Computed tomographic features of canine intracranial and jugular foraminal masses involving the combined glossopharyngeal, vagus, and accessory nerve roots.

A chronic cough, gag, or retch is a common presenting clinical complaint in dogs. Those refractory to conservative management frequently undergo further diagnostic tests to investigate the cause, including CT examination of their head, neck, and thorax for detailed morphological assessment of their respiratory and upper gastrointestinal tract. This case series describes five patients with CT characteristics consistent with an intracranial and jugular foraminal mass of the combined glossopharyngeal (IX), vagus (X), and accessory (XI) cranial nerves and secondary features consistent with their paresis. The consistent primary CT characteristics included an intracranial, extra-axial, cerebellomedullary angle, and jugular foraminal soft tissue attenuating, strongly enhancing mass (5/5). Secondary characteristics included smooth widening of the bony jugular foramen (5/5), mild hyperostosis of the petrous temporal bone (3/5), isolated severe atrophy of the ipsilateral sternocephalic, cleidocephalic, and trapezius muscles (5/5), atrophy of the ipsilateral thyroarytenoideus and cricoarytenoideus muscles of the vocal fold (5/5), and an ipsilateral "dropped" shoulder (4/5). Positional variation of the patient in CT under general anesthesia made the "dropped" shoulder of equivocal significance. The reported clinical signs and secondary CT features reflect a unilateral paresis of the combined cranial nerves (IX, X, and XI) and are consistent with jugular foramen syndrome/Vernet's syndrome reported in humans. The authors believe this condition is likely chronically underdiagnosed without CT examination, and this case series should enable earlier CT diagnosis in future cases.

Imaging manifestations on sequential magnetic resonance imaging in pharyngolaryngeal involvement by varicella zoster virus.

Clarifying temporal changes in magnetic resonance imaging (MRI) offers a good chance to understand the pathology of neural lesions; however, such information is scarce in varicella zoster virus (VZV) neuropathies for the glossopharyngeal and vagus nerves. Here, we present the changes in sequential MR images of such a pathology over a period of 12 months from symptom onset.A 27-year-old woman with difficulty in swallowing and hoarseness due to a palatal palsy and arytenoid fixation on the left presented 2 days after onset. MRI revealed a lesion which largely filled the left jugular foramen on T2-weighted images (T2-WI) with high diffusion-weighted imaging (DWI) signals, which has never been previously described, on the 3rd day after onset. The DWI signals were highest on day 3, then deteriorated over 2 months until the signal was only detectable at the intracranial level, but not in the jugular foramen. The glossopharyngeal nerve had returned to normal by 2 months.The time course of the glossopharyngeal and vagus nerve swelling detected on T2-WI suggests that nerve swelling reduces over several months, even though the paralytic symptoms persist. Furthermore, the high DWI signal suggests that nerve swelling was caused by edematous swelling of the nerve fibers, rather than fiber disruption with water displacement in the extracellular space. These findings may provide good clues to speculate on the dynamically changing pathology of VZV neuropathies of the glossopharyngeal and vagus nerves.

Purely intracranial vagal schwannoma: A case report of a rare lesion.

We present a rare intracranial vagal schwannoma along with its preoperative, intraoperative, and postoperative course.

Magnetic Resonance Imaging Features of Collet-Sicard Syndrome Associated With Glomus Jugulare Paraganglioma.

Collet-Sicard syndrome is an unusual disorder. The authors here demonstrated the Magnetic resonance (MR) imaging findings of the Collet-Sicard syndrome associated with glomus jugulare tumor. Neoplastic or non-neoplastic lesion of skull base can cause Collet-Sicard syndrome. MR imaging can be used successfully to demonstrate the etiology of this syndrome.

Report of a vagal paraganglioma at the cervicothoracic junction.

BACKGROUND: Vagus nerve paragangliomas are rare tumors, comprising 0.03% of head and neck neoplasms. These tumors are usually located cephalad to the hyoid bone, and there is only one previously reported case that arose from the lower third of the neck. METHODS: We describe the second reported case of a lower neck vagus nerve paraganglioma that was managed with a limited sternotomy for access and surgical removal. RESULTS: A 66-year-old male presented with a long-standing lesion of the cervicothoracic junction. CT, MRI, and Ga-68 DOTATATE PET/CT showed an avidly enhancing 5.2 × 4.2 × 11.5 cm mass extending from C6 to approximately T4 level. FNA confirmed the diagnosis. The patient underwent catheter angiography and embolization via direct puncture technique followed by excision of the mass via a combined transcervical and limited sternotomy approach. CONCLUSION: We describe an unusual case of vagal paraganglioma at the cervicothoracic junction with retrosternal extension requiring a sternotomy for surgical excision.

Multiple paragangliomas: a case report.

BACKGROUND: Carotid and vagal paragangliomas (CPGLs and VPGLs) are rare neoplasms that arise from the paraganglia located at the bifurcation of carotid arteries and vagal trunk, respectively. Both tumors can occur jointly as multiple paragangliomas accounting for approximately 10 to 20% of all head and neck paragangliomas. However, molecular and genetic mechanisms underlying the pathogenesis of multiple paragangliomas remain elusive. CASE PRESENTATION: We report a case of multiple paragangliomas in a patient, manifesting as bilateral CPGL and unilateral VPGL. Tumors were revealed via computed tomography and ultrasound study and were resected in two subsequent surgeries. Both CPGLs and VPGL were subjected to immunostaining for succinate dehydrogenase (SDH) subunits and exome analysis. A likely pathogenic germline variant in the SDHD gene was indicated, while likely pathogenic somatic variants differed among the tumors. CONCLUSIONS: The identified germline variant in the SDHD gene seems to be a driver in the development of multiple paragangliomas. However, different spectra of somatic variants identified in each tumor indicate individual molecular mechanisms underlying their pathogenesis.

Radiologic signs in thoracic imaging: case-based review and self-assessment module.

OBJECTIVE: Chest imaging remains one of the most complicated sub-specialties of diagnostic radiology. The successful interpretation of thoracic imaging studies requires the recognition and understanding of the radiologic signs that are characteristic of many complex disease processes. CONCLUSION: The educational objectives for this case-based self-assessment module are for the participant to exercise, self-assess, and improve his or her understanding of important thoracic radiologic signs that are useful in establishing the diagnosis of particular diseases of the chest.

Sonographic diagnosis of a cervical vagal schwannoma.

This case report illustrates the role of high-resolution sonography in the preoperative assessment of a schwannoma of the vagus nerve in the neck. Sonography identified the tumor in the right carotid space and determined its origin from the right vagus nerve, facilitating the surgeon's approach to preserve nerve function.

Tumor multifocality with vagus nerve involvement as a phenotypic marker of SDHD mutation in patients with head and neck paragangliomas: A 18 F-FDOPA PET/CT study.

BACKGROUND: 18 F-FDOPA PET/CT was proved to be a highly sensitive imaging method for detecting head and neck paraganglioma (HNPGL). The primary aim of the study was to evaluate the relationship between tumor characteristics and the SDHx-mutational status in a large series of patients with HNPGL evaluated by 18 F-FDOPA PET/CT. METHODS: A total of 104 patients with HNPGL (65 sporadic/39 SDHx-mutated) were included. RESULTS: In comparison to SDHB/SDC/SDHx-negative cases, patients with SDHD were younger at diagnosis and had a higher rate of multifocal, vagal, and carotid paraganglioma. In patients with SDHD, vagal paraganglia represented the primary site of tumor origin. Multicentric involvement of the vagus nerve alone or in association with other locations was found to be a typical feature of SDHD cases compared to other cases (odds ratio = 59.4). CONCLUSION: The present study shows that tumor multifocality within the vagus nerve is a phenotypic marker of SDHD mutation. This information is essential in the choice of the therapeutic strategy.

A rare case of bilateral vagus nerve schwanomatosis.

Schwanomatosis is the third most common form of neurofibromatosis. Schwanomatosis affecting the vagus nerve is particularly rare. In this report, we describe an extremely rare case bilateral vagus nerve schwanomatosis in a 45-year-old male patient. The patient initially presented with bilateral neck tumors and hoarseness arising after thoracic surgery. We performed left neck surgery in order to diagnose and resect the remaining tumors followed by laryngeal framework surgery to improve vocal cord closure and symptoms of hoarseness. Voice recovery was successfully achieved after surgery. An appropriate diagnosis and surgical tumor resection followed by phonosurgery improved patient quality of life in this rare case.

A rare case of vagus nerve schwannoma.

Schwannomas of the vagus nerve are very rare and the differential diagnosis is extremely difficult. They are very often asymptomatic and imaging techniques are helpful, but fine needle biopsy is often inconclusive. Total resection is the treatment of choice.

Endocrine tumors associated with the vagus nerve.

The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of an endocrine tumor associated with the vagus nerve. This rare, neural crest tumor constitutes the second most common site of hereditary head and neck paragangliomas (HNPGLs), most often in relation to mutations in the succinate dehydrogenase complex subunit D (SDHD) gene. The treatment paradigm for VPGL has progressively shifted from surgery to abstention or therapeutic radiation with curative-like outcomes. Parathyroid tissue and parathyroid adenoma can also be found in close association with the vagus nerve in intra or paravagal situations. Vagal parathyroid adenoma can be identified with preoperative imaging or suspected intraoperatively by experienced surgeons. Vagal parathyroid adenomas located in the neck or superior mediastinum can be removed via initial cervicotomy, while those located in the aortopulmonary window require a thoracic approach. This review particularly emphasizes the embryology, molecular genetics, and modern imaging of these tumors.

Repeat Imaging in Idiopathic Unilateral Vocal Fold Paralysis: Is It Necessary?

OBJECTIVE: Imaging plays a critical role in the evaluation of patients presenting with unilateral vocal fold paresis or paralysis of unknown etiology. In those with idiopathic unilateral vocal fold paralysis (iUVFP), there is no consensus regarding the need or timing of repeat imaging. This study seeks to establish the rate of delayed detection of alternate etiologies for these patients to determine if and when imaging should be repeated. METHODS: Retrospective chart review was conducted identifying patients at our institution with vocal fold movement impairment between 1998 and 2014. Idiopathic paralysis was diagnosed if physical examination, laryngoscopy, and initial imaging excluded a cause. Demographic data, length of follow-up, and the presence of late lesions were noted. Time to detection was plotted using the Kaplan-Meier method. RESULTS: Of 3210 patients reviewed, 207 had a diagnosis of iUVFP. Of these patients, 8 went on to develop alternate diagnoses, including pulmonary disease, skull-base and laryngeal neoplasms, and thyroid malignancy. In Kaplan-Meir analysis, 90% remained "idiopathic" at 5 years of follow-up. The mean time to detection was 27 months. CONCLUSIONS: Patients initially diagnosed with iUVFP may have an occult cause that later becomes evident. We recommend repeat imaging within 2 years after diagnosis, but this is likely unnecessary beyond 5 years.

Videofluoroscopic swallow studies in unilateral cricopharyngeal dysfunction.

OBJECTIVES/HYPOTHESIS: Although the cricopharyngeus muscle is a ring-like structure, unilateral cricopharyngeal dysfunction can produce significant dysphagia. This entity has not been well described in the literature. The aims of the study were to identify the characteristic findings on videofluoroscopic swallow studies in patients with dysphagia secondary to unilateral cricopharyngeal dysfunction, to note the associated vagal nerve injury, and to evaluate patient outcomes following ipsilateral cricopharyngeal myotomy. STUDY DESIGN: Retrospective clinical investigation. METHODS: The clinic charts, electromyographic tests, videostroboscopic examinations, and videofluoroscopic swallow studies were reviewed from a series of patients who presented to our institution from 1993 to 2001 with dysphagia and findings on videofluoroscopic swallow studies suggestive of unilateral cricopharyngeal dysfunction on posterior-anterior view. In patients treated with ipsilateral cricopharyngeal myotomy, postoperative findings on swallow studies and patient outcomes were also reviewed. RESULTS: Eighteen patients demonstrated findings characteristic of unilateral cricopharyngeal muscle dysfunction on videofluoroscopic swallow study. The common feature was a unilateral shelf-like barrier at the cricopharyngeus on the posterior-anterior view with pooling of liquid bolus in the ipsilateral pyriform sinus and episodic shunting to the contralateral side. Eight patients did not have evidence of cricopharyngeal dysfunction (ie, cricopharyngeal bar) on lateral films. Of the 18 patients, 14 had histories consistent with vagal injury secondary to trauma (n = 2), neoplastic involvement (n = 7), iatrogenic injury (n = 2), or central nervous system disease (n = 3). Results of videostroboscopic examinations demonstrated vocal fold motion impairment in 14 patients, and electromyographic test results confirmed unilateral vagal injuries in those who underwent electromyographic testing (n = 6). In the remaining 4 of 18 patients, videostroboscopic examinations demonstrated normal vocal fold abduction but impaired lengthening with a posterior glottic gap, and electromyographic test results (n = 4) indicated unilateral superior laryngeal nerve involvement. Of the 15 patients treated with ipsilateral cricopharyngeal myotomy, 1 patient required postoperative esophageal dilations for an esophageal stricture distal to the cricopharyngeus, whereas the remaining 14 patients had functional resolution of their dysphagia. CONCLUSION: In patients presenting with dysphagia and evidence of unilateral vagal injury, careful assessment of posterior-anterior view on videofluoroscopic swallow study should be included to evaluate for unilateral cricopharyngeal dysfunction.

Malignant peripheral nerve sheath tumors of intrathoracic vagus nerve.

The authors report two cases of malignant peripheral nerve sheath tumors arising from the vagus nerve in the mediastinum in patients who had no stigmata of neurofibromatosis (von Recklinghausen's disease). Computed tomography showed homogeneous soft tissue masses, with minimal calcification seen in one patient. T2-weighted magnetic resonance images showed regions of low signal intensity caused by a dense cellular population.

Vagal paraganglioma. Report of a case surgically treated and review of the literature.

The authors report the observation of one case of vagal paraganglioma occurred in a young woman. The tumor manifested itself as a left sub-mandibular tumescence; the very first diagnostic approach was achieved through echography, which showed a mass behind the internal carotid artery and compressing the internal jugular vein. The following examinations, represented by neck CT, NMR, angiography and fine needle aspiration initially directed towards the suspicion of chemodectoma. Only at operation, the anatomical situation of the tumor, which encapsulated the vagus nerve and the subsequent results of the hystological examination revealed the correct diagnosis of vagal paraganglioma.

Cervical paragangliomas: diagnosis, management and complications.

Sixteen patients were diagnosed as suffering from cervical paragangliomas. Eleven patients (68.75 per cent) had twelve carotid paragangliomas (CPs), and five patients (31.25 per cent) had six vagal paragangliomas (VP). One CP (8.33 per cent) originated from paraganglia around the common carotid artery (CCA). Three cases of multiple paragangliomas are presented (18.75 per cent). In 80 per cent (4/5) of VP patients there was widening of the carotid bifurcation similar to that seen with CP. This widening occurred whenever the VP was large enough to grown in between the external carotid artery and internal carotid artery (ECA and ICA). Large VPs may displace the vessels either anterolaterally or anteromedially. Knowledge of the direction of the carotid displacement is essential to avoid intra-operative vascular injuries. Colour flow doppler ultrasound (CFD-US) was found to be a good non-invasive method for diagnosis of vascular neck swellings. It enabled the diagnosis of CP with 100 per cent accuracy, but it was not sufficient for diagnosis of high VP. A transcervical approach, cutting the digastric muscle and the styloid process with the attached ligaments and muscles, was sufficient for excision of most VP. However, midline mandibulotomy might be necessary with high VP. Vascular injuries occurred in 12.5 per cent (2/16) of patients. Superior laryngeal nerve and hypoglossal nerve paralysis occurred, respectively, in (2/11) and (1/11) of patients with CP. Vagal paralysis occurred in all patients with VP. Cerebrovascular accident and post-operative death occurred in one patient (6.26 per cent).