William A. Weiss, MD, PhD

• Professor, Neurology UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA

https://profiles.ucsf.edu/william.weiss

T3 the tumor is limited to the larynx and paralyzes at least one of the vocal folds medicine information buy discount septra 480mg on line. T4a the tumor has spread to the thyroid cartilage and/or the tissue beyond the larynx medicine while breastfeeding order 480mg septra. T4b the tumor has spread to the area in front of the spine (prevertebral space) symptoms 6 months pregnant buy septra 480mg visa, chest area symptoms uterine fibroids buy generic septra online, or encases the arteries medicine zetia purchase septra 480mg without prescription. Mancuso and colleagues treatment quotes images order genuine septra, in an important prospective series published in 1999, demonstrated that local control was 89% in tumors less than 6 cc volume; in tumors larger than 6 cc, the local control rate at two years was 52% when primary radiotherapy was used as the principal treatment. In contrast, Hoorweg and colleagues studied 55 patients in whom the interrater reliability of tumor volume calculation, cartilage invasion, and cartilage sclerosis were found to have significant clinical variation. Other factors having a significant predictive value for likelihood of local recurrence in primary staging of glottic cancer irrespective of T stage include paraglottic fat invasion, preepigglottic fat invasion, subglottic invasion, anterior commisure involvement and cartilage invasion. Disease spread to these locations significantly impacts staging and treatment and would potentially be missed with clinical evaluation alone. Ultimately, disease extent is determined using a combination of clinical, endoscopic and radiological assessments. The larger impact is in assessment of nodal spread, metastases and local recurrence. As noted above, cartilage invasion is an important prognostic indicator for survival and resistance to primary radiotherapy. Although the presence of neoplasm on both sides of the cartilage (understandably) is the single best indicator of cartilage invasion,33,34 other features are also important. The presence of neoplasm adjacent to non-ossified cartilage as well as the obliteration of marrow space are specific but not sensitive signs of invasion of the arytenoid or cricoid cartilage. This was because peri-tumoral inflammation can alter adjacent marrow signal mimicking tumor invasion. If the T2 signal or T1 Gadolinium enhancement was more intense than the primary tumor, that implied peritumoral inflammation over direct invasion. This location is occult to direct clinical or endoscopic evaluation and best assessed with cross sectional imaging. The sagittal image highlights how this region is occult to direct visualization from the mucosal side of the airway. Although the larynx, or what is left of it, is technically easy to examine following cancer treatment, the interpretation of clinical findings is often more challenging. The following discussion focuses on the follow-up of patients treated non-operatively as well as the detection of second primaries. The significance of surveillance with regard to a second primary is illustrated in a comprehensive study by McGuirt and colleagues in which 377 surviving patients with non-total laryngectomy were examined for metachronous lesions. Five percent of the patients who survive more than three years developed second primary cancers in the larynx. The likelihood of a new laryngeal cancer was lower in the patients who were originally treated with radiotherapy (4. Patients with earlier stage malignancies are more likely, paradoxically, to develop second malignancies as they are more likely to survive their first malignancy. Often, the clinician is faced with a painful and dysfunctional larynx following radiation with or without chemotherapy; the larynx may be swollen and its endoscopic appearance significantly distorted. Detection of recurrence or metachronous lesions is understandably difficult in this situation. In 12 of 29 patients with local failure, "grade 3" findings were present a median of 5. Ideally, follow-up scans will show interval resolution of volume/mass effect and decreased inflammatory changes to support treatment change versus recurrent or residual disease; however, this is often not straight forward. The patient had a primary supraglottic squamous cell carcinoma with bulky ipsilateral cervical lymphadenopathy treated with radiation and chemotherapy. Image courtesy of Cameron Foster, Nuclear medicine department, University of California, Davis, 2014. Image courtesy of Cameron Foster, Nuclear Medicine, University of California, Davis, 2014. Although hyperbaric oxygen has been recommended for treatment, many patients undergo laryngectomy for the removal of a painful and dysfunctional larynx. Although uncommon overall, imaging is particularly helpful in identifying and determining the extent of non-epithelial malignant neoplasms, such as chondrosarcoma. In this paper, the authors noted that coarse or stippled calcification within the neoplasm was the most helpful radiologic finding and was universally present in their 10 3858 patients with chondrosarcoma. Other neoplasms do occur in the larynx, including neuroendocrine carcinomas, basilosquamous malignancies, and soft tissue sarcomas. Inflammatory Disorders Acute laryngeal inflammation ranges from the routine, such as viral laryngitis accompanying an upper respiratory infection, to the life threatening, such as epiglottitis. It is uncommon for imaging to be performed for the clinical extremes of these problems; for example, when a patient is in marked distress, management is focused on relief of airway obstruction. In less severe situations, a soft tissue plain lateral radiograph of the neck may be obtained by the emergency-department physician. In the patient with suspected epiglottitis, the classic "thumb print" sign can be seen. The clinical impression made by the patient on the physician will be dominated by the history and endoscopic examination, if it is possible to perform laryngoscopy on the patient. Laryngeal tuberculosis, although rare, may be endoscopically confused with carcinoma of the larynx due to the irregular appearance of the mucosa and chronic symptoms such as hoarseness and cough. In two review papers from the last decade, mycobacterial infection of the larynx was studied and noted to have several suggestive features that may help to distinguish it from malignancy. In addition, cartilage destruction was not seen in any patients with tuberculosis in these series. It would be hard to imagine deferring a biopsy of the laryngeal abnormality despite a high clinical suspicion of mycobacterial disease. The supportive radiographic information may be helpful in alerting the clinician to handle the sampled tissue with caution and obtaining appropriate cultures in addition to histopathological examination. Nonetheless, the principal motivation for clinicians pursuing imaging related to vocal-fold paralysis is to evaluate for a possible neoplastic cause. It is not clear what is the likelihood of detecting an otherwise occult neoplasm with radiography along the course of the laryngeal nerves. A recent publication from Finland reported that nine of 34 patients in the "non-iatrogenic" category harbored neoplasia, including those secondary to goiters and chest malignancies. Overall, the relative incidence of neoplasia as the cause of vocal-fold paralysis has declined in the past few years, with more patients with idiopathic and iatrogenic neuropathy. This matter has not been settled because of the advent of any thorough investigation or even a major retrospective study; it is a matter of practicality. The normal trachea is composed of 14 to 22 C-shaped rings of cartilage with a membranous posterior segment. Global airway physiology is dependent on respiratory mechanics, in addition to the "baseline" cross-sectional area, as the trachea lies partially in the neck and partially in the chest; it is thus subject to the thoracic negative pressure necessary for normal inspiration. This dynamic quality is exploited in radiographic examination of patients with suspected tracheomalacia. Tracheal Stenosis As noted in the section on laryngeal stenosis, imaging may be helpful in characterizing the length of the stenotic segment as well as in detecting 3862 secondary lesions. The radiological findings are generally not exclusive to any one cause, although, as noted above, patients with no history of mechanical injury, that is, no intubation or external trauma, are less likely to have calcification or ossification on their studies. In this modality, rapid image acquisition has significantly reduced respiratory and cardiac motion artifact in airway studies; prior to this, 3D reconstructions of airway lesions were put together from multislice images taken from different breath-holds. The patients (often debilitated and dyspneic) are required to hold their breath for 15 to 45 seconds, a potential limitation to this technique. It shows smooth narrowing of the tracheal air column (arrow) a few centimeters below the level of the larynx. The narrowing of the tracheal air column is readily evident although detailed characterization is limited. The next phase of imaging in airway evaluation for stenosis is referred to as "virtual endoscopy" by its proponents. The overall contribution of virtual endoscopy to clinical care is not known for tracheal stenosis. Neoplasms Affecting the Trachea Primary neoplasms of the trachea are rare; when they do occur, more are likely to be malignant than benign. There are few imaging distinctions among these cell types, other than their local behavior and what is known about their natural history. Other tumors, such as mucoepidermoid carcinomas, are believed to feature more "endoluminal" growth patterns but no one of these findings is consistent enough to be diagnostic. Carcinoid tumors, derived from neuroendocrine cells, tend to arise in central bronchi and not in the trachea. If the tracheocele becomes fluid filled, it may be more difficult to distinguish it from other paratracheal masses. Tracheomalacia Tracheomalacia typically presents with dyspnea on exertion or chronic cough. It is believed that the endotracheal tube cuff pressure destabilizes the cartilaginous trachea. In their study, the images of patients with bronchoscopically proven malacia were reviewed in a blinded manner. During inspiration, 16 of 17 patients had normal configuration; on expiration, all of the patients demonstrated some degree of abnormality, the "frown sign" being the most common, seen in nine of 17 patients. The presence of normal trachea above and below the stenosis is particularly helpful in these patients, as is the determination of the length of the stenosis. Dysphagia is a major clinical issue, and aspiration is a common cause of morbidity and mortality. More patients die each year of aspiration pneumonia complicating stroke than the head and neck cancers combined. The indications for this study include the assessment of patients with dysphagia, with or without suspected aspiration. In addition to providing structural and dynamic information about the area, this study provides an opportunity for a speech-language pathologist to assess the impact of postural maneuvers, swallowing strategies, and choices of ingested material in an attempt to guide the patient toward idealized safe oral intake if at all possible. Contrast entering the aditus of the larynx but not passing inferior to the vocal folds is referred to as penetration. If the ingested material enters the subglottis or trachea, aspiration has occurred. The authors concluded that "the low percentage of normal studies coupled with the high percentage of change in measurable variables indicate high clinical utility for the modified barium swallow study. Some concern has been raised over the possibility that the subjective nature of the test leads to variability in test results. Interestingly, the changes implied by the presence of a bar are more complex than simple mechanical impedance. They suggested that the underlying pathogenesis of the bar reflected reduced muscle compliance. In this valuable procedure, the patient is "fed" while a flexible nasopharyngoscope is in place. With the addition of laryngopharyngeal sensory testing, this purely endoscopic approach may have significant benefit in the care of stroke patients to give one important example. In addition, the examination can take place at the bedside or in the clinic at the time of the initial examination and does not require transportation to the radiography site. A major prospective paper from Aviv studied 126 subjects prospectively and 3870 followed them for one year. Unfortunately, despite excellent clinical research and promising initial data, enthusiasm for the laryngopharyngeal sensory testing component of the endoscopic swallowing evaluation has not expanded into general practice. The initial examination may be for the detection of a "leak" or extravasation of contrast from the hypopharynx into the soft tissues of the neck or the mediastinum. It is important to note that there are several other findings which may be of note on these "leak" studies. Jaramillo and colleagues reported on a series of patients with Zenker diverticula treated with endoscopic stapling; 15 of the 32 patients were restudied two years postoperatively. Twelve of the 15 patients surveyed were satisfied with the results of their procedure. The structure between the sac and the native esophagus is composed mostly of the cricopharyngeus muscle. Tsikoudas and colleagues investigated the association between radiological findings and outcomes in endoscopic stapling of Zenker diverticula. In short sacs with a broad angle between the sac and the native esophagus, there was a higher incidence of perioperative complications, both technical and medical. Zenker sacs with long necks and large pouches were associated with a higher rate of revision surgery. Few topics in laryngology and bronchoesophagology generate as much disagreement as gastroesophageal and laryngopharyngeal reflux. Generally, radiographic studies are not used as the first line of investigation for these disorders; nonetheless, contrast examinations of the esophagus may be helpful in some patients both in the assessment for reflux as well as for the detection of peptic complications. Esophagitis, resulting from peptic injury of the esophagus, can be readily detected in the double (barium and air) contrast esophagram; when used in combination with single-contrast views, the sensitivity approaches 90%. These reflux-associated findings typically occur in the area immediately superior to the gastroesophageal junction. Contrast esophagography is useful in distinguishing between neoplasia and luminal narrowing of the esophagus, as in the case of stricture. In symptomatic lower esophageal concentric narrowing, that is, Schatzki ring, if the lumen is compromised to a maximum diameter of 13 mm, dysphagia is almost always present; in contrast, a lumen of 20 mm rarely results in swallowing complaints. Benign tumors, such as leimyoma, represent the minority (20%) of esophageal neoplasia. Computed tomography reveals much of the same structural description; one review noted that leiomyomata mostly featured eccentrically elevated filling defects with homogeneous low- or isoattenuation.

Brannon and colleagues described 19 patients with ductal papillomas: 13 inverted ductal papillomas 98941 treatment code purchase septra 480 mg on-line, three sialadenoma papilliferums medications like zovirax and valtrex purchase septra online pills, and three intraductal papillomas symptoms pancreatic cancer buy septra cheap online. The inverted ductal papillomas and intraductal papillomas appeared as submucosal nodules symptoms zithromax generic septra 480 mg with amex. The lip and the palate were the most common locations for inverted ductal papilloma and sialadenoma papilliferum treatment of gout purchase 480mg septra amex, respectively treatment quadriceps strain purchase septra cheap. The sites for the three intraductal papillomas were the parotid papilla of Stensen duct, the upper lip, and the buccal mucosa. With light microscopy, inverted ductal papillomas appeared to arise from the excretory ducts near the mucosal surface, whereas intraductal papillomas appeared to arise from the excretory ducts at a deeper level. Sialadenoma papilliferum had a more complex histology, with a biphasic growth pattern of exophytic papillary and endophytic components. Histologic Types of Malignant Neoplasms Just as is the case with their benign counterparts, there is a wide variety of histologic types of malignant neoplasms. The broad categories include carcinomas, adenocarcinomas, malignant mixed neoplasms, lymphomas, and metastatic. Malignant neoplasms account for less than 15% of all salivary neoplasms; hence they are even more rare than benign-salivary neoplasms. Several malignant histologic types are more frequent and emphasis will be 4614 placed on them. The location in the salivary subunit in which the neoplasm originates is believed to correlate with its clinical aggressiveness with those of the excretory duct behaving more aggressively than neoplasms from the intercalated-duct region. Mucoepidermoid carcinoma is the most common malignancy of the salivary glands; the parotid gland represents the most common site accounting for 70% of these neoplasms. For minor salivary neoplasms, the palate and buccal mucosa are the most frequent sites. Patients may present with a range of symptoms, including a painless, slow-growing mass to a rapidly enlarging mass and cervical metastasis, with or without facial-nerve paralysis. Moderately differentiated (intermediate-grade) neoplasms have a tendency for local recurrence but metastasize uncommonly. Histologically low-grade neoplasms have more mucinous cells (>50%) while high-grade neoplasms may have a paucity of mucinous cells that may only be detected with immunohistochemical markers. Histopathologic features that are associated with poor outcome were cystic component less than 20%, four or more mitotic figures per 10 high-power fields, neural involvement, necrosis, and anaplasia. Billroth is credited for first describing this intriguing neoplasm with unique features, but the term "adenoid cystic carcinoma," which describes its histologic appearance was not coined until 1953. Adenoid cystic carcinoma is the second most common salivary malignancy with most series reporting the majority occurring in the submandibular gland and minor-salivary glands. A painless growth is the most common presentation with neural deficits depending on the site of involvement, including unilateral tongue weakness, facial paralysis, and trigeminal nerve distribution symptoms. The cribiform pattern has a glandular architecture with the classic features of a "Swiss cheese" appearance where cells are arranged in clusters and are separated by oval spaces. The tubular pattern demonstrates elongated tubular structures that have a central lumen and also show a glandular architecture. The solid pattern is characterized by high cellularity with sheets of cells with little evidence of glandular architecture. Any given neoplasm may have elements of all of the patterns, which leaves the pathologist with the challenge of deciding which pattern predominates. Patients with neoplasms with the solid pattern predominating have the worst prognosis while those with the cribiform pattern have the best. Adenoid cystic carcinoma has a strong proclivity for neurotropism and perineural spread, with a tendency for tracking along major neural structures. Skip lesions are common and are seen in most of these neoplasms, although it is believed that perineural spread occurs along the path of least resistance and is microscopically contiguous with the primary neoplasm. Distant metastases were the most common sites of failure (37%), but skull base and neck recurrences were also frequently observed. Microscopic disease and involvement of named nerves lead to worse prognosis, but with postoperative radiation, locoregional control was improved. Most recurrences occur in the first five years but recurrences occur commonly 20 years later and more. Because of frequent late failure, actual survival rates have been difficult to assess. Initial extent of disease was the dominant prognostic factor with advanced age and duration of symptoms also associated with poor outcomes. Combined treatment yielded an 85% locoregional freedom from relapse, and disease-specific survival at five, 10, and 15 years was 89, 67. Perineural invasion of major nerves, positive margins at surgery, and solid histological features were associated with increased treatment failures. Four or more symptoms present at diagnosis, positive-lymph nodes, solid histology, and perineural invasion of major nerves were associated with increased mortality from the neoplasm. Disease-free intervals varied from one month to 19 years (median 36 months) and exceeded 10 years in 9 of 113 patients (8%) with adequate information about treatment failure. The only significant factors influencing survival were the size of the primary neoplasm (p<0. Acinic cell carcinoma accounts for approximately 6 to 8% of salivary-gland malignancies, with 81 to 97% occurring in the parotid gland and it is the second most common pediatric salivary gland malignancy. It has a 3% chance of occurring bilaterally, second only to Warthin neoplasm in that regard. It tends to have a benign biological course and 4619 has the best overall outcome of all salivary gland malignancies. There are several histologic subtypes that do not generally correlate with survival. They include: solid, microcystic, papillary cystic, and follicular with the majority of cancers displaying multiple cell types. The intercalated-duct or reserve cells of the terminal duct are believed to be sites of origin. Rarely, there is a form of acinic cell carcinoma that is dedifferentiated with a more aggressive biological activity and worse prognosis; it usually has a higher rate of mitotic activity, nuclear atypia. Hoffman and colleagues evaluated 1,353 patients registered in the National Cancer Data Base for the years 1985 to 1995. Although acinic cell carcinoma usually has a favorable course, rarely an aggressive form occurs. It is important to clarify that because malignancies arising in the salivary-ductal unit are technically termed adenocarcinomas, adenocarcinoma not otherwise specified (nos) refers to a salivary malignancy that is generally high grade, has a high rate of metastases, and has a poor survival. They frequently have the morphologic features of adenocarcinomas of the breast and lung. They represent 1 to 9% of salivary malignancies,106 and they occur in minor-salivary glands most commonly (68%), followed by the parotid gland (28%) and submandibular gland (8%). It was first described in 1983 and has a strong propensity for minor-salivary glands, although it infrequently occurs in the parotid gland as well. It has also been termed terminal duct carcinoma and is 4620 thought to be derived from the intercalated-duct region of the salivary unit. The neoplasms were characterized by a polymorphous growth pattern, with individual neoplasms demonstrating solid, ductotubular, cribiform, trabecular, and single-file growth. There is no established benefit of radiation therapy for patients with this disease. It accounts for approximately 1% of salivary-gland malignancies and generally occurs in the parotid gland. The five-year survival was only 30% with 77% of the patients dying of the neoplasm at a mean interval of three years after diagnosis. It typically presents as a rapidly enlarging mass in the setting of a preexisting lesion. For those patients with recurrent pleomorphic adenoma, the risk of malignant degeneration is 7 to 10%. Tortoledo and colleagues reported in their series of 40 patients that depth of invasion <8 mm yielded a five-year survival of 100% compared to 50% for those with >8 mm of invasion. In this instance, both the primary and metastatic neoplasms have a completely benign morphology. The most common sequence is multiple local recurrences with eventual metastases to lung and bone. At least two recurrences generally occurred prior to identification of metastases. The metastases were discovered from six to 52 years following the occurrence of the primary neoplasm. Metastases were identified in bone, lung, regional lymph nodes, skin, kidney, retroperitoneum, oral cavity, pharynx, calvaria, and central nervous system. The usual presentation is a firm painless mass, either with or without facial paralysis. Five-year survival was 24% for patients with parotid lesions and 20% for those with submandibular neoplasms. As with other malignant salivary gland neoplasms, advanced stage and pain as a presenting symptom were ominous findings. Locoregional recurrence was the most common site of failure in primaries both in the parotid (51%) and in the submandibular (67%) glands. Salivary-gland lymphoma may be a localized or systemic disease and most often affects the parotid gland and rarely the submandibular gland. Although non-Hodgkin B-cell lymphoma is most prevalent, all forms of lymphoma may be observed. Auclair and colleagues reported 42 patients with sarcoma; 17 experienced recurrences, 16 developed metastases (most commonly to lung), and 15 died of disease. For large neoplasms, prognosis is poor, despite radical-surgical resection and radiation therapy. Enucleation without an adequate cuff of normal tissue for benign neoplasms such as pleomorphic adenomas leads to an unacceptably high rate of recurrence. Treatment of benign minor salivary gland neoplasms requires wide resection of the anatomic site where they occur. For malignant-parotid neoplasms, a total parotidectomy is recommended with preservation of the facial nerve, whenever possible. The management of nerves is always an important consideration for both parotid (facial) and submandibular (hypoglossal, lingual, marginal mandibular) malignancies, especially for tumors with a predilection for perineural involvement, ie, adenoid cystic carcinoma. When a nerve is non-functioning preoperatively, no attempts should be made to preserve it; rather obtaining clear margins should be the focus. When there is complete encasement of the facial nerve or its branches, sacrifice of the involved portions of the nerve is required with repair in the same setting. If the nerve is abutting tumor or can be dissected free of tumor, every effort should be made to preserve it. When the extratemporal portion of the facial nerve is sacrificed, primary repair with a cable graft is desired, irrespective of the need for postoperative radiotherapy. For malignant-submandibular neoplasms, complete excision of the submandibular gland is the minimum therapy. Because of the proximity of the mandible, a marginal or segmental mandibulectomy may be necessary. The extent of resection of minor-salivary neoplasms depends on the 4624 anatomic site involved. Lymphadenectomy Whenever there is clinically positive regional adenopathy, a therapeutic neck dissection is performed as part of definitive treatment. While not all of the indications for elective surgical treatment of the neck have universal agreement, generally a neck dissection is recommended when there is presence of highgrade tumors, advanced T-stage (T3, T4), and histology with extensive infiltration. Armstrong and colleagues compared 46 matchedpairs with malignant parotid neoplasms, one group treated with complete surgical resection whereas the other was treated with surgical excision and postoperative radiotherapy. The prognostic factors associated with worse outcome were skull-base involvement and surgical biopsy versus gross resection. Whether inadvertent or planned, facial-nerve section results in a cosmetic and functional deformity that requires rehabilitation. Certainly, temporary facial nerve paresis related to neurapraxia may result even when the nerve is preserved and able to be stimulated at low voltage at the conclusion of the operation. Gaillard and colleagues reviewed 131 patients in whom a superficial parotidectomy or total parotidectomy with nerve preservation was performed. The most common injury was of one branch (48%), with the marginal mandibular branch being the one most commonly affected. Total parotidectomy was associated with an increased incidence of early dysfunction, but there was complete resolution at the end of a six-month period. Otherwise, a cable graft should be performed with the possible donornerves including the greater auricular, sural, or antebrachial cutaneous. Static repair with facial slings, gold weight, and lid-shortening procedures are also important options depending on patient factors. Details of the surgical management of facial paralysis are discussed in Chapter 34, "Facial Paralysis. It is hypothesized to be the result of severed postganglionic parasympathetic nerve fibers reestablishing connection with sweat glands located in the dermis. Linder and colleagues, in a prospective study, demonstrated that 43% of patients were clinically symptomatic at one year. Whereas most patients are not markedly disturbed, a few patients (5 to 10%) suffer from severe gustatory sweating.

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Although it is often assumed that the effect of neurologic disease or damage is essentially the same for the speech musculature as it is for the muscles of the trunk or limbs symptoms quotes generic septra 480 mg visa, recent studies show that the muscle fiber types of the craniofacial muscles are distinct from those in the limb and trunk muscles symptoms your period is coming cheap septra 480mg overnight delivery, generally showing considerable polymorphism medications on backorder order 480mg septra otc. Muscle-fiber composition varies within and across muscles in the craniofacial muscles medicine shoppe locations generic septra 480mg mastercard, and the composition in humans differs from that in homologous muscles in nonhuman species medicine hat weather generic 480 mg septra fast delivery. This paragraph summarizes information on muscle fibers within different parts of the speech production system treatment 3rd degree hemorrhoids order 480 mg septra fast delivery. The vocalis muscle compartment of the thyroarytenoid muscle contains a large population of slow tonic muscle fibers that do not exhibit a twitch contraction but rather have contractions that are prolonged, stable, precisely controlled, and fatigue resistant (properties that seem highly suited to the demands of phonation in human speech). The mandibular 3548 muscles contain at least four different isoforms of myosin heavy chain, have a continuous range of contraction speeds, and have a high oxidative capacity (properties that are suited to variable dynamics and fatigue resistance). Like the other speech muscles just described, the palatal muscles also are fatigue resistant. In general, it can be said that the craniofacial muscles are unique in their genetic, developmental, functional, and phenotypical properties. Neurogenic speech disorders in adults usually appear as acquired conditions secondary to neural trauma or disease. The two general types are dysarthria and apraxia of speech (also known as verbal apraxia or dyspraxia). Dysarthria is a disorder of speech production (sometimes described as a disorder of motor execution) associated with lesions to the central nervous system, peripheral nervous system, or both, and generally is accompanied by motor signs of weakness, paralysis, or incoordination. As shown in Table 86-5, the acquired dysarthrias in adults are further classified into seven major types identified through their auditory-perceptual features and interpreted in terms of classic neurology. Apraxia of speech (sometimes described as a disorder of motor programming) is an isolated disorder of speech production characterized by difficulties in the sequencing or patterning of movements, but in the absence of motoric deficiencies in non-speech movements performed by the same musculature. The responsible lesion is nearly always in the language-dominant hemisphere, especially in the frontal or parietal lobes, but occasionally in subcortical regions. However, loss of praxis may be fundamentally different from a failure to develop praxis in the first place. Whether or not the terminology is apt, these terms are well established clinically and probably will continue to be used. Epidemiology of these disorders tracks that of the diseases with which they are associated. Most of these diseases are those of advancing age, especially stroke and the neurodegenerative diseases. An individual patient may have aphasia along with dysarthria, or aphasia along with apraxia of speech. For example, in respect to flaccid dysarthria associated with peripheral nervous system damage, the speech abnormalities depend on the particular pattern of cranial-nerve or spinal-nerve injury. Auditory-perceptual assessment is the core method of assessment, often done in accord with the classic descriptions of dysarthria. Instrumental methods can provide valuable supplementary information; among the most commonly used are acoustic, aerodynamic, and kinematic descriptions. Because surgical or pharmacological treatments often do not lead to complete resolution of the speech disorder, behavioral treatments are commonly used. The latter are employed especially when the speech disorder is severe and when exacerbation is likely, as in the case of neurodegenerative diseases. Behavioral speech treatments may accompany or follow other treatments including pharmacotherapy, neurosurgery, or prosthodontics. This definition is intended to exclude language impairments associated with diffuse brain damage, such as that in dementia. The focal nature of the neural damage invites hypotheses on clinicoanatomic relationships, and this issue has been a major topic of the literature on aphasia. The nature and severity of the impairment varies considerably, and there is controversy over the most appropriate classification of deficits in aphasia. One of the most well-established and frequently used systems is the Boston Diagnostic Aphasia Examination, which uses the classification summarized in Table 86-6. Stroke is the main cause of aphasia, and the epidemiology of aphasia relates closely to the epidemiology of stroke. Aphasia is found in 21 to 38% of acute stroke patients and is linked with high morbidity, mortality, and expenditure. Although aphasia usually is associated with stroke, it can result from other neuropathologies such as tumors or trauma. The responsible lesion is typically cortical (left hemisphere in the majority of cases), but aphasia can result from subcortical lesions, including those of the thalamus, putamen, and internal capsule. Several tests or test batteries have been developed for the assessment of aphasia, with the basic goal of classifying type and severity of aphasia. The classifications shown in Table 86-6 are frequently, but not universally, used. A number of pharmacological treatments for aphasia have been suggested, but the evidence favors only a modest benefit from amphetamine and the dopamine agonist piracetam. The effects on speech and voice depend on the extent of damage (eg, laryngectomy, glossectomy, mandibulectomy). Epidemiology follows the responsible medical condition, making it difficult to offer a concise summary. The main objective in assessment is to identify the effects on 3553 speech of limitations in anatomy and physiology. Fortunately, a number of compensations permit adequate speech production even in individuals who have experienced serious alteration of the oral and laryngeal structures. Prosthetics can often restore a basic functionality that can be further enhanced by behavioral therapy. Few studies have been reported on the prevalence and risk factors for voice disorders in adults. Discussion of the assessment of vocal function is found in Chapter 85, "Assessment of Vocal Function. Several behavioral interventions have been described, many of which are similar to those mentioned previously with respect to voice disorders in children. Dysphagia is an impairment of deglutition and can affect any aspect or stage of this process. Dysphagia is a critical concern in many medical conditions, and it may contribute to malnutrition, dehydration, aspiration pneumonia, and death. Less severe difficulties with swallowing can interfere with quality of life, including reduced pleasure in eating and reduced 3554 social interaction, particularly when eating is involved. Prevalence of dysphagia in the general population is difficult to estimate, but a study of 947 primary care patients noted that 22. As noted in the preceding discussion of epidemiology, dysphagia is associated with various causes. Among the most frequently occurring are neural disorders that impair one or more of the stages of swallowing (oral preparation, oral transit, pharyngeal, or esophageal). Dysphagia also can result from trauma to the aerodigestive tract or from surgical procedures such as laryngectomy or pharyngolaryngectomy. Patient complaint is not always a reliable indication of swallowing difficulties, as these problems may be insidious in their development. Videofluoroscopic swallowing studies are useful to detect abnormalities in the different stages of swallow. Treatment is based on an understanding of the contributing factors, and a variety of surgical, pharmacologic, and behavioral interventions may be considered. Recent studies of oropharyngeal dysphagia have shown a positive outcome for lingual strengthening exercises. Although prevalence estimates vary considerably, it is likely that these disorders have a prevalence of about 6 to 8% in the general population. These disorders may be of increasing concern because of the growing emphasis placed on communication for educational, vocational, and social pursuits in this digital age. Epidemiologic and etiologic information is far from satisfactory for some of the most frequently occurring disorders. Multifactorial origins may underlie a large proportion of patients with specific language impairment, stuttering, speech sound disorders, and possibly craniofacial anomalies. As shown in Table 86-4, 3555 speech and language disorders are linked to loci on nearly all autosomes as well as the X sex chromosome. These complex genetic influences are matched by a tangle of environmental variables that affect the development of speech and language in children. Some disorders of speech and language persist into adulthood, either in essentially the same form (eg, stuttering) or in different forms (such as a reading disorder that may be related to an earlier phonological or language disorder). Co-occurrence of disorders can complicate diagnosis and treatment, but it may also provide insights into the etiology of these conditions. Above all, the development of speech and language in children is a dynamic process that involves the interplay of sensory, motor, and cognitive systems in a social environment of complex stimuli and interaction. Speech and language capabilities can be compromised in both children and adults, often with profound effects on communicative interactions in a host of settings. The relative frequency of English consonant sounds in words in the speech of children in grades one, two, and three. Childhood speech disorders: reported prevalence, cormorbidity and socioeconomic profile. Prevalence of speech delay in 6year-old children and comorbidity with language impairment. A genome scan in multigenerational families with dyslexia: identification of a novel locus on chromosome 2q that contributes to phonological decoding efficiency. Motor sequencing deficit as an endophenotype of speech sound disorder: a genome-wide linkage analysis in a multigenerational family. Synthesis of research on phonological awareness: principles and implications for reading acquisition. Outcomes at school age of preschool children with developmental language impairment. Screening for speech and language delay in preschool children: recommendation statement. Non-specific nature of specific language impairment: a review of the literature with regard to concomitant motor impairments. Motor impairments in children associated with impairments of speech or language: a meta-analytic review of research literature. A major susceptibility locus for specific language impairment is located on 13q21. Ten principles of grammar facilitation for children with specific language impairments. The efficacy of treatment for children with developmental speech and language delay/disorder: a meta-analysis. Effects of computer-based intervention through acoustically modified speech (Fast ForWord) in a severe mixed receptive-expressive language impairment: outcomes from a randomized controlled trial. Changes in the birth prevalence of selected birth defects after grain fortification with folic acid in the United States: findings from a multi-state population-based study. New complexities in the genetics of stuttering: significant sex-specific linkage signals. Dissecting and reinterpreting the evidence in light of spontaneous recovery rates. Randomised controlled trial of the Lidcombe programme of early stuttering intervention. Epidemiology of aphasia attributable to first ischemic stroke: incidence, severity, fluency, etiology, and thrombolysis. Evidencebased systematic review: effects of intensity of treatment and constraintinduced language therapy for individuals with stroke-induced aphasia. Voice disorders in the general population: prevalence, risk factors, and occupational impact. The prevalence of dysphagia in primary care patients: a HamesNet research network study. While a full history and physical exam remains cornerstone to diagnosis, there is well-established role for high-speed, high-quality visualization of the vocal folds to narrow the differential diagnosis. The use of such imaging allows real-time visualization of nearly the entire larynx including propogation of the vibratory mucosal wave, symmetry of propagation, and glottic closure when videostroboscopy is employed. Even subtle benign lesions are now visualized, allowing greater accuracy of diagnosis and improved efficacy of treatment. This chapter serves to highlight the spectrum of benign disease that can affect the larynx. This rapid vibration causes incredible mechanical stress during the collision of the vibratory surfaces of the folds. These lesions tend to be unilateral but can present as bilateral lesions; they can be pedunculated or have a broad, sessile base. While cysts may also arise from these same traumatic mechanisms, such lesions are differentiated by the existence of an epithelial-lined space. Generally bilateral, symmetric, midmembranous, and subepithelial vocal-fold lesions. Diagnosis Videostroboscopy is incredibly useful in differentiating nodules, polyps and cysts. These lesions appear white and opaque and result in an hourglass closure pattern of the glottis. These are most visible at higher registers of phonation as the stretching of the vocal folds thins the density of the fold, allowing more obvious pathology. Polyps can be visualized in similar fashion and tend to be unilateral but can present bilaterally.

It is imperative to keep the child breathing spontaneously during this evaluation to avoid loss of the airway if a completely obstructing lesion is present medicine zoloft buy 480mg septra free shipping. Complete obstruction may be identifiable on preoperative imaging evaluation medicine 5113 v discount septra 480 mg amex, but in many patients medicine runny nose purchase 480mg septra with mastercard, sedation is required for imaging which can lead to unexpected airway loss in the radiology suite symptoms 8 days after iui generic septra 480mg line. If the airway obstruction is encountered during operative evaluation treatment west nile virus order septra 480mg on-line, mask ventilation can be carried out while a tracheotomy is performed distal to the lesion medications equivalent to asmanex inhaler generic 480 mg septra with visa. A laser delivering bronchoscope or flexible laser can be of help in obtaining hemostasis. Treatment and Follow-Up Treatment of pediatric laryngeal and tracheal tumors centers upon resection. Both open and endoscopic approaches are advocated depending on the site and pathology of the lesion. Endoscopic approaches should be limited to tumors that do not extend beyond the airway lumen otherwise clear resection margins are not attainable. For isolated laryngeal lesions, an open approach via a thyrotomy and extended cricoid incision provides optimal access for removal. Depending on the site of the tracheal lesion, trans-cervical or trans-thoracic approaches may be necessary. Key points to determine are the location of the mass in relation to the carina and the subglottis, whether resection will allow for primary anastomosis, and whether enough trachea can be mobilized to ensure a tension-free repair. Postoperative-airway management will depend on the size of the child and the ancillary support of the hospital. Many older children undergoing tracheal resection can be extubated at the end of the procedure. Extensive open laryngeal procedures may require a temporary tracheostomy or laryngeal stenting if grafting materials are used for reconstruction. Adjunctive chemotherapy and radiation therapy are dependent on the final pathology of the tumor. Although trauma accounts for 35 to 50% of childhood mortality, less than 1% of blunt trauma leads to laryngotracheal injury. The pediatric larynx lies at the level of C3 to C4 vertebra affording greater protection by the hyoid and mandible. The broader and more pliable cartilage also allows for increased endolaryngeal protection. There is, however, an increased risk of swelling due to the loose attachment of the submucosal laryngeal tissues to the perichondrium. This arrangement predisposes children to airway compromise due to minimal edema in small-diameter airways. Traumatic intubation identified after multiple attempts at intubation by emergency services. Signs and Symptoms the most common presenting symptoms of blunt trauma include neck pain, hoarseness, cough, and shortness of breath. The most common signs include cervical ecchymosis, endolaryngeal hematoma, and hemoptysis. Although laryngeal trauma is most commonly associated with motor vehicle injury, whenever one evaluates an injured child, the potential for abuse should be kept in the differential. Concerning history in this respect includes repeated injury, multiple injury sites, and injury not consistent with the history. Diagnosis 3143 Early suspicion and recognition of laryngeal and tracheal trauma are keys to avoiding long-term airway, voice, or swallowing complications in the pediatric patient. A child in acute airway distress obviously mandates emergent evaluation in the operating room. Less obvious signs and symptoms can be further evaluated with flexible nasopharyngoscopy and imaging. With small diameter chip tip endoscopes, it is possible to evaluate children with subtle injuries to the vocal-fold structure that previously were missed with standard laryngoscopes. Young children who cannot tolerate a flexible endoscopic examination, or in whom such a bedside examination is contraindicated, may need to be considered for a formal operative endoscopic evaluation. Imaging can play an adjunctive role in the evaluation and management of laryngeal trauma. Plain x-rays are of limited benefit, but free air may suggest laryngotracheal injury. Common traumatic injuries include edema or hematoma of the glottal structures, laceration of the false- and true-vocal folds, arytenoid cartilage dislocation, anterior commissure disruption, cartilage fracture, and laryngotracheal separation. Injury staging is based on the widely used adult system devised by Schafer and Fuhrman Table 76-1). Nondisplaced fractures Group 3 Massive edema, large mucosal lacerations, exposed cartilage, displaced fractures, vocal-cord immobility Group 4 Similar to Group 3 but more severe with disruption of anterior larynx, unstable fractures, two or more fracture lines, severe mucosal injuries Group Complete laryngotracheal separation 3144 5 Treatment and Follow-Up the principles of trauma treatment for children mirror those of adults with priority given to airway, breathing, and circulation. Basic trauma management principles should also be followed such as those pertaining to cervical spine protection. Securing the airway quickly and successfully is of utmost importance as children are susceptible to rapid demise if oxygenation and ventilation cannot be obtained in a timely manner. As opposed to adults, in whom emergent cricothyroidotomy or tracheostomy is a relatively uncomplicated procedure, an emergent cricothyroidotomy can be extremely difficult in children and result in iatrogenic laryngeal trauma. The higher positioning of the pediatric larynx, together with the cricoid telescoping under the thyroid cartilage, prevent accurate landmark palpation in the majority of children. With major laryngotracheal injury, securing a definitive airway either via an endotracheal tube or a tracheostomy is of utmost importance. This requires coordination with the anesthesia team and ancillary support services. The disadvantages of emergent intubation are lack of familiarity with pediatric intubation among first response personnel and the potential to worsen a tenuous airway. The difficulties of a cricothyroidotomy or tracheostomy in an uncontrolled setting were discussed in the previous paragraph. In general, the safest manner to secure an injured pediatric airway is in the operating room using spontaneous ventilation techniques. Although rapid sequence induction and intubation remain the gold standard for pediatric airway trauma management, one should give pause in considering this modality. The overall risk of aspiration is low in children and should be weighed against the risks of an inability to establish an airway in a 3145 traumatic setting. If spontaneous ventilation can be maintained, more options for securing a definitive airway can be executed, including intubation over flexible and rigid endoscopes as well as ventilation through a rigid bronchoscope. Once a stable airway has been secured and a detailed evaluation of the larynx and trachea has been made, a decision regarding conservative or surgical therapy is needed. Observation is recommended when there is minimal edema, small hematomas, and small lacerations not involving the vibratory edge of the vocal fold or the anterior commissure. Surgical management is required when there is a likelihood of poor airway patency and suboptimal swallowing or voice results. Repair of significantly displaced or comminuted thyroid or cricoid cartilage fractures can be done with a combination of grafts, permanent sutures, or plates. If needed, resorbable plates may be an optimal solution as titanium plates have the theoretical disadvantage of growth restriction. Laryngeal procedures may require endoscopic or open approaches used in isolation or combination, similar to those described for airway reconstruction. Decisions regarding the necessity of stents and tracheostomy versus extended intubation are left to the discretion and training of the surgeon and the support of ancillary hospital services. Adjuvant medical treatment in children with laryngeal injuries includes antibiotics, shortterm corticosteroids, and reflux therapy. The acute management of inhalation injury does not differ from that of blunt injury. Intubation is often indicated with upper aerodigestive tract edema or pulmonary injury. With inhalation injuries, a low threshold should be given toward airway evaluation and stabilization, especially in infants who require more than 180 mL/kg of fluid resuscitation where resultant edema can lead to airway loss. Early evaluation via direct laryngoscopy and bronchoscopy can help identify the extent of an inhalation injury, but historically repair is deferred until the scar has matured. This "wait and see" approach is being reassessed in this era of balloon technology in which balloon dilation may be able to prevent a worsening scar. Once placed correctly in the airway, withdrawal of the plunger will reveal air bubbles. Algorithm provides a reasonable management process for pediatric laryngeal fractures. Insights into the maturation of the child larynx and functional changes in voice are providing objective methods to help analyze surgical indications and outcomes in the growing child. There is ethical controversy with respect to laryngotracheal transplantation in which the risks of chronic immunosuppression must be weighed against the benefit of transplanting a non-vital organ. Although tracheal transplantation is less complicated than laryngeal transplantation in which airway, voice and swallowing all need to be coordinated, tracheal transplantation is not as simple as reconnecting an autologous trachea. Successful transplantation is extremely challenging due to the absence of a defined arterial blood supply, as well as constant contamination from the outside world. The only successful pediatric tracheal graft to date involved an autologous stem cell based tissue engineered trachea in a child with congenital tracheal stenosis; this child continues to do well three years after surgery. Airway manifestations of pediatric eosinophilic esophagitis: a clinical and histopathologic report of an emerging association. Eosinophilic esophagitis in children: a pathologic or clinicopathologic diagnosis The spectrum of pediatric eosinophilic esophagitis beyond infancy: a clinical series of 30 children. Characterization of supraglottic phonation in children after airway reconstruction. Pediatric laryngotracheal stenosis and airway reconstruction: a review of voice outcomes, assessment, and treatment issues. Comparison of pediatric voice handicap index scores with perceptual voice analysis in patients following airway reconstruction. Current research in voice and swallowing outcomes following pediatric airway reconstruction. Partial cricotracheal resection in children: potential pitfalls and avoidance of complications. Partial cricotracheal resection for severe pediatric subglottic stenosis: update of the Lausanne experience. Aerodynamic and acoustic assessment in children following airway reconstruction: an assessment of feasibility. The use of posterior cricoid grafting in managing isolated posterior glottic stenosis in children. Posterior glottic stenosis and bilateral vocal fold immobility: diagnosis and treatment. Minimally invasive endoscopic management of subglottic stenosis in children: success and failure. Endoscopic anterior cricoid split with balloon dilation in infants with failed extubation. Refining indications for the use of mitomycin C using a randomized controlled trial with an animal model. Preservation of function and histologic appearance in the injured glottis with topical mitomycin-C. The role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract: friend or foe Mitomycin: effects on laryngeal and tracheal stenosis, benefits, and complications. Preliminary results of intraoperative mitomycin-C in the treatment and prevention of glottic and subglottic stenosis. Topical mitomycin application after laryngotracheal reconstruction: a randomized, double-blind, placebocontrolled trial. The anterior cricoid split procedure for the management of subglottic stenosis in infants and children. Laryngotracheoplasty as an alternative to tracheotomy in infants younger than 6 months. Pediatric partial cricotracheal resection: a new technique for the posterior cricoid anastomosis. Partial cricotracheal resection with primary anastomosis in the pediatric age group. Cricotracheal resection for pediatric subglottic stenosis: update of the Lausanne experience. Cricotracheal resection as a primary procedure for laryngotracheal stenosis in children. Partial cricotracheal resection for congenital subglottic stenosis in children: the effect of concomitant anomalies. Sandu K, Monnier P Partial cricotracheal resection with tracheal intussusception and cricoarytenoid joint mobilization: early experience in a new technical variant. Pediatric laryngotracheal reconstruction with cartilage grafts and endotracheal tube stenting: the single-stage approach. Single-stage laryngotracheal reconstruction: the Great Ormond Street experience and guidelines for patient selection. One slide fits all: the versatility of slide tracheoplasty with cardiopulmonary bypass support for airway reconstruction in children. Slide tracheoplasty in infants and children: risk factors for prolonged postoperative ventilatory support. Management of pediatric airway granular cell tumor: role of laryngotracheal reconstruction. Percutaneous transtracheal needle insufflation: a useful emergency airway adjunct simply constructed from common items found on your anesthesia cart.