A case of cervical paraganglioma: usefulness of FDG PET imaging and a possibility of rare origination

Cervical paragangliomas are rare tumors derived from neural crest cells. Anatomic imaging techniques rely upon the characteristic spatial relations and the high vascularity of this tumor. Results of computed tomography (CT), magnetic resonance imaging (MRI), and arteriography initially led us to the preoperative diagnosis of cervical paraganglioma. In our case, 2-[18F]-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET), a type of functional imaging, was also carried out and demonstrated abnormally increased tracer uptake; this approach was thus successful for visualizing paraganglioma. FDG PET imaging may be useful in the detection of benign paraganglioma. On surgical exploration of the neck, the tumor was found to arise from the vagus nerve, whereas the hypoglossal nerve was encompassed by the tumor in its upper portion. The intraoperative findings suggested the possibility that the tumor had arisen from the hypoglossal nerve.     

Computed tomography's ability to predict sacrifice of hypoglossal nerve at resection

OBJECTIVE: To assess whether preoperative computed tomography (CT) scan can determine if the hypoglossal nerve (cranial nerve XII) will be sacrificed in floor-of-mouth, oral tongue, and tongue base tumor resections. STUDY DESIGN: Retrospective review. METHODS: Patients who underwent resection of floor-of-mouth, oral tongue, and tongue base tumors from 1990 to 1999 were identified. Preoperative CT scans were reviewed by a neuroradiologist. The postoperative status of cranial nerve XII was predicted to be "saved" or "sacrificed." Hypoglossal nerve "sacrifice" was predicted if the fat planes surrounding the takeoff of the proximal lingual artery were obliterated by tumor. The nerve was determined to be sacrificed or spared during resection by review of the operative report. RESULTS: Of the 45 patients, 14 tumors were predicted radiographically to involve the hypoglossal nerve. Twenty-seven of 31 nerves that were predicted to be saved were saved at the time of surgery. Seven of 14 nerves that were predicted to be sacrificed were sacrificed at the time of surgery. The sensitivity was 0.64 (95% confidence interval [CI], 0.35-0.86) with a specificity of 0.79 (95% CI, 0.70-0.87). The positive predictive value was 0.50 (95% CI, 0.27-0.68) with a negative predictive value of 0.87 (95% CI, 0.77-0.95). CONCLUSIONS: The ability to predict preoperatively whether a tumor can be resected without sacrificing the hypoglossal nerve would be an important factor in determining management of these tumors. The results indicate that CT scan accurately predicts the ability of the surgeon to spare the hypoglossal nerve (negative predictive value, 87%) with a specificity of 0.79.     

Cervical paragangliomas: neurovascular surgical risk and therapeutic management

Paragangliomas of the neck region arise most commonly in the carotid and vagal bodies. The goal of this retrospective study is to evaluate intraoperative vascular and neurological morbidity and to define the therapeutic strategy. During the period 1990–2004, 32 patients with 42 neck paragangliomas were referred to our institution (Head and Neck Service, Otolaryngology Federation, CHU La Timone, Marseilles, France). There were 29 carotid body tumors and 11 vagal body tumors. There were 14 men and 18 women. Forty paragangliomas were surgically excised. Mean age of patients with family history of paragangliomas was 34 years and that of patients without any familial history was 47 years. Only one patient had a malignant paraganglioma. A vascular repair procedure was performed in 10% and always occurred in carotid body tumors including the malignant one. Postoperative hypoglossal nerve deficit was reported in five cases (12.5%). Paralysis of vagus nerve was reported in 11 cases (27.5%), nine of whom were patients with vagal body tumors. Knowledge of number of paragangliomas and their location is of main importance and influences the therapeutic strategy. The goal of this strategy is to avoid major neurovascular morbidity and to optimize treatment of multiple or bilateral tumors. Early management of patients prevents progressive neurological deficit due to an enlarging tumor mass and minimizes neurovascular complications.     

Rare case of the cervical vagal neurinoma

Neurinoma is the most common tumor of the neurogenic origin. Primary location in the neck with the vagal nerve as a source is very rare clinical situation (less than 100 cases published in the literature). The authors would like to present a case of 35 old men with vagal neurinoma. Main symptoms included painless neck tumor found on palpation. Differential diagnosis included the pedicled cyst and metastatic neck mass. The ultrasound picture was unclear. The intraoperative findings suggested the tumor arising from the vagal nerve. In first day after the surgery hoarseness appeared with paresis of the right vocal cord in the examination. The final histological evaluation revealed neurinoma.     

Parapharyngeal space tumors: Anatomical and image analysis findings

Objective

The parapharyngeal space (PS) is defined as the deepest space in the neck and it consists of the pre- and post-styloid regions. PS tumors originating in these regions are thought to dislocate the carotid artery (CA) in either the posterior or anterior direction. To determine the precise anatomy of the PS and its relationship with the CA in diagnostic images, we conducted cadaveric and imagining analysis.

Materials and methods

We examined the posterior and lateral aspects of the PS in three cadavers. We also examined 17 patients who suffered from PS tumors, in which the carotid artery was dislocated (CA), then compared the results with surgical and pathological findings.

Results

The anterior part of the PS was mainly composed of fatty tissue and is generally referred to as the pre-styloid region of the PS. In the posterior of this fatty region, blood vessels and nerves were tightly covered with muscles and adjacent fascias to form a compact musculo-fascial structure containing the CA, jugular vein, vagal, glossopharyngeal, accessory and sympathetic nerves, and a portion of the hypoglossal nerves. The hypoglossal nerves emerged from the hypoglossal canal posterior to this structure and coursed behind it, entering it at the upper third of the PS. These anatomical findings indicated that the PS was actually comprises of three regions. Image analysis showed that the CA was dislocated in the postero-lateral direction by a pleomorphic adenoma originating from the parotid gland and by a trigeminal schwannoma, both of which were pre-styloid tumors. On the other hand, the post-styloid tumors did not always dislocate the CA in the anterior direction. Tumors that developed within the musculo-fascial structure such as those of the carotid body tumor or sympathetic nerve schwannoma dislocated the CA from both the antero- to the postero-lateral directions. A hypoglossal nerve schwannoma originating from the most posterior part of the PS, which was behind the musculo-fascial structure dislocated the CA in the anterior direction.

Conclusion

The present findings indicated that the post-styloid region of the PS is considered to consist of two regions. As the CA is a component of this musculo-fascial structure, tumors originating from it that are defined as post-styloid did not always displace the CA in the anterior direction. Such anatomical recognition is helpful for diagnostic imaging of PS tumors.     

手术治疗面神经鞘瘤

目的：探讨诊断面神经鞘瘤的方法、手术治疗该病的效果及修复面神经功能的途径。方法：入住本院的面神经鞘瘤患者11例，在确诊前全部误诊为他病，确诊后，对6例中耳乳突腔面神经鞘瘤患者行神经移植，其中5例应用耳大神经移植，1例行干热骨骼肌桥接；对2例中耳乳突腔-颅内面神经鞘瘤的患者，1例行舌下神经移植（舌下神经攀植入舌下神经），1例直接将舌下神经襻植入面神经远端；其余患者面瘫时间较长，已不能恢复面神经功能，仅1例行阔筋膜口轮匝肌悬吊术，其他放弃面神经功能的重建。结果：3例未行面神经功能重建的患者，遗留完全面瘫；6例中耳乳突腔面神经鞘瘤的患者，面神经功能恢复分别为（Brakmann House功能分级）功能2级3例、3级2例、未恢复1例；2例舌下神经以及攀支重建面神经的患者，功能2级1例、3级1例。结论：面神经鞘瘤的临床症状多种多样，应进行系统检查排除误诊。术中为了完整切除肿瘤，可采用面神经改道吻合术、面神经移植等方法，在断端套用静脉血管给予保护，予以耳脑胶进行粘合。另外，干热骨骼肌的桥接在临床上应该推广使用。     

Accessory nerve schwannoma extending extracranially to the second cervical level of vertebra

We described a 67-year-old man who had a right jugular foramen tumour expanding extracranially to the level of C2 cervical body. Paralysis of the glossopharyngeal, vagal, accessory, hypoglossal nerves and sensorineural hearing disturbance were found. The tumour originated from the accessory nerve and the histological examination revealed it was schwannoma. Accessory nerve as an origin of the jugular foramen neurinoma and its pre-operative neurological signs are reviewed.     

Neck nerve trunks schwannomas: clinical features and postoperative neurologic outcome

Objectives/Hypothesis: To analyze clinical and epidemiological features of neck nerve schwannomas, with emphasis on the neurologic outcome after surgical excision sparing as much of nerve fibers as possible with enucleation technique. Study Design: Retrospective study. Methods: Review of medical records from 1987 to 2006 of patients with neck nerve schwannomas, treated in a single institution. Results: Twenty-two patients were identified. Gender distribution was equal and age ranged from 15 to 61 years (mean: 38.6 years). Seven vagal, four brachial plexus, four sympathetic trunk, three cervical plexus, and two lesions on other sites could be identified. Most common symptom was neck mass. Local or irradiated pain also occurred in five cases. Median growing rate of tumors was 3 mm per year. Nerve paralysis was noted twice (a vagal schwannoma and a hypoglossal paralysis compressed by a vagal schwannoma). Different techniques were employed, and seven out of nine patients kept their nerve function (78%) after enucleation. No recurrence was observed in follow-up. Conclusions: Schwannomas should be treated surgically because of its growing potential, leading to local and neural compression symptoms. When possible, enucleation, which was employed in 10 patients of this series, is the recommended surgical option, allowing neural function preservation or restoration in most instances. This is especially important in the head and neck, where denervation may have a significant impact on the quality of life.     

Occult functioning vagal paraganglioma in the infrahyoid carotid sheath

Paragangliomas are highly vascular tumors that arise from chief cells in extra-adrenal paraganglia of the autonomic nervous system. Vagal paragangliomas occur along the vagal nerve, usually located in the rostral portion of the vagus nerve in the vicinity of the gangliom nodosum. Actively functional vagal paragangliomas are rare. We report a patient with functioning vagal paraganglioma located in the infrahyoid carotid sheath. The patient had no history of hypertension and catecholamines were not measured before surgery. The findings of diagnostic imaging workup, including computed tomography, ultrasonography and magnetic resonance, were suggestive of paraganglioma. The blood pressure and pulse rates increased sharply intraoperatively during tumor manipulation, together with spikes in noradrenaline and dopamine. The tumor was removed with successful preservation of the vagus nerve trunk. The blood catecholamine levels returned to normal immediately after surgery. Head and neck surgeons should be aware of occult functioning paragangliomas and patients with such tumors should undergo full hormonal assessment.     

舌下神经-面神经侧端吻合术治疗小脑脑桥角肿瘤术后面瘫

摘要：目的探讨舌下神经-面神经侧端吻合术治疗小脑脑桥角肿瘤术后面瘫的效果。方法6例小脑脑桥角肿瘤切除术后面瘫患者均行舌下神经-面神经侧端吻合术。所有患者术后每3个月随访1次,评估House Brackmann(H B)分级和舌下神经功能。结果术后1年H B III级2例,H B Ⅳ级3例, H B V级1例。静态面部张力4例患者在吻合术后6个月改善明显,1例患者在吻合术后 9个月改善,1例患者在吻合术后1年改善。所有患者均未出现术侧舌肌瘫痪萎缩,发音和吞咽功能均正常。结论舌下神经-面神经侧端吻合术可改善小脑脑桥角肿瘤切除术后面瘫患者的面部张力和面肌功能,借助神经监护可尽量减小对舌肌功能的影响。     

Hypoglossal nerve paralysis following tonsillectomy

Lesions of the lingual nerve and the glossopharyngeal nerve following tonsillectomy are rare but can be expected because of their anatomical course. What is extremely rare is a lesion of the hypoglossal nerve, whose course behind the carotid artery protects it from direct injury. The few cases described in the literature are thought to have been caused by inflammatory processes. It became necessary to look for other causes when, after a regular tonsillectomy, a hypoglossal palsy became evident in the absence of any inflammation. In an experiment, it was possible to demonstrate that both the insertion of a spatula and of an intubation spatula caused a strain of the hypoglossal nerve when the spatulas were inserted in the lateral lingual region. The nerve was distended by as much as 1.3 cm. The more the head was reclined, the more the nerve was distended. It would seem probable that this extension of the hypoglossal nerve causes its palsy following tonsillectomy.     

Isolated hypoglossal nerve palsy: a harbinger of malignancy

The hypoglossal nerve is an underrated nerve usually consigned to a few words in anatomical text books, under the last four cranial nerves. However, paralysis of this nerve may be the first indication of a serious underlying disorder. Excluding previous surgery, radiotherapy and trauma, 50 per cent of cases of isolated hypoglossal nerve palsy are idiopathic. A further 20 per cent are malignant, 20 per cent are vascular and 10 per cent are due to miscellaneous causes. Presentation of an isolated hypoglossal nerve palsy is therefore an ominous sign. There is confusion over both cause and investigation, and management protocols for isolated hypoglossal nerve palsy are ill-defined. We present a case of isolated hypoglossal palsy which was due to a metastatic skull base deposit. This case illustrates the fact that magnetic resonance imaging is the investigation of choice in assessing the entire course of the hypoglossal nerve.     

Subacute hypoglossal nerve paresis with internal carotid artery dissection

OBJECTIVES: To describe a case of an isolated hypoglossal nerve palsy in a patient with a spontaneous internal carotid artery dissection (ICAD). This condition is a well-recognized cause of cerebral ischemic stroke in patients younger than 45 years of age. Isolated cranial nerve neuropathy is a rare presentation. More common manifestations include incomplete hemiparesis, hemicrania, Horner syndrome, cervical bruit, pulsatile tinnitus, and multiple cranial nerve palsies. METHODS: A comprehensive literature search (Ovid, MEDLINE) for the presentation, diagnostic evaluation, treatment, and outcome of patients with internal carotid artery dissection was performed. RESULTS: A 43-year-old man presented with a 3-week history of mild dysarthria. There was no history of craniocervical trauma. The physical examination revealed an isolated left hypoglossal nerve paresis. Magnetic resonance imaging and angiography findings were consistent with a left skull base ICAD. The patient was successfully treated with anticoagulation therapy. The current rate of cranial nerve involvement is estimated at 10% of all ICADs. This is the second report of isolated hypoglossal nerve palsy without hemicrania in a case of atraumatic ICAD. CONCLUSIONS: Patients with an ICAD infrequently present to the otolaryngologist because of its head and neck manifestations. It is crucial to recognize atypical findings and to perform an accurate and prompt diagnostic evaluation. The foundation of treatment is aggressive anticoagulation, with surgical or radiologic intervention reserved for cases demonstrating life-threatening progression.     

颈动脉体瘤诊断与处理

目的 探讨颈动脉体瘤（CBT)诊断、手术治疗以及防治术后并发症的经验。方法 整理15例CBT患者的病历资料,回顾性分析并加以总结。结果 15例按照Shamblin分型,Ⅰ型2例,Ⅱ型8例,Ⅲ型5例。术前影像学评估包括B超、CT、MRA,以及数字减影血管造影DSA、64排CT增强扫描结合血管成像CTA。行手术切除14例,手术方式包括伴或不伴颈外动脉切除的完整剥离;瘤体连同颈内-颈总动脉切除血管重建。术后病理检查均为良性。所有患者经3个月～9年4个月随访, 出现单侧喉返神经麻痹2例,单侧舌下神经麻痹1例,单侧颈交感神经损伤1例,无脑血管意外及死亡,均无局部复发。结论 64排CTA可作为CBT术前诊断、评估的首选方法。而正确诊断、周密完善的术前评估及合理的手术方案能减少术后严重并发症。     

Role of the trigeminal nerve in regrowth of hypoglossal motoneurons after hypoglossal-facial anastomosis

Conclusion. Functional recovery of facial muscles following hypoglossal-facial anastomosis (HFA) may be dependent not only on sensory information, relayed via the trigeminal nuclei to the hypoglossal nucleus, but also on extratrigeminal fibers, originating from the hypoglossal nucleus that travel in the infraorbital nerve (ION). This fact helps to explain the ability of hypoglossal neurons, after HFA, to induce contractions of muscles originally innervated from other nervous structures. Objective. The aim of the study was to better understand the role of the trigeminal nerve in reinnervation of facial muscles by hypoglossal motoneurons following HFA. Materials and methods. Central afferences of the ION were analyzed in rats by labeling the exposed nerve with horseradish peroxidase (HRP), whereas central organization of the efferent projections to the vibrissal area was analyzed by labeling the whisker pad muscles of the rat with a 5% solution of 1,1'-dioctadecyl-3,3,3',3'-tetramethylindocarbocyanine perchlorate (Dil) in N,N-dimethylformamide. Results. The results show that extratrigeminal fibers, originating in the hypoglossal nucleus, travel along the ION. Retrograde tracing applied to ION or injected into the whisker pad showed labeled neurons in the Pr5 nucleus and all Sp5 trigeminal subnuclei. Small labeled neurons (10-15 microm diameter; 10-12 neurons per section), were also found in the hypoglossal nucleus.     

植入式舌下神经刺激器治疗阻塞性睡眠呼吸暂停低通气综合征

阻塞性睡眠呼吸暂停低通气综合征(OSAHS)是一种常见且对人类健康有重要影响的疾病.其发病机制尚不完全清楚,目前普遍认为上气道扩张肌功能紊乱是OSAHS的发病基础.在上气道扩张肌群中,颏舌肌受舌下神经支配,在对抗吸气相气道负压、维持上气道开放中的作用最为重要.大量实验证实刺激舌下神经内侧支或直接刺激颏舌肌时可使舌前伸,咽腔扩大,最大气流量增大.随着对上气道扩张肌生理学研究的深入,为舌下神经刺激治疗OSAHS提供了理论基础.本文就应用植入式舌下神经刺激器治疗OSAHS的研究进展进行综述.     

Schwannoma of the tongue--case report

The author described a rare case of lingual Schwannoma in 19 years old female. The tumor originated from lingual ramus at the hypoglossal nerve and was removed by surgery.     

Therapeutic electrical stimulation of the hypoglossal nerve in obstructive sleep apnea

BACKGROUND: Hypoglossal nerve stimulation has been demonstrated to relieve upper airway obstruction acutely, but its effect on obstructive sleep apnea is not known. OBJECTIVE: To determine the response in obstructive sleep apnea to electrical stimulation of the hypoglossal nerve. METHODS: Eight patients with obstructive sleep apnea were implanted with a device that stimulated the hypoglossal nerve unilaterally during inspiration. Sleep and breathing patterns were examined at baseline before implantation and after implantation at 1, 3, and 6 months and last follow-up. RESULTS: Unilateral hypoglossal nerve stimulation decreased the severity of obstructive sleep apnea throughout the entire study period. Specifically, stimulation significantly reduced the mean apnea-hypopnea indices in non-rapid eye movement (mean +/- SD episodes per hour, 52.0 +/- 20.4 for baseline nights and 22.6 +/- 12.1 for stimulation nights; P<.001) and rapid eye movement (48.2 +/- 30.5 and 16.6 +/- 17.1, respectively; P<.001) sleep and reduced the severity of oxyhemoglobin desaturations. With improvement in sleep apnea, a trend toward deeper stages of non-rapid eye movement sleep was observed. Moreover, all patients tolerated long-term stimulation at night and did not experience any adverse effects from stimulation. Even after completing the study protocol, the 3 patients who remained free from stimulator malfunction continued to use this device as primary treatment. CONCLUSION: The findings demonstrate the feasibility and therapeutic potential for hypoglossal nerve stimulation in obstructive sleep apnea.     

Determination of the changes in the hypoglossal nerve function after suspension laryngoscopy with needle electromyography of the tongue

The purpose of this study was to determine changes in the hypoglossal nerve function after suspension laryngoscopy with needle electromyography of the tongue. This study also attempted to determine the possible relationship between the predictive factors of intubation difficulty by using the intubation difficulty scale, which was introduced by Adnet et al., duration of suspension laryngoscopy and changes in hypoglossal nerve function after suspension laryngoscopy. The study was performed on 39 patients who underwent suspension laryngoscopy for benign glottic pathology. Pre-operative airway assessment was evaluated by the intubation difficulty scale and the duration of suspension laryngoscopy was recorded. Needle electromyography of the tongue was performed three or four weeks after the suspension laryngoscopy. After needle electromyography of the tongue, increased polyphasia was found in 13 patients (33 per cent), bilaterally in three of them. The interference pattern was reduced in two of these 13 patients. There was no statistically significant difference in predictive factors of intubation difficulty and the duration of the operation between these 13 patients with increased polyphasia and the remaining 26 patients with completely normal electromyography findings. These findings show that, in spite of normal clinical tongue function, subclinical changes can be detected by needle electromyography of the tongue after suspension laryngoscopy.     

Lesions of the hypoglossal nerve —diagnosis,treatment and rehabilitation

The hypoglossal nerve is one of the four nerves in the posterior cranial fossa. These include the vagus, glosspharyngeal, and spinal accessory nerves. The hypoglossal is, however, distinctly different from these other three nerves, having a separate blood supply in the brain stem, a separate exit from the skull, and a distinct physiologic function. As such, its distinctive findings provide an integral link to the diagnosis of posterior fossa disease. By coordinating anatomy and physiology to diagnosis, specific pathologic entities may be ascertained. This methodology can be applied to the classic supranuclear, nuclear and peripheral division of neurologic disorders. It is the purpose of this paper to provide a systemized approach to the diagnosis of disease involving the hypoglossal nerve. This approach will, in turn, allow an accurate diagnosis over an extensive part of the nervous system, especially in the brain stem and posterior fossa.