Sinonasal adenoid cystic carcinoma is a rare malignancy characterized by an insidious growth pattern and a tendency for perineural spread along major and minor nerves, resulting in invasion of the skull base and intracranial extension. Therefore, many patients present with advanced disease and involvement of critical structures, making treatment difficult and potentially associated with high morbidity. Surgery represents the mainstay of treatment of the primary tumor. Complete resection of the tumor with negative margins, whenever feasible, is associated with better survival outcomes. However, in the case of extensive involvement of vital structures (e.g., carotid artery, cavernous sinus, optic nerve, Meckel's cave) or when radical surgery could seriously affect the patient's quality of life, a function-preserving subtotal removal of the tumor followed by irradiation can be proposed. The role of surgery is limited to a biopsy in unresectable lesions that are more suitable for non-surgical treatments (e.g., exclusive chemoradiation). Given the difficulty in obtaining negative margins and the propensity for submucosal and perineural spread, adjuvant radiotherapy is strongly recommended. Recently, heavy-particle radiotherapy using protons or carbon ions has emerged as a promising treatment with improved local control. Local failures (60%) and distant metastases (40%) are common and can occur even decades after definitive treatment. The 5-year overall survival ranges from 55 to 70% and it exceeds that of other sinonasal malignancies, but dramatically drops down at 10 years (40%) and further decreases at 20 years (15%). Therefore, a prolonged follow-up of at least 15 years, and possibly lifelong, is mandatory.
Sinonasal adenoid cystic carcinoma is a rare malignancy characterized by an insidious growth pattern and a tendency for perineural spread along major and minor nerves, resulting in invasion of the skull base and intracranial extension. Therefore, many patients present with advanced disease and involvement of critical structures, making treatment difficult and potentially associated with high morbidity. Surgery represents the mainstay of treatment of the primary tumor. Complete resection of the tumor with negative margins, whenever feasible, is associated with better survival outcomes. However, in the case of extensive involvement of vital structures (e.g., carotid artery, cavernous sinus, optic nerve, Meckel's cave) or when radical surgery could seriously affect the patient's quality of life, a function-preserving subtotal removal of the tumor followed by irradiation can be proposed. The role of surgery is limited to a biopsy in unresectable lesions that are more suitable for non-surgical treatments (e.g., exclusive chemoradiation). Given the difficulty in obtaining negative margins and the propensity for submucosal and perineural spread, adjuvant radiotherapy is strongly recommended. Recently, heavy-particle radiotherapy using protons or carbon ions has emerged as a promising treatment with improved local control. Local failures (60%) and distant metastases (40%) are common and can occur even decades after definitive treatment. The 5-year overall survival ranges from 55 to 70% and it exceeds that of other sinonasal malignancies, but dramatically drops down at 10 years (40%) and further decreases at 20 years (15%). Therefore, a prolonged follow-up of at least 15 years, and possibly lifelong, is mandatory. (c) 2020 S. Karger AG, Basel
Laryngeal transplantation offers the hope of replacing voice and laryngeal function in patients with debilitating laryngeal injuries or loss of the larynx from trauma or oncologic reasons. Our group at UC Davis performed a laryngotracheal transplantation, and our experience is reviewed in this chapter. The indications, challenges, and limitations of this procedure are highlighted, and the world's other published cases are reviewed.
Olfactory neuroblastoma is a rare tumor. Nasal endoscopy typically identifies a soft mass arising from the olfactory cleft. Computer tomography and magnetic resonance imaging are mandatory for staging (in association with F-18-fluorodeoxyglucose positron emission tomography) in high-grade and/or high-stage tumors. Biopsy must be representative to confirm a diagnosis and for grading purposes. Two complementary classifications are described: one (Kadish) based on clinical-radiological analysis, and the other (Hyams) on histological criteria. Based on Hyams grading, studies have pointed out that grades III-IV entail significantly different behavior and prognosis. A multimodal approach, which may combine surgery, chemotherapy, and radiotherapy, is essential to manage these tumors. Treatment schedules which include surgery seem to be superior to others. Surgery classically consisted of anterior craniofacial resection to obtain good exposure. However, the role of transnasal endoscopic surgery has expanded because of its association with fewer complications, shorter hospital stays, and comparable oncologic results to the open surgical techniques. Unilateral endoscopic craniectomy can be performed for limited lesions to avoid definitive anosmia. Treatment that includes radio- and chemotherapy is recommended for advanced and high-grade tumors. The role of neoadjuvant chemotherapy in advanced-stage lesions is emerging. The main prognostic factors associated with poor patient outcome are Hyams grade III-IV, Kadish C-D, and positive surgical margins. Lifelong follow up is recommended. (c) 2020 S. Karger AG, Basel
Vocal fold immobility can be either unilateral or bilateral and partial or complete. The aim of this chapter is to discuss the management of unilateral paresis using medialization thyroplasty with or without arytenoid adduction as a means of treating neurogenic causes as opposed to mechanical fixation. Medialization thyroplasty is an open surgical procedure that is performed under local or general anesthesia. Essentially, it aims to close the glottic gap, approximating both vocal folds together and thereby allowing for restoration of the efficiency of the larynx. The glottic gap results from atrophy of the affected vocal fold and in so doing results in glottic insufficiency which causes voice breathiness, strain, fatigue, aspiration, and swallowing difficulties that make up the bulk of symptoms associated with this condition. Unlike injection laryngoplasty, medialization thyroplasty does not increase the "bulk" of the atrophic vocal fold but merely brings the fold closer to its unaffected partner. Besides the obvious lateralization, there is occasionally a third dimensional component to the affected fold. The slipping and prolapse forward of the arytenoid cartilage due to atrophy of the muscles supporting it and the natural declination of the facet joint it rests on cause a vertical drop of the level of the affected vocal fold that may not be remedied with the medialization procedure, hence requiring arytenoid adduction. Although attempts to medialize the vocal fold have been described in the past with limited access, the basic premise of creating a window in the thyroid cartilage remains central. The differences between materials used, their respective strengths and weaknesses, the pitfalls and pearls in achieving a good closure and improvement in voice, swallow, and safety of the airway are all discussed accordingly.
The role of imaging in assessment of neurolaryngology will predominantly involve the assessment of pathology along that of laryngeal nerve pathways. The anatomical pathways of the vagus and laryngeal nerves are well described in standard anatomical textbooks and will not be detailed here. Whilst there are 3 principle laryngeal nerves/branches, the recurrent laryngeal nerve will clearly constitute the mainstay of imaging input. This chapter will elaborate on the pathology encountered at the various levels and the imaging characteristics of these disorders. Prior to this, there will be a summary of normal laryngeal imaging appearances to familiarise with the characteristics of radiological anatomy.
Nowadays, surgeons have a wide armamentarium of surgical approaches available to safely treat sinonasal malignancies, which includes open approaches, with the traditional craniofacial resection (CFR), and endoscopic transnasal techniques. The correct choice depends on the features of the pathology. It is well known that endoscopic approaches have a lower morbidity compared with traditional open techniques, due to a shorter hospitalization, absence of facial incisions, and avoidance of brain retraction. Moreover, endoscopic surgery presents clear technical advantages. For example, magnification of the surgical field allows the surgeon to carefully identify tumor margins, the site of origin, and the anatomical structures involved by the lesion. Nevertheless, a purely endoscopic approach cannot always provide successful resection of the tumor; the patient must be informed about the possibility of switching to a combined cranioendoscopic resection or CRF, depending on the effective extension of the disease evaluated intraoperatively. Despite these advantages, postoperative complications can occur after endoscopic endonasal surgery, as in any surgical intervention; however, complications after these procedures are less severe and less frequent compared with traditional open approaches. The most common complications observed include skull base reconstruction failure, intraoperative vascular lesions, and orbital or central nervous system complications. Thus, endoscopic endonasal resection, when properly planned and performed by experienced surgeons, is an acceptable treatment for well-selected skull base malignancies with long-term outcomes comparable to those achieved with traditional external approaches.
Sinonasal tumours are rare, and among these there exist a small number of histologic subtypes that are infrequently encountered and rarely mentioned in the literature. These have been presented as either case reports or small case series, and their very low incidence makes prospective studies practically impossible. This review analyses the available literature, including our own experience and endeavours to outline management strategies, which involve a high index of suspicion and counselling of patients. In most instances, these tumours require aggressive multimodal treatment to improve survival outcomes. The overall prognosis remains dismal.
Adduction arytenopexy is a surgical procedure that allows the surgeon to position the arytenoid cartilage in a vocally favorable position with a suture. It is not needed in most cases of vocal paralysis when there is favorable synkinesis and good positioning of the arytenoid body. When there is a large posterior gap (intercartilaginous region), height discrepancy, or an anteriorly displaced arytenoid, adduction arytenopexy is used to suture the arytenoid cartilage into the posterior and medial aspect of the cricoarytenoid joint facet, bringing the medial bodies of the arytenoid cartilages together allowing increased closure resulting in higher dynamic range in postoperative voices. It increases the tension in the vocal fold, allowing for improved vocal quality. Adduction arytenopexy is typically combined with medialization laryngoplasty to support the vocal fold and increase the subglottic pressure that can be achieved. Cricothyroid subluxation is performed to allow the surgeon to select the degree of tension to match the opposite, working vocal fold. When there is accumulation of secretions and food in the ipsilateral dilated pyriform sinus, a hypopharyngoplasty is added to decrease the volume of the pyriform sinus and improve swallowing.
Anterior skull base meningiomas are a diverse group of tumors that involve different locations. The role of the expanded endoscopic transnasal approach in the management of anterior cranial fossa meningiomas has significantly changed over the past 2 decades. Patient selection is paramount to benefit from the advantages of endoscopic transnasal surgery such as direct access to ventral skull base lesions avoiding brain and brainstem retraction, early de-vascularization, removal of bone infiltrated by tumor, near-field magnification, better surgical field illumination, and minimal manipulation of neurovascular structures. Here we discuss some of the main limitations and advantages of the endoscopic transnasal approach related to patient selection, including nuances of their management, discussing preoperative imaging and planning, the surgical approach, and future perspectives in the treatment of these tumors.
Neuromuscular diseases (NMDs) are diseases involving the motor nuclei of the cranial nerves and anterior horn cells of the spinal cord, the peripheral nerves, the neuromuscular junction, and/or muscle itself. The following groups of disorders from the WHO International Classification of Diseases are usually included in the classification of NMDs: (1) Motor neuron diseases or related disorders; (2) Disorders of nerve root, plexus or peripheral nerves; (3) Diseases of neuromuscular junction or muscle. These diseases include myasthenia gravis or certain specified neuromuscular junction disorders, primary disorders of muscle, and secondary myopathies. The following discussion selects NMDs from each group, which may have unique laryngeal features and more commonly involve the laryngologist and highlights different diagnostic and treatment considerations related to the larynx.
Sinonasal malignant mucosal melanoma (SNMM) is a rare, aggressive, and capricious tumour accounting for 4% of sinonasal malignancies. Recent studies suggest an increasing frequency. There are few large published series, but all authors report poor outcomes irrespective of treatment of approximately 25% 5-year survival. As a consequence, the American Joint Committee on Cancer (AJCC) have restaged all SNMMs as T3 or greater, irrespective of extent. Surgery remains the principle treatment modality. Survival and recurrence data analysis from a single-centre prospective cohort of 125 cases (all treated surgically with or without radiotherapy) showed 5-year overall survival was 28% and disease-free survival was 23.7%. Local control was achieved for a median of 21 months, with a 5-year disease control rate of 27.7%. However, endoscopically resected cases showed a significant overall survival advantage up to 5 years, confirming that endoscopic resection of SNMM does not adversely affect outcome and may even be beneficial up to 5 years. These findings are supported by other recent series in the literature. Radiotherapy did not improve local control or survival in this study, though there is debate in the literature as to its value. Cervical metastases confer a dramatically worse outcome. Chemotherapy has not previously shown much advantage, but more recently immunologic manipulation with drugs such as ipilimumab have shown promise. Thus far, the mutation status does not appear to affect survival outcomes, but NRAS mutations are relatively frequent and could be targeted in this disease by MEK inhibitors in the future.
Spasmodic dysphonia (SD) is a rare focal laryngeal dystonia. It is characterized by task-specific voice dysfluency resulting from selective intrinsic laryngeal musculature hyperfunction. Symptoms may be attenuated by a sensory trick. Although SD can be seen at times in generalized dystonia syndromes, it is typically a sporadic phenomenon. Involvement of the laryngeal adductor muscles is more common than abductor muscles. The standard treatment of this disorder is with botulinum toxin injection, usually electromyography-guided, which must be repeated periodically as the toxin wears off. A number of non-reversible surgical procedures have also been described to mitigate the symptoms. Other treatment modalities are under investigation, including implantable electrical stimulation devices and deep brain stimulation.
Visual diagnosis of laryngeal neurologic impairments is not only possible but is perhaps the most accurate method for evaluating the neurologic status of the upper airway. Precise assessments may lead to appropriate treatment without an EMG study. Principles of the neurolaryngology examination include: (1) each muscle has a single action; (2) that action can be elicited and to some degree isolated and visualized; (3) each muscle has an appropriate time to contract; (4) that timing can be compared to the opposite side; (5) inappropriate timing represents reinnervation; (6) inappropriate degree of motion represents reinnervation; (7) patients naturally compensate for any loss; (8) removing compensation during an exam reveals pathology. Some of the visual diagnostic findings available to the astute endoscopic examiner are (1) paralysis, (2) paresis, (3) synkinesis, (4) fixation, (5) tremor, (6) spasm, and (7) reinnervation.
In the setting of a voice clinic, the voice may be assessed in a number of different ways. As a bare minimum, assessment should include stroboscopic examination, patient self-reported questionnaires, and clinician-reported perceptual evaluation. In addition, recordings of the voice may be analyzed using computer software: several different measures exist, but the most widely used are jitter, shimmer, and noise-to-harmonic ratio. There are, however, significant limitations of these measures, including access to the equipment, inter-test reliability of the measurements, and a lack of correlation with clinical improvement. Other mathematical techniques (nonlinear algorithms) may provide more robust measurements. A pragmatic approach to assessment in the voice clinic suggests that stroboscopic examination should be accompanied by patient-reported questionnaires and clinician-rated voice assessments.
Laryngeal electromyography (LEMG) is an important diagnostic and prognostic tool in neurolaryngology. This chapter presents the advances in LEMG in the last 5 years based on clinical trials. LEMG is in most cases an office-based procedure for decision making in neurolaryngology. When performed in a standardized way, LEMG helps to differentiate abnormal function of the vocal folds from nonorganic disorders. It is mainly used to confirm the diagnosis of vocal fold paralysis (VFP) and is less frequently used for the diagnosis of myopathies and motor neuron disorders. Also, the value of LEMG for other diseases than VFP is presented. First consensus recommendations for standard performance of LEMG have been published. This will facilitate the comparison of results from different researchers in the future. For many years, LEMG focused on the thyroarytenoid muscle. Data are presented showing that now more and more studies are performed including other laryngeal muscles, most importantly the posterior cricoarytenoid muscle and cricothyroid muscle. Diagnostics and estimating the prognosis for patients with VFP remains the most important issue for LEMG. First meta-analyses are now available analyzing which LEMG parameters are the best prognosticators for recovery or persistent palsy. Due to the advances in multichannel LEMG, laryngeal nerve monitoring can now control the recurrent laryngeal nerve and the external branch of the superior laryngeal nerve during thyroid surgery. Finally, also the advances of LEMG for laryngeal pacing, guided laryngeal injections, laryngeal electrostimulation, and other indications beyond otolaryngology are discussed.
Posttreatment imaging surveillance in patients treated for anterior skull base tumors is a multifaceted issue which - as a first step - requires tailoring of the selection of imaging technique and acquisition protocol to the clinical scenario. As a general rule, acute symptoms suggesting the onset of a complication of treatment require prompt diagnosis through an easily accessible technique, such as CT; on the other hand, monitoring recurrences in asymptomatic patients is best achieved with MRI, exploiting the inherently higher contrast resolution. The interpretation of follow-up imaging studies is challenging. First of all, it is essential to be aware of specific characteristics of the natural history that may differ significantly between histologies, influencing the pattern and timing of recurrences. Additionally, resection of the lesion and reconstruction of the defect (as well as radiation treatment) produce complex anatomical changes, which may mislead inexperienced radiologists; this concept emphasizes the centrality of collecting accurate information on treatment modalities and procedures applied before scanning in each patient. This enables the pattern of expected posttreatment changes to be anticipated and, consequently, recurrences or complications to be more easily identified.
