pennsylvania Health System from 1996 to 2006. Data collected included demographics, nature of presentation, body mass index (BMI), site of skull base defect, surgical approach, intracranial pressure, and clinical follow-up. Fifty-six patients underwent repair of spontaneous CSF leaks. Eighty-two percent (46 of 56) were obese (average BMI 36.2 kg/m(2)). Nine patients had multiple CSF leaks. Fifty-four patients (96%) had associated encephaloceles. Fifty-three CSF leaks (95%) were successfully repaired at first attempt (34 months of follow-up). Intracranial pressures averaged 27 cm H(2)O. Patients were treated with acetazolamide or, in severe cases, with a ventriculoperitoneal shunt. Spontaneous CSF leaks have the highest recurrence rate of any etiology. With treatment of underlying intracranial hypertension coupled with endoscopic repair, the success rate (95%) approaches that of other etiologies of CSF leaks.

                Author and article information

                Journal
                J Korean Soc Radiol
                J Korean Soc Radiol
                JKSR
                Journal of the Korean Society of Radiology
                The Korean Society of Radiology
                2951-0805
                September 2024
                02 August 2024
                : 85
                : 5
                : 937-942
                Affiliations
                Department of Radiology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea. 인제대학교 의과대학 일산백병원 영상의학과
                Author notes
                Corresponding author: Byunghoon Lee, MD. Department of Radiology, Ilsan Paik Hospital, Inje University College of Medicine, 170 Juhwa-ro, Ilsanseo-gu, Goyang 10380, Korea. Tel 82-31-910-7694, Fax 82-31-910-7366, hoonbeer@ 123456hanmail.net
                Article
                10.3348/jksr.2023.0150
                11473977
                39416321
                cd0489a5-9893-4c5a-b250-357f2e22cbb8
                Copyrights © 2024 The Korean Society of Radiology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( https://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
                : 04 December 2023
                : 21 January 2024
                : 04 March 2024
                Categories
                Neuroradiology & Neurointervention
                Case Report

                cephalocele,sphenoid bone,idiopathic intracranial hypertension,computed tomography,magnetic resonance imaging

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