Dural ectasia in neurofibromatosis Type 1

Bone ◽  
2007 ◽  
Vol 40 (6) ◽  
pp. S83-S84
Author(s):  
D.H. Viskochil ◽  
D.A. Stevenson
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Dural Ectasia

Dystrophic Lumbar Kyphoscoliosis Associated with Giant Dural Ectasia in a 19-Year-Old Patient with Neurofibromatosis Type 1. Case Report

2020 ◽  
Vol 2 (10) ◽  
pp. 1926-1930
Author(s):  
Nicolas Plais ◽  
Peter H. Connolly ◽  
Renaud Lafage ◽  
Debra Jacobs ◽  
Virginie Lafage ◽  
...  
Keyword(s):  
Case Report ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Dural Ectasia

Neurofibromatosis type 1 with dural ectasia

2012 ◽  
Vol 39 (7) ◽  
pp. 655-656 ◽  
Author(s):  
Woo Jin LEE ◽  
Oun Jae PARK ◽  
Chong Hyun WON ◽  
Sung Eun CHANG ◽  
Mi Woo LEE ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Dural Ectasia

Dural Ectasia of the Optic Nerve Sheath in Neurofibromatosis Type 1

1994 ◽  
Vol 18 (5) ◽  
pp. 728-730 ◽  
Author(s):  
Karl O. Lövblad ◽  
Luca Remonda ◽  
Christoph Ozdoba ◽  
Peter Huber ◽  
Gerhard Schroth
Keyword(s):  
Optic Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Nerve Sheath ◽  
Dural Ectasia ◽  
Nerve Sheath

scholarly journals A Large Intrathoracic Mass in A Patient With Neurofibromatosis Type 1

2021 ◽  
Vol 7 (2) ◽  
pp. 107-112
Author(s):  
Babak Alijani ◽  
◽  
Mahsa Pakseresht Mogharab ◽  
Amin Naseri ◽  
◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Cord Compression ◽  
Tumor Formation ◽  
Spinal Deformities ◽  
Dural Ectasia ◽  
X Ray ◽  
Dural Sac ◽  
Chest X Ray

Background and Importance: Dural ectasia is circumferential extension or dilatation of the dural sac, it is commonly associated with Neurofibromatosis type 1 (NF1). Because NF1 is associated with a high likelihood of tumor formation, it is critical to distinguish it from other posterior mediastinal cancers such as neurofibroma, neuroblastoma, and ganglioneuroma. Scoliosis is the most frequently observed feature in NF1 patients with spinal deformities. Moreover, there are several distinctive radiographic features, including dural ectasia, defective pedicles, and spondylolisthesis, which are relatively less common in these patients. Surgery may be performed for stabilization of the spine. Case Presentation: The present study reports on a 62-year-old female with neurofibromatosis type 1 who was referred for an unusual chest X-ray and chest Computed Tomography (CT) revealing a thoracic mass while she was hospitalized and under observation for dyspnea. The chest X-ray film showed homogenous opacity with a well-defined margin in the right apex. The chest CT showed an enlarged intervertebral foramen and defected vertebral arch around the mass and scoliosis. Although the pre-operative diagnosis was dumbbell type neurinoma, the tumor was found to be a protrusion of dura mater with spinal fluid out of the spinal canal. Part of the wall was excised, the residual opening was repaired, and surgical stabilization was performed. Conclusion: Retrospectively, magnetic resonance imaging showed the characteristics of thoracic dural ectasia and anterior meningocele, which, in an asymptomatic case, require regular radiographic follow-ups. Surgical intervention is an alternative for patients with spinal deformities or symptomatic patients due to the adverse effects of spinal cord compression and mediastinal structures.

scholarly journals Sudden bilateral lower limb paralysis with dural ectasia in Neurofibromatosis type 1

2021 ◽  
Vol 2 (9) ◽  
pp. e0165
Author(s):  
Victor Mutua ◽  
Newnex Mong’are ◽  
Brian Bundi ◽  
Chris von Csefalvay ◽  
David Oriko ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Lower Limb ◽  
Neurofibromatosis Type ◽  
Dural Ectasia ◽  
Lower Limb Paralysis ◽  
Bilateral Lower Limb

Calvarial defects and skeletal dysplasia in patients with neurofibromatosis Type 1

2013 ◽  
Vol 11 (4) ◽  
pp. 410-416 ◽  
Author(s):  
Daniel K. Arrington ◽  
Amy R. Danehy ◽  
Analise Peleggi ◽  
Mark R. Proctor ◽  
Mira B. Irons ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Plexiform Neurofibroma ◽  
Neurofibromatosis Type ◽  
Skull Defect ◽  
Dural Ectasia ◽  
Skull Defects ◽  
Calvarial Defects ◽  
Sphenoid Dysplasia ◽  
The Mean

Object Skull defects, including sphenoid dysplasia and calvarial defects, are rare but distinct findings in patients with neurofibromatosis Type 1 (NF1). The underlying pathophysiology is unclear. The goal of this study was to identify the clinical characteristics and natural history of skull defects in patients with NF1. Methods An electronic search engine of medical records was used to identify patients with NF1 and bony skull anomalies. All clinical, radiographic, pathology, and operative reports were reviewed. The relationship between bony anomalies and significant clinical associations was evaluated. This study received institutional review board approval. Results Twenty-one patients were identified. The mean age at NF1 diagnosis was 4.2 years. The mean age at skull defect diagnosis was 8.8 years (9.7 years in the sphenoid wing dysplasia group and 11.9 years in the calvarial defect group). Sphenoid dysplasia was associated with a plexiform neurofibroma or dural ectasia in 73.3% and 80.0% of cases, respectively. Calvarial defects were associated with a plexiform neurofibroma or dural ectasia in 66.7% and 33.3% of patients, respectively. An absence of either an associated neurofibroma or ectasia was not noted in any patient with sphenoid wing dysplasia or 25.0% of those with calvarial defects. In 6 patients, both types of skull defects presented simultaneously. Serial imaging studies were obtained for a mean follow-up time of 7.5 years (range 0.4–20.0 years). Of these patients with serial imaging, radiographic progression was found in 60% of cases of calvarial defects and 56% of cases of sphenoid wing dysplasia. Two patients underwent surgical repair of a skull defect, and both required repeat procedures. Conclusions The majority of skull defects in patients with NF1 were associated with an adjacent structural lesion, such as a plexiform neurofibroma or dural ectasia. This findings from this cohort also support the concept of progression in defect size in more than half of the patients. Potential mechanisms by which these secondary lesions contribute to pathogenesis of the bony defect may include changes in the bony microenvironment. A better understanding of the pathophysiology of skull defects will help guide detection, improve treatment and outcome, and may contribute to the understanding of the pathogenesis of bony lesions in NF1.

scholarly journals Characterisation of a Novel Radiological Entity in Neurofibromatosis Type 1 - Diffuse Neurofibromatous Tissue

2021 ◽  
Author(s):  
Venkata Amruth Nadella ◽  
K. Joshi George ◽  
Calvin Soh
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Sacroiliac Joint ◽  
Bone Erosion ◽  
Mass Effect ◽  
Neurofibromatosis Type ◽  
Descriptive Study ◽  
Dural Ectasia ◽  
Mri Features ◽  
Mri Scans

Objectives: To describe the prevalence, demographics and characteristics of a novel radiological entity in neurofibromatosis type 1: diffuse neurofibromatous tissue (DNFT) Design: Aretrospective, descriptive review of MDT and radiology notes. Methods: Of the 1049 patients from the NF1 adult radiology MDT minutes (2009–2021), 77 patients with DNFT were identified and clinical data were collected. MRI scans from 20 DNFT cases were interpreted. Results: Although overall gender distribution of DNFT was roughly even, it was more prevalent in females (73.9%) at the sacroiliac joint—where this entity was most common (29.9%). DNFT often involves the fibrous part of the sacroiliac joint and is seen as diffuse, streaky infiltrating tissues that cause bone erosion without mass effect. The period prevalence of scoliosis and dural ectasia on corresponding spinal levels with spinal DNFT was 62.8 and 51.2%, respectively (n=43). Conclusions: This is the first reported descriptive study of DNFT in NF1 and the first to describe its MRI features in detail. The predilection for the sacroiliac joint and the possible associations with scoliosis and dural ectasia provide important insights that can form the basis for future studies whilst also suggesting the need for active surveillance of this tissue in NF1 patients.

scholarly journals Neurofibromatosis Type 1 Associated with Dural Ectasia: A Case Report

2007 ◽  
Vol 56 (3) ◽  
pp. 370-374
Author(s):  
Tatsuya Okada ◽  
Akira Sei ◽  
Toru Fujimoto ◽  
Masaya Mizutamari ◽  
Takuya Taniwaki ◽  
...  
Keyword(s):  
Case Report ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Dural Ectasia
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