A case of cervicogenic headache caused by C5 nerve root derived shwannoma: Case report.

Introduction We report a case of cervicogenic headache caused by an intradural extramedullary tumor of the middle cervical spine, which has not previously been reported. Case presentation The patient was a 73-year-old male who visited a physician for a chief complaint of pain from the left lower jaw to the auricle and occipital region. The headache was induced with retroflexion of the neck. On cervical magnetic resonance imaging, an intradural extramedullary tumor was noted on the left side at the C4/5 level. The intradural tumor, which arose from the C5 nerve root, was excised and the pain was resolved. The pathological diagnosis was schwannoma. Conclusion Previously reported cases of spinal cord tumor-induced cervicogenic headache were due to upper cervical spinal tumors. This is the first report that a middle-lower cervical intradural extramedullary tumor caused cervicogenic headache.

Surgical Management of Peripheral Nerve Pathology in Patients With Neurofibromatosis Type 2.

BACKGROUND: Neurofibromatosis type 2 (NF2) is rare genetic disorder mainly characterized by the development of central nervous system lesions, but peripheral nerve pathology may also cause high morbidity including pain, motor, and sensory loss. OBJECTIVE: To describe the tumor burden of patients with peripheral nerve pathology in NF2 including peripheral neuropathies and schwannomas and the results of surgery in the latter group. METHODS: We conducted a retrospective chart review of all patients with NF2 followed up at our NF2 Reference Center to include all patients suffering from peripheral nerve pathology. Tumor detection relied on focal MRIs based on symptoms. RESULTS: Thirty-four patients harboring 105 peripheral nerve schwannomas and 1 perineurioma were included. Schwannomas were mainly located in major nerves (n = 74, 71%) compared with subcutaneous (n = 23, 22%) and intramuscular (n = 8, 7%) cases. Most schwannomas (81/90-90%) were classical discrete tumors while multinodular cases represented only 9 cases (10%). During follow-up, 63 (60%) tumors were operated in 24 patients, including 39 schwannomas of major nerves. A complete resection was achieved in most of the cases (52/63, 83%) with a complete relief of preoperative pain in most patients (57/60, 95%). Persistent motor deficits (5/39, 13%) were mostly encountered in patients operated from multinodular schwannomas (4/5, 80%). Six patients had an associated peripheral neuropathy with 5 cases of pseudo-Charcot-Marie-Tooth-associated amyotrophy. CONCLUSION: Surgery remains a safe and effective method of treating peripheral nerve schwannoma-associated pain in NF2, with the exception of rare multinodular tumors. Special attention should be drawn to patients harboring severely debilitating neuropathies and perineuriomas.

Utilization of a modified bite guard for preventing traumatic macroglossia.

This clinical report describes the treatment of a patient having traumatic macroglossia due to schwannoma in the craniocervical region. Enlarged tongue or macroglossia may compromise vital functions of the patient. To avoid a chronic tongue bite trauma, a bite guard that was inspired from a habit breaker was fabricated. Tongue injury has significantly healed with the use of this appliance, and the patient was able to masticate without biting on his tongue.

Charcot-Marie-Tooth 1A concurrent with schwannomas of the spinal cord and median nerve.

We identified Charcot-Marie-Tooth disease type 1A (CMT1A) in a family with schwannomas in the spinal cord and median nerve. The CMT1A in this family showed an autosomal dominant pattern, like other CMT patients with PMP22 duplication, and the family also indicated a possible genetic predisposition to schwannomas by 'mother-to-son' transmission. CMT1A is mainly caused by duplication of chromosome 17p11.2-p12 (PMP22 gene duplication). A schwannoma is a benign encapsulated tumor originating from a Schwann cell. A case of hereditary neuropathy with liability to pressure palsies (HNPP) concurrent with schwannoma has been previously reported. Although it seems that the co-occurrence of CMT1A and schwannomas in a family would be the result of independent events, we could not completely ignore the possibility that the coincidence of two diseases might be due to a shared genetic background.

Two case reports of orofacial paraesthesia demonstrating the role of the general dental practitioner in identifying patients with intracranial tumours.

The following case reports describe the clinical features, diagnosis and management of two patients who presented to their general dental practitioner with a complaint of orofacial paraesthesia. After appropriate investigations, both patients were diagnosed as having benign intracranial tumours and were managed by a neurosurgeon. These cases illustrate the important role the general dental practitioner has in the early recognition of potentially life-threatening conditions.

First-bite syndrome after parapharyngeal surgery for cervical schwannoma.

BACKGROUND: First-bite syndrome (FBS) is a rare complication that occurs after patients undergo parapharyngeal space surgery. Characteristically, inadvertent ablation of the parotid gland's sympathetic innervation results in the development of severe parotid gland-area pain at the first bite of food. CASE DESCRIPTION: The authors evaluated a patient who underwent parapharyngeal surgery for cervical schwannoma. This surgery involved the sympathetic chain's superior cervical ganglion (SCG). With destruction of the SCG, the patient developed FBS and Horner syndrome. CONCLUSION AND CLINICAL IMPLICATIONS: Destruction of the SCG or the sympathetic postganglionic supply to the parotid gland causes severe parotid pain when food is first introduced into the mouth. The absence of discomfort during mechanical joint movements helps dentists differentiate this pain from myofascial pain or pain caused by temporomandibular dysfunction. The frequent presence of Horner syndrome also facilitates diagnosis.

Hypoglossal canal schwannoma causing isolated left 12th cranial nerve palsy.

A 40-year-old woman presented with insidious onset, gradually progressive dysarthria and inability to manoeuvre bolus of food in her mouth while eating. The duration of her symptoms was 3 months. On evaluation, the left half of her tongue was wasted. The tongue deviated to the left on protrusion. There were no clinical features suggestive of involvement of the ipsilateral 9th, 10th or 11th cranial nerves. MRI of the brain showed a large, fusiform lesion in the left hypoglossal canal, extending into the jugular canal. The lesion was surgically excised and found to be a schwannoma.

Malignant schwannoma of the infratemporal fossa: a case report.

INTRODUCTION: Malignant schwannomas or neurofibrosarcomas are rare nerve tumors of unknown etiology. These neoplasms are highly aggressive with a marked propensity for local recurrence and metastatic spread. Their management continues to be a challenge for pathologists and surgeons. Maxillofacial locations are very exceptional. We report the case of a patient with unusual malignant schwannoma of the infratemporal fossa discovered at a late evolving stage. CASE PRESENTATION: A 56-year-old woman, of Moroccan nationality, presented to our hospital in 2013 with a large right-sided hemifacial swelling that had evolved over the previous 4 months, with a limitation of mouth opening, nasal obstruction and episodes of epistaxis. A CT scan and MRI showed a large and invasive tumor occupying her right infratemporal fossa and maxillary sinus, with sphenoidal, ethmoidonasal, nasopharyngeal and intraorbital extension. A nasal endoscopic biopsy was performed. Immunohistochemical examination concluded a diagnosis of malignant schwannoma, and a palliative radiotherapy was decided; however, our patient died 10 days later. CONCLUSIONS: Malignant schwannoma of paranasal sinuses and the anterior skull base is a rare tumor that involves a high rate of local invasion. The prognosis is poorer compared to that occurring in the trunk and extremities.

Contralateral external carotid-to-middle cerebral artery graft using the saphenous vein. Case report.

A variation of the extracranial-intracranial arterial bypass, using a long saphenous vein graft, is presented. The saphenous vein graft was inserted from the contralateral external carotid artery to the distal middle cerebral artery to replace the common and internal carotid arteries in a patient with a large neck tumor that invaded the common and internal carotid arteries, the esophagus, and the trachea. The patient had a positive balloon Matas' test. The saphenous vein was covered with an artificial vascular graft so that turning of the head or movement of the mandible did not displace or compress the graft. A large volume of flow began immediately after anastomosis. A description of the case and the operative technique is presented herein.

E.N.T. manifestations of Von Recklinghausen's disease.

Von Recklinghausen's Disease (VRD) is a neurocutaneous, systemic disease characterized by CNS tumors and disorders, cafe-au-lait spots, generalized cutaneous neurofibromata, skeletal deformities, and somatic and endocrine abnormalities. It is an autosomal dominant, hereditary disorder found in approximately 1:2500 to 3300 births. There are many manifestations of this disease in the head and neck region of interest to the otolaryngologist. Case reports of three patients with multiple ENT involvements are detailed. A review of the literature is presented with a brief discussion of diagnosis and treatment. The most common intracranial tumor in the adult is the acoustic neuroma, usually bilateral, while in the child it is the astrocytoma. A defect in the sphenoid bone is common and may produce temporal lobe herniation into the orbit causing pulsatile exophthalmos. Involvement of the facial bones usually causes radiolucent defects secondary to neurofibromata within nerve pathways, and a variety of asymmetrical changes, especially within the mandible. "Elephantiasis" of the face is a hypertrophy of the soft tissues overlying a neurofibroma, often quite extensive and disfiguring. Laryngeal and neck involvement may compromise the airway and early and repeated surgical intervention is required. The over-all malignancy rate approaches 30%, indicating that the patient with VRD may be predisposed to developing a malignancy. There appears to be an increased surgical risk in these patients, with some demonstrating abnormal responses to neuromuscular blockade.

Oral manifestations and differential diagnosis of isolated hypoglossal nerve palsy: report of two cases.

Isolated hypoglossal nerve palsy is rare, but occasionally it appears as the initial or solitary sign of an intracranial or extracranial space-occupying lesion, a head and neck injury, or a vascular abnormality of the internal carotid artery. Therefore it should be considered in differential diagnosis. We report two cases of isolated unilateral hypoglossal nerve palsy. In Case 1 the cause of the palsy appeared to be hypoglossal nerve neurilemmoma within the hypoglossal canal, whereas in Case 2 the cause could not be identified. Neither patient complained of any disability other than slight dysarthria. The tongue deviated toward the healthy side at rest and toward the affected side on protrusion. Hemiatrophy of the tongue with fatty displacement was demonstrated by means of T1-weighted magnetic resonance imaging. Dentists who might at times see patients with isolated hypoglossal nerve palsy should be aware of the significance of its oral manifestation, and they should be able to perform differential diagnosis of patients with the condition who appear for treatment.

Surgical access to parapharyngeal space tumours--the Manipal experience.

A few series of parapharyngeal space tumours have been reported earlier but recently not many series have been published in English literature. It is rare for any medical center, let alone an individual surgeon, to develop sufficient experience in evaluating these tumours. We present our experience in the treatment of 41 cases of parapharyngeal tumours from January 1992 to December 2001. FNAC, ultrasound and CT scan of the presenting mass was done in most of the patients as the main pre-operative work-up. The strategic location and extension of the tumour may occasionally alter the surgical approach for tumour excision.

Infraorbital nerve schwannoma: a rare cause of upper jaw swelling.

INTRODUCTION: Schwannoma of the infraorbital nerve is a rare entity, with lesser than a dozen cases reported in literature and only one from India. REPORT: This article reports a 23-year-old male presenting with a painless swelling in the cheek, which was eventually diagnosed as infraorbital nerve schwannoma. He underwent a complete excision of the tumor via a Caldwell Luc approach and continues to be disease free on 3 year follow-up. CONCLUSION: Despite its rarity, infraorbital nerve schwannomas should be considered in the differential diagnosis, of upper jaw swelling. We recommend the Caldwell Luc approach as safe, effective and cosmetically acceptable, for anteriorly based infraorbital schwannomas, and review literature on this unusual entity.

Endoscopic approach to the infratemporal fossa: anatomic study.

OBJECTIVE: Classic surgical exposures of the infratemporal fossa region, including the adjacent intracranial space, temporal bone, and sinonasal region, require the extensive exposure associated with the transcranial, transfacial, and transmandibular approaches with their inherent neurological and cosmetic morbidities. In this study, we evaluated the feasibility and exposure afforded by combining 2 endoscopic transmaxillary approaches, endonasal and Caldwell-Luc supplement, to the infratemporal fossa. METHODS: Endoscopic transmaxillary dissection was performed in 4 formalin-fixed cadaver heads (8 sides). We quantified the extent of exposure achieved within the pterygopalatine and infratemporal fossae after our initial dissection, which was endonasal with a medial antrostomy, and after addition of a Caldwell-Luc incision with an anterior antrostomy. Complementing this anatomic study, we report on a patient in whom this endoscopic transmaxillary approach combining the endonasal and Caldwell-Luc approaches was used for resection of a trigeminal schwannoma in the infratemporal fossa. RESULTS: The combination of these 2 endoscopic transmaxillary approaches enabled visualization of the entire region of the pterygopalatine fossa and anteromedial aspect of the infratemporal fossa. Additional posterolateral exposure of the infratemporal fossa requires significant traumatic traction on the nose. Addition of the Caldwell-Luc transmaxillary approach exposed the remainder of the infratemporal fossa, including the mandibular nerve and branches, middle meningeal artery, and even the distal cervical portion of the internal carotid artery. CONCLUSION: Endoscopic exposure of the infratemporal fossa is feasible. Using the combination of the endonasal and Caldwell-Luc approaches for direct transmaxillary access significantly extended exposure, allowing safe and effective resection of infratemporal fossa lesions.