Technique Nuances for Functional Preservation of Lower Cranial Nerves during Surgical Management of Ventral Foramen Magnum Meningiomas Via a Dorsal Lateral Approach.

BACKGROUND: This study established novel technique nuances in surgery for ventral foramen magnum meningiomas (vFMMs) via a dorsal lateral approach. METHODS: From July 2012 to July 2019, 37 patients with vFMMs underwent tumor resection surgery and were operated on with a dorsal lateral approach. Two safe zones were selected as the entrance of the surgical corridor. Safe zone I was located between the dural attachment of the first dental ligament (FDL) and the branches of C1; safe zone II lay between the dural attachment of the FDL and the jugular foramen. The tumor was debulked first through safe zone I and then through safe zone II. The tumor was removed through a trajectory from the caudal to cephalad to allow tumor debulking from below and downward delivery, away from the brainstem and lower cranial nerves. RESULTS: Thirty-three patients underwent gross total resection, and 4 patients underwent subtotal resection. Four patients transiently required a nasogastric feeding tube. All patients recovered within 3 months postoperatively. Three patients (8.1%) developed permanent mild hoarseness and dysphagia as a result of postoperative damage of cranial nerves IX and X. One patient underwent tracheotomy. No patient experienced tumor recurrence during the follow-up period. CONCLUSIONS: We established a minimal retraction principle, in which the selection of 2 safe zones as the entrance of the surgical corridor, tumor removal from the inferior to superior direction, and debulking followed by devascularization were the key elements to implement the minimal retraction principle in vFMM surgery.

Cranial Nerve IX and X Weakness: An Unusual Initial Presentation of Myasthenia Gravis.

BACKGROUND Myasthenia gravis (MG) is an autoimmune disease characterized by antibodies binding skeletal muscle acetylcholine receptors (AChR). Rarely does the disease manifest with orolaryngeal symptoms before ocular ones. We present a case of MG that on initial presentation had symptoms of cranial nerves (CN) IX and X weakness, including dysphagia and dysphonia. CASE REPORT A 51-year-old woman with panic attacks presented to the Emergency Department (ED) with complaints of her throat closing, swallowing difficulty, and hoarse voice. Multiple ED visits revealed no etiology. However, she developed stridor, which prompted further evaluation. Laryngoscopy and imaging studies revealed no gross abnormalities; therefore, her symptoms of dysphonia and difficulty breathing were attributed to anxiety. Her hospital course was complicated by a cardiac arrest requiring intubation. ECHO, CTA chest, and MRI brain were unremarkable. Her cardiac arrest was hypothesized as being secondary to laryngeal spasm. During her ICU course, she failed extubation multiple times due to acute respiratory failure. An autoimmune etiology was suspected, prompting a paraneoplastic screen, which revealed elevated levels of AChR antibodies at 124 mmol/L. MG was diagnosed and treatment with plasmapheresis and steroids was initiated. However, complications of thrombocytopenia, anemia, and ARDS ensued, so MG treatment was discontinued. The patient was eventually transferred to a LTACH. Thereafter, at outpatient followup, her MG was treated with mycophenolate and prednisone, which led to significant symptom improvement. CONCLUSIONS MG commonly presents in the third decade with clinical features of ptosis, diplopia, and facial weakness. However, initial and isolated symptoms of dysphagia and dysphonia are rare, leading to missed diagnoses. Our case of a middle-aged woman posed a diagnostic challenge because of her uncommon presentation and comorbidities of panic attacks and obesity. This case highlights the crucial need for a high index of clinical suspicion for MG in any patient presenting with symptoms of CN IX and X weakness.

Utility of the plasma pancreatic polypeptide response to modified sham feeding in diabetic gastroenteropathy and non-ulcer dyspepsia.

BACKGROUND: The relationship between cardiovascular and gastrointestinal (ie, plasma pancreatic polypeptide [PP] response to modified sham feeding [MSF]) indices of vagal function is unclear. Hyperglycemia inhibits PP secretion via vagally mediated mechanisms. Our aims were to (a) compare the PP response, (b) its relationship with glycemia, and (c) the relationship between PP response to MSF, gastric emptying (GE) of solids, and symptoms during GE study in healthy controls, patients with diabetic gastroenteropathy (DM), and non-ulcer dyspepsia (NUD). METHODS: In 24 healthy controls, 40 DM, and 40 NUD patients, we measured plasma PP concentrations during MSF, cardiovagal functions, GE, and symptoms during a GE study. KEY RESULTS: Baseline PP concentrations were higher in DM than in controls and NUD (P = .01), and in type 2 than in type 1 DM patients (P < .01). The PP increment during MSF was normal (≥20 pg/mL) in 70% of controls, 54% of DM, and 47% of NUD patients. Overall, the PP response and cardiovagal tests were concordant (P = .01). Among patients with a reduced PP increment with MSF, 7/10 of T1DM and 1/7 of T2DM patients had moderate or severe cardiovagal dysfunctions (P < .05). The PP response to MSF was not associated with GE. CONCLUSIONS & INFERENCES: Up to 30% of healthy controls have a reduced PP increment during MSF, limiting the utility of this test to detect vagal injury. The PP response is more useful when it is normal than abnormal. A reduced PP response is more likely to be associated with cardiovagal dysfunctions in T1DM than in T2DM.

Vocal cord paralysis due to ectopic parathyroid adenoma and function recovery: a case report and review of the literature.

Ectopic parathyroid adenomas (PAs) can occur in numerous locations and are thought to be the cause of a significant portion of failed primary surgery for hyperparathyroidism. PA is a rare cause of hoarseness, which may be harbingers of a malignant process. Here, we describe an unusual case of an ectopic PA in the carotid sheath presenting as unilateral vocal cord paralysis (VCP). A 49-year-old lady presented with a 1-week history of hoarseness, irritating cough and shortness of breath. Fibreoptic laryngoscopy revealed left VCP. Ultrasound and computed tomography of the neck demonstrated a mass in the carotid sheath. Laboratory investigations revealed hypercalcemia (3.10 mmol/L), hypophosphatemia (0.81 mmol/L) and elevated intact parathyroid hormone (iPTH) level (381.6 pg/mL), despite of a negative 99mTc-sestamibi scan. After more rigorous tests, the ectopic tumor adjacent to the left vagus nerve was successfully resected, with subsequent histopathological confirmation of PA. The patient eventually got a normal iPTH level and serum calcium postoperatively, and regular voice function was also regained 4 months after surgery. This case emphasizes the importance of broad differential diagnosis and thorough workup. Although most patients with PA present with hypercalcemia, this disease entity also need to be considered in the differentials of neck masses and VCP.

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.

Tapia's syndrome: pathogenetic mechanisms, diagnostic management, and proper treatment: a case series.

BACKGROUND: Tapia's syndrome is an uncommon disease described in 1904 by Antonio Garcia Tapia, a Spanish otolaryngologist. It is characterized by concomitant paralysis of the hypoglossal (XIIth) and pneumogastric (Xth) nerves. Only 69 cases have been described in the literature. Typically, the reported patients presented with a history of orotracheal intubation. Common symptoms are dysphonia, tongue deviation toward the affected side, lingual motility disturbance, and swallowing difficulty. CASE PRESENTATION: In the report, we describe three cases of Tapia's syndrome in three Caucasian patients who underwent surgery with general anesthesia. Two of these patients underwent neck abscess drainage, and the third had an open reduction of a shoulder fracture. The clinical symptoms of Tapia's syndrome appeared after extubation. All three of our patients recovered their lost function at 3 months after diagnosis. CONCLUSIONS: We underline the importance of performing airway endoscopy and a specific program of swallowing rehabilitation for the proper management of Tapia's syndrome.

Carotid artery pseudoaneurysm after orthognathic surgery causing lower cranial nerve palsies: endovascular repair.

PURPOSE: Reported complications following Le Fort osteotomies are rare but can include epistaxis from disruptions or pseudo-aneurysms of the maxillary artery or its distal branches the descending palatine and sphenopalatine arteries, aseptic necrosis of the maxilla, ophthalmic injuries including blindness, ophthalmoplegia, and keratitis sicca, and arteriovenous fistulas or false aneurysms of the carotid arteries (external and/or internal). The mechanism of injury to neurovascular structures can be the result of direct or indirect trauma, such as injuries from surgical instruments, traction injuries during manipulation of the osteotomized bone segments or during inadvertent manipulations of the head and neck, or from fractures extending to the base of the skull, orbit, or pterygopalatine fossa associated with the pterygomandibular dysjunction or maxillary downfracture. CASE REPORT: An 18 year-old male with facial bone dysplasia, apertognathia, maxillary hypoplasia and mandibular hyperplasia was treated with maxillary Le Fort I osteotomy with internal fixation and elastic intermaxillary fixation. Following surgery, the patient developed palsies of the vagus and accessory nerves manifesting as dysphagia, cough, vocal cord paralysis and trapezius muscle atrophy. Cross sectional imaging revealed a small, laterally pointing pseudoaneurysm of the high cervical internal carotid artery (ICA) at the skull base, exerting pulsatile mass effect on adjacent lower cranial nerves. The patient was treated with carotid artery stent reconstruction and pseudoaneurysm coil obliteration, and kept on dual antiplatelet therapy for two months. Partial recovery from cranial nerve palsies was observed within a year. CONCLUSION: A small, broad-based, laterally-pointing ICA pseudoaneurysm at the exit of the carotid canal without surrounding hematoma was clearly demonstrated on CTA, which visualization was difficult on MRA due to considerable metallic artifact from surgical hardware. Angiography exquisitely demonstrated the pseudoaneurysm, which was fully repaired with the combination of stenting and coil obliteration, allowing total preservation of the ICA.

Persistent cough associated with osteophyte formation and vagus nerve impingement following cervical spinal surgery: case report.

Persistent cough due to irritation of the vagus nerve by osteophytes resulting from cervical spinal surgery is a very rare condition. The authors report the case of a 49-year-old woman who presented with a persistent cough subsequent to cervical spinal surgery. One year after the initial operation, the patient underwent surgery to free the larynx from the prevertebral fascia and cut the pharyngeal plexus, but her symptoms persisted. In order to control the cough, she used a soft cervical collar with padding inserted in the left side so that the larynx would be pushed to the right, a solution she discovered on her own. Without the collar, she coughed uncontrollably. A CT scan was performed and showed an osteophyte that had developed at the level of the prosthesis. Based on these findings, the authors hypothesized that the cough was caused by vagus nerve irritation due to the osteophyte. The osteophyte was resected and the vagus nerve was moved to a position anterior to the carotid artery and was isolated by means of an autogenous tensor fascia lata graft. The patient's symptom disappeared immediately after the surgery. At the most recent follow-up visit, 18 months after surgery, the patient was symptom free and was pursuing regular daily activities without using a cervical collar.

[A case of Churg-Strauss syndrome presenting with lower cranial neuropathy].

We reported a 60 year-old man with Churg-Strauss syndrome (CSS). Three months later, he presented with dysarthria, dysphagia and severe headache. We detected glossopharyngeal and vagal nerve palsy, and made a diagnosis of cranial nerve involvement comorbid with CSS. Intravenous administration of methypredonisolone was effective for alleviating clinical signs and symptoms. Two months later, he complained of headache and facial numbness, but symptoms improved with an escalating dose of prednisolon. As compared to previously reported cases, our case was characteristic because of involvement of lower cranial nerve with CSS, which has been reported previously in only one case.

Successful treatment of a rare metastatic malignant carotid body tumour in a young adult, with conservative surgery and local radiotherapy.

OBJECTIVE: We report a patient with a malignant carotid body paraganglioma treated with surgery and adjuvant radiotherapy. We discuss her treatment and outcome in the light of the published literature. CASE REPORT: A 26-year-old woman presented with a 12-month history of a painless, left-sided neck lump. Ultrasound, computed tomography and magnetic resonance imaging revealed a carotid body tumour, which at surgical excision was found to be adherent to the vagus and hypoglossal cranial nerves (X and XII). The tumour was resected from the surrounding structures. Two local lymph nodes were removed to allow access. The internal carotid artery was also involved and had to be repaired with a synthetic graft. Histology and immunohistochemistry confirmed malignant carotid body paraganglioma. There were positive resection margins, and cervical lymph node metastasis was reported in one of the two nodes. Post-operatively, she had left Horner's syndrome, left vocal fold palsy and right upper limb weakness, all of which resolved spontaneously. She underwent adjuvant radiotherapy and remained recurrence free after 30 months. CONCLUSION: Malignant carotid body paraganglioma can affect young adults, with an insidious onset of symptoms. In this patient, local excision (without neck dissection) and adjuvant radiotherapy were well tolerated and resulted in satisfactory local disease control.

The relationship between heart rate variability and time-course of carcinoembryonic antigen in colorectal cancer.

BACKGROUND: Identifying new prognostic factors is important for guiding treatments and preventing metastasis in cancer. Vagal nerve activity may predict prognosis in cancer due to its roles in modulating inflammation, sympathetic activity and oxidative stress. This study tested the relationship between heart rate variability (HRV), a vagal nerve index, and the colon cancer (CC) marker carcinoembryonic antigen (CEA), in an 'historical prospective' design. METHODS: We examined data of 72 CC patients, without inflammatory or cardiac diseases, of whom 38 had baseline electrocardiograms (ECG) and 12 month CEA levels. We measured HRV (SDNN, RMSSD) from brief archived ECG. Multiple confounders were considered. RESULTS: Controlling for effects of tumor stage and treatment-orientation, baseline HRV predicted CEA levels at 12 months (r=-.43, p=.006). Patients with SDNN<20 ms had significantly higher CEA at 12months than those with SDNN>20 ms. CONCLUSION: These preliminary results showed that higher HRV predicts lower levels of a tumor marker, one year later, independent of confounders. This supports the hypothesized role of vagal activity in tumor modulation. Replication in larger samples is needed.

[Villaret's syndrome caused by internal carotid artery dissection].

We report a patient with Villaret's syndrome (left hypoglossopharyngeal nerve, vagus nerve, accessory nerve, and hypoglossal nerve palsies and left Horner's sign) caused by internal carotid artery dissection. He had neck pain on the left side, Horner's sign on the left side and paralysis of the left hypoglossopharyngeal nerve, vagus nerve, accessory nerve, and hypoglossal nerve. Brain MRI revealed no signal from the left internal carotid artery and no brain infarction, although a tumor-like lesion was observed in the left internal carotid artery. Subsequent MRI studies revealed intramural hematoma in the left internal carotid artery, and on the basis of this finding, he was diagnosed with internal carotid artery dissection. He received anticoagulant and antiplatelet therapy. His symptoms improved gradually. The symptoms of internal carotid artery dissection are neck pain, Horner's sign, brain infarction, and lower cranial nerve palsy. A characteristic feature in this case was that brain infarction was not observed. Only 3 similar cases have been reported in the past In all these cases, the patients had a good clinical course and showed complete recovery from the symptoms. Compared with western countries, in Japan, carotid artery dissection is rare. Carotid artery dissection should be considered as a differential diagnosis of lower cranial nerve palsy.

Cricothyroid approximation for voice and swallowing rehabilitation of high vagal paralysis secondary to skull base neoplasms.

This study documents the speech and swallowing outcomes of isolated ipsilateral cricothyroid approximation (aka tensioning thyroplasty; Type IV thyroplasty) for the treatment of high vagal paralysis (combined superior laryngeal nerve and recurrent laryngeal nerve paralysis). This is a pilot study of five cases with high vagal paralysis consequent to skull base neoplasms. Unilateral cricothyroid tensioning sutures were used. In all cases, vocal fold tensioning and vertical realignment of lax vocal folds were achieved. A partial, but acceptable medialization of vocal cord position was achieved. In all cases, aspiration was minimized and normal swallow function was restored by 6 weeks. The voice outcome was excellent in four cases and acceptable in one. Cricothyroid approximation restores vocal fold tension; in addition, it restores vertical vocal fold position and partially restores horizontal vocal fold position. Good voice and swallowing outcomes have been achieved. The procedure is quick, safe, and convenient when combined with a skull-base excision procedure. Further evaluation is merited.

Cardiac autonomic control in patients with myasthenia gravis and thymoma.

OBJECTIVE: To evaluate cardiac autonomic control in patients with myasthenia gravis (MG) and thymoma. MATERIALS AND METHODS: The study was performed on 21 patients with MG and thymoma and the same number of matched healthy volunteers. Standard cardiovascular reflex tests according to Ewing and baroreflex sensitivity (BRS) at rest was applied. Spectral analysis of heart rate variability (HRV) at rest was assessed using a 20-minute ECG recording (normalized low- and high-frequency bands-LFnu-RRI, HFnu-RRI and LF/HF-RRI) Time-domain analysis of HRV was derived from 24-hour ECG monitoring. RESULTS: Overall autonomic score according to Ewing was significantly increased in patients with MG and thymoma (p<0.05), mostly due to parasympathetic dysfunction. Time-domain parameters representing the overall and long-term sympathetic activity of HRV did not differ significantly between the two groups (p>0.05), but there was a significant decrease in measures of the short-term vagal variations in HRV (p<0.01). HFnu-RRI was lower, while LFnu-RRI and LF/HF-RRI were higher in patients with MG and thymoma in comparison to healthy controls but these differences were not of statistical significance (p>0.05). BRS at rest was highly significantly reduced in patients group (p<0.01). CONCLUSIONS: Our results showed mainly parasympathetic cardiac impairment in patients with myasthenia gravis and thymoma. Since autonomic dysfunction may lead to cardiac conduction abnormalities and sudden death, the investigation of autonomic nervous system function in these patients may be significant in everyday clinical practice.

Unilateral high vagal paralysis: relationship of the severity of swallowing disturbance and types of injuries.

OBJECTIVE: To evaluate the prognoses of high vagal lesions and their association with their clinical presentation, and seek to determine the best strategy of management for each patient. STUDY DESIGN: Retrospective case series with chart review. MATERIALS AND METHODS: Patients following laryngoplasty from 2001 to 2008 at a tertiary referral voice and swallowing center in Taiwan were reviewed. They were divided into two groups according to the etiologies of high vagal trauma. The pre- and posttreatment voice and swallowing functions were reviewed; swallowing status was compared between patient groups. RESULTS: Of 186 consecutive patients who underwent laryngoplasty, 8 females and 9 males were diagnosed of unilateral high vagal damage. One group of 11 patients suffered vagal damaging suddenly from skull base trauma, cerebrovascular accidents, and surgical complications; symptoms of vagal paralysis occurred immediately after those events. The second group was comprised of six patients who lost their high vagal functions gradually from a skull base tumor or mass compression. Feeding tube status was significantly different between the groups. All except one patient in group 2 recovered their voice and swallowing abilities after appropriate laryngoplasty. CONCLUSIONS: High vagal nerve damage from skull base surgery or trauma leads to a higher incidence of feeding tube dependency than that from skull base tumor compression. Patients can be treated successfully with an appropriate injection or medialization thyroplasty. Immediate laryngoplasty is suggested for cases right after skull base surgery or trauma.

A case of Collet-Sicard syndrome presenting to the Oral and Maxillofacial Surgery Department and a review of the literature.

A case of Collet-Sicard Syndrome caused by skull base metastasis of probable breast adenocarcinoma is reported. A 79-year-old lady presented to the Oral and Maxillofacial Surgery Department with progressive left-sided tongue symptoms that she described as swelling. This was initially attributed to local disease, and a biopsy showed the patient was suffering from Necrotizing Sialometaplasia. However, her symptoms rapidly evolved into cranial nerve palsies affecting IX-XII, not initially diagnosed. Subsequent imaging revealed the cause of her worsening symptoms to be a metastatic lesion at her left skull base. Cranial nerve palsies due to metastases to the skull base are rare, and the authors would advise clinicians to adopt a high-index of suspicion in ruling out cranial nerve pathology at the skull base when encountering unusual signs and symptoms in the head and neck region.

Craniofacial gunshot injury resulting in pseudoaneurysm of the left internal maxillary artery and Collet-Sicard syndrome.

Facial gunshot is a challenging medico-surgical emergency because of the complex neurovascular structure in the area. We present a patient who sustained a facial gunshot injury, with massive nasal and oral bleeding that resulted in shock. We review the management of gunshot injury to the internal maxillary artery, both in acute and chronic stages. In addition, we describe the clinical course of the rarely occurring Collet-Sicard syndrome, which involves injury to the 9th, 10th, 11th, and 12th cranial nerves associated with gunshot injury.

Vagolysis.

We describe the case of a 75-year-old man presenting with labile hypertension and symptomatic postural hypotension 13 months following radiotherapy for squamous cell carcinoma of his external auditory canal. Magnetic resonance image (MRI) scan demonstrated scarring and a probable recurrence of his tumour. He underwent autonomic testing, including muscle sympathetic nerve activity (MSNA), heart rate (HR) and blood pressure (BP) responses to a variety of stimuli. Results were consistent with baroreflex failure. Urinary catecholamine levels were within the high normal range. We postulate that baroreflex failure was caused by vagal and glossopharyngeal nerve damage secondary to radiotherapy and tumour recurrence. This diagnosis is rare, but should be considered with pure autonomic failure and phaeochromocytoma in the presence of labile hypertension, especially in patients with a history of radiotherapy to the neck and high-normal catecholamine levels.