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Altered cerebrospinal fluid dynamics in neurofibromatosis type l: severe arachnoid thickening in patients with neurofibromatosis type 1 may cause abnormal CSF dynamic

 Young Sill Kang  ;  Eun-Kyung Park  ;  Yong-Oock Kim  ;  Ju-Seong Kim  ;  Dong-Seok Kim  ;  U. W. Thomale  ;  Kyu-Won Shim 
 CHILDS NERVOUS SYSTEM, Vol.33(5) : 767-775, 2017 
Journal Title
Issue Date
Adolescent ; Adult ; Arachnoid/diagnostic imaging* ; Arachnoid/surgery ; Cerebrospinal Fluid Pressure/physiology* ; Child ; Female ; Humans ; Hydrodynamics* ; Male ; Neurofibromatosis 1/complications ; Neurofibromatosis 1/diagnostic imaging* ; Neurofibromatosis 1/surgery ; Retrospective Studies ; Severity of Illness Index* ; Temporal Lobe/diagnostic imaging* ; Temporal Lobe/surgery
INTRODUCTION: The object of this study is to understand abnormal dynamic of cerebrospinal fluid (CSF) in patients with neurofibromatosis type 1 (NF1), which may cause temporal lobe herniation and bulging of temporal fossa. METHODS: Four patients, three females and one male, with NF1 were studied retrospectively. They presented with a similar craniofacial deformity, which consisted of pulsatile exophthalmos, an enlarged bony orbit, dysplasia of the sphenoid wing with the presence of a herniation of the temporal lobe into the orbit, and a bulging temporal fossa. RESULTS AND DISCUSSION: Surgical exploration demonstrated abnormally thickened arachnoid membrane in one case. Protruding temporal lobe, which was one of the main symptoms in NF1 patients, could be stopped by control of intracranial pressure (ICP) via programmable ventriculoperitoneal shunt (VPS) or extra ventricle drainage implantation. The dense fibrosis of the arachnoid membrane and consequent altered hemispheric CSF dynamics may cause symptoms including pulsatile exophthalmos and consequent worsening of vision, prolapse of the temporal lobe, and enlargement of the temporal fossa. This finding may not present with general features of hydrocephalus, so that delays in diagnosis often result. CONCLUSION: For the NF1 patients with cranio-orbito-temporal deformities, prior to any surgical reconstruction, control of increased ICP (IICP) should be primarily considered.
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1. College of Medicine (의과대학) > Dept. of Neurosurgery (신경외과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Dong Seok(김동석)
Kim, Ju Seong(김주성)
Park, Eun Kyung(박은경)
Shim, Kyu Won(심규원) ORCID logo https://orcid.org/0000-0002-9441-7354
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