Intermittent atlantoaxial subluxation caused by a prolapsing neurofibroma

2008 ◽  
Vol 8 (3) ◽  
pp. 288-291 ◽  
Author(s):  
Michael O. Kelleher ◽  
Nasir A. Quarishi ◽  
Gamaliel Tan ◽  
Abhijit Guha ◽  
Eric M. Massicotte
Keyword(s):  
Neurofibromatosis Type ◽  
Cord Compression ◽  
Preoperative Imaging ◽  
Cervical Cord ◽  
Unique Case ◽  
Unusual Lesion ◽  
Operative Findings ◽  
High Cervical ◽  
Cervicomedullary Compression

✓In this report, the authors describe a unique case of intermittent high cervical cord compression caused by a prolapsing neurofibroma at the C1–2 level. This 21-year-old man with known neurofibromatosis Type 1 presented with a mass between the anterior arch of the atlas and the odontoid peg, causing atlantoaxial dissociation and cord compression. The cervicomedullary compression appeared to be caused in part by the neurofibroma but also by the abnormal alignment and thickening of the ligaments between the clivus and C-2. Preoperative imaging repeated on the morning of surgery revealed that the atlantoaxial dissociation had reduced with relief of cord compression and the lesion prolapsed inferiorly. The authors discuss this unusual lesion and describe the associated operative findings and surgical management.

scholarly journals Newborn Apnea Caused by a Neurofibroma at the Craniocervical Junction

1994 ◽  
Vol 21 (1) ◽  
pp. 64-66 ◽  
Author(s):  
David B. Clarke ◽  
Jean-Pierre Farmer ◽  
José L. Montes ◽  
Gordon V. Watters ◽  
Guy Rouleau
Keyword(s):  
Neurofibromatosis Type ◽  
Craniocervical Junction ◽  
Cervical Cord ◽  
Subtotal Resection ◽  
Upper Respiratory Tract ◽  
Brainstem Compression ◽  
Upper Cervical ◽  
First Time ◽  
Dependent Patient

ABSTRACT:The authors report, for the first time, the finding by magnetic resonance imaging of a neurofibroma at the craniocervical junction with upper cervical cord and lower brainstem compression causing complete apnea from birth. Subsequent subtotal resection of the neurofibroma resulted in the successful extubation of a previously ventilator-dependent patient. After a two month period of breathing spontaneously, the newborn developed an upper respiratory tract infection and was reintubated. The patient, unable to be weaned off of the respirator, was extubated and expired shortly thereafter, at the age of five months. The authors suggest that in newborns with unexplained apnea, MRI of the craniocervical junction is indicated. Certain patients may be discovered who have less compromised cervicomedullary function and are afflicted by less aggressive forms of neurofibromatosis type 1. These patients may benefit permanently from a surgical decompression.

scholarly journals Pericranial and scalp rotation flaps for occipitocervical hardware exposure with CSF leak in rheumatoid arthritis patient: A case report and review of the literature

2021 ◽  
Vol 12 ◽  
pp. 229
Author(s):  
Claudio Schonauer ◽  
Ciro Mastantuoni ◽  
Oreste de Divitiis ◽  
Francesco D’Andrea ◽  
Raffaele de Falco ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Cord Compression ◽  
Craniocervical Junction ◽  
Csf Leak ◽  
Cervical Cord ◽  
Congenital Deformity ◽  
Rotation Flap ◽  
Pull Out ◽  
Csf Leakage ◽  
High Cervical

Background: There are several etiologies of craniocervical junction instability (CCJI); trauma, rheumatoid arthritis (RA), infections, tumors, congenital deformity, and degenerative processes. These conditions often require surgery and craniocervical fixation. In rare cases, breakdown of such CCJI fusions (i.e., due to cerebrospinal fluid [CSF] leaks, infection, and wound necrosis) may warrant the utilization of occipital periosteal rescue flaps and scalp rotation flaps to achieve adequate closure. Case Description: A 33-year-old female with RA, cranial settling, and high cervical cord compression underwent an occipitocervical instrumented C0–C3/C4 fusion. Two months later, revision surgery was required due to articular screws pull out, CSF leakage, and infection. At the second surgery, the patient required screws removal, the application of laminar clamps, and sealing the leak with fibrin glue. However, the CSF leak persisted, and the skin edges necrosed leaving the hardware exposed. The third surgery was performed in conjunction with a plastic surgeon. It included operative debridement and covering the instrumentation with a pericranial flap. The resulting cutaneous defect was then additionally reconstructed with a scalp rotation flap. Postoperatively, the patient adequately recovered without sequelae. Conclusion: A 33-year-old female undergoing an occipitocervical fusion developed a postoperative persistent CSF leak, infection, and wound necrosis. This complication warranted the assistance of plastic surgery to attain closure. This required an occipital periosteal rescue flap with an added scalp rotation flap.

A 17-Year-Old Male with Large Retropharyngeal Neurofibroma as the Only Symptom of Neurofibromatosis Type 1 (NF1): A Case Report and Review of Literature

2019 ◽  
Author(s):  
Heather A. Voss-Hoynes ◽  
Naeem Mahfooz ◽  
Waseem Ostwani
Keyword(s):  
Plexiform Neurofibroma ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
Review Of Literature ◽  
Unique Case ◽  
Lower Lip ◽  
Head And Neck Imaging ◽  
Extensive Involvement

AbstractRetropharyngeal neurofibromas are rarely described in the literature and are commonly associated with severe airway-related symptoms. We report a unique case of a large retropharyngeal plexiform neurofibroma in an asymptomatic 17-year-old male patient. This patient was asymptomatic and presented for lower lip edema secondary to an insect bite. Head and neck imaging demonstrated an extensive retropharyngeal neurofibroma measuring 5.7 cm × 2.2 cm × 6.8 cm. Because of extensive involvement of cervical vasculature, the lesion was not resected in our institution and his care was referred to a large neurofibromatosis type I center.

353 C2 Segmental Neurofibromas in Patients with Neurofibromatosis Type 1: A Particularly Aggressive Phenotype

Neurosurgery ◽  
2017 ◽  
Vol 64 (CN_suppl_1) ◽  
pp. 280-281
Author(s):  
Mueez Waqar ◽  
Calvin Soh ◽  
John Ealing ◽  
Susan Huson ◽  
Gareth Evans ◽  
...  
Keyword(s):  
Cervical Spine ◽  
Neurofibromatosis Type 1 ◽  
Multivariate Logistic Regression Analysis ◽  
Transverse Process ◽  
Neurofibromatosis Type ◽  
Cord Compression ◽  
Factors Associated ◽  
Imaging Characteristics ◽  
Whole Spine

Abstract INTRODUCTION Segmental neurofibromas affecting the C2 nerve roots in patients with neurofibromatosis type 1 (NF1) can be particularly aggressive, though their clinical course and imaging characteristics are unexplored. The aim of this study was to present clinical and radiological outcomes of C2 neurofibromas in patients referred to a supra-regional NF1 centre. METHODS Imaging review of regional NF1 referrals 2009–2016. Inclusion criteria: (1) diagnosis of NF1; (2) at least one C2 root neurofibroma; (3) magnetic resonance imaging of the C-spine or whole spine. Multivariate logistic regression analysis was used to identify factors associated with need for surgery. RESULTS >54 patients with 106 neurofibromas were included. The median age was 32.5 years (range 15–61 years) and there was a slight male excess (M: F, 33:21). 32% had myelopathy. Neurofibromas were distributed in all spine regions (65%) or the cervical spine alone (22%). Intradural invasion and cord compression in the cervical spine included the C2 level in 95% and 80% of patients, respectively. Compared to all other cervical spine neurofibromas, C2 neurofibromas had higher rates of intraspinal extension (75% vs. 33%; OR = 6.03, 95% CI 3.75 - 9.71; P < 0.001), intradural invasion (53% vs. 26%; OR = 3.13, 95% CI 2.04 - 4.82; P < 0.001) and cord compression (25% vs. 13%; OR = 2.20, 95% CI 1.32 - 3.69; P = 0.003). However, C2 neurofibromas had lower rates of extraforaminal growth beyond the transverse process (12% vs. 63%; OR = 0.08, 95% CI 0.04 - 0.15; P < 0.001). 13% of patients underwent surgery to decompress the C2 level. Factors associated with surgery included myelopathy (P = 0.03) but not radiological cord compression (p > 0.99). CONCLUSION C2 neurofibromas are particularly aggressive tumours due to preferential intraspinal growth.

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.

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