Pathology

Rhabdomyosarcoma is classified into three cell types embryonal, alveolar (which has botryoid and spindle cell variants), and pleomorphic. The embryonal subtype accounts for 60 to 70 of childhood cases and is the most common subtype. It is also the most common subtype presenting in the head and neck (20-22). Rhabdomyosarcomas are malignant tumors of skeletal muscle. They are part of the larger group of soft-tissue sarcomas. Sarcomas of bony origin are classified separately. Like most malignant...

Jugular foramen syndrome

Jugular foramen syndrome (JFS) is an uncommon clinical entity that often presents a diagnostic and management challenge to the otolaryngologist. The purpose of this section is to define this syndrome and elucidate the epidemiology, pathogenesis, clinical manifestations, diagnostic workup, and general treatment options for the syndrome. Various combinations of palsies of cranial nerves IX, X, and XI resulting from lesions in the area of the jugular foramen have been referred to as JFS. Eponyms...

Head and Neck

Ulcerative Lesions Skin

Oral ulcerations represent the most common finding of disease and are seen in over 95 of patients with Adamantiades-Behget's. They are required for diagnosis (Table 1), although some argue that in rare cases, oral ulcers need not be present for the diagnosis to be made. The oral ulcerations seen in Adamantiades-Behget' s may be present on the tongue, lips, gingival surfaces, buccal mucosa, soft palate, or posterior pharynx (Fig. 1). In general, they are shallow and painful, resolving in...

Sarcoidosis

This is an idiopathic chronic granulomatous disease that occasionally affects the larynx. Peak onset is from the age of 20 to 40 years. There is a higher incidence in African Americans, Puerto Ricans, and Scandinavians, and it is sometimes familial. The pathogenesis of sarcoidosis is unknown. One theory is that it results from a mycobacterial infection. It is usually systemic, most commonly including the lungs and hilar lymph nodes. Isolated local involvement is rare, so head and neck...

Clinical Manifestations

The clinical presentation of AFS can be similar to CRS. Patients often report chronic nasal congestion and nasal obstruction that has failed to respond to antibiotic therapy. Examination of the nasal cavity may reveal nasal polyps within the olfactory cleft, middle meatus, and sphenoethmoid recess. Occasionally, locally destructive changes from longstanding polyposis lead to proptosis and malar flattening (7). AFS is unilateral in more than 50 of patients but may involve several sinuses...

Treatment

Systemic treatment of WG consists of a combination of glucocorticoids (GC) and a cyto-toxic agent. For life- or major organ-threatening disease, daily cyclophosphamide (CYC) therapy is used (7). After three to six months, when disease remission or significant improvement is achieved, CYC is discontinued to avoid the significant morbidity associated with prolonged exposure to this agent. A less toxic agent such as methotrexate (MTX) (15) or azathioprine (AZA) (16) is used to try to sustain...

Diagnosis

The vast majority of patients with hearing loss need only an audiogram to confirm the diagnosis. As stated earlier, most of these patients will be diagnosed with diseases limited to the inner ear such as presbycusis, noise-induced hearing loss, and ototoxicity. Asymmetry in hearing loss warrants the performance of magnetic resonance imaging (MRI) scan with paramagnetic enhancement to rule out a retrocochlear etiology. In the pediatric patient with unilateral sensorineural hearing loss, a CT...

Crohns Disease

Crohn's disease is a systemic inflammatory disorder affecting the entire GI tract. Although Crohn's disease most commonly affects the distal alimentary tract, esophageal involvement does occur. One study of patients without esophageal symptoms showed evidence of esophageal involvement in 5 by upper endoscopy (21). The typical findings are small, punctuate ulcerations in the esophageal mucosa. Rarely, fissures may form leading to fistula formation with adjacent organs. When patients are...

Rhinocerebral Mucormycosis

RCM represents one-third to one-half of all cases of Zygomycosis. The process originates in the nose and paranasal sinuses following inspiration of fungal spores. It is estimated that 70 of the cases of rhinocerebral zygomycosis occur in the setting of DKA (7). Disease starts with symptoms consistent with sinusitis. Low-grade fever, dull sinus pain, drainage, and soft-tissue swelling are initially seen, followed in a few days by double vision, increasing fever, and obtundation. Examination...

FIGURE 16 Oral hairy leukoplakia associated with EBV Source From

(PCR) for CMV viral load in serum can be used, but the clinical utility of the test is not yet clear. Whether treatment is beneficial for oral ulcers caused by CMV is not clear. Ulcerative esophagitis-causing symptoms can be treated with IV antivirals such as ganciclovir, foscarnet, or cidofovir, followed by oral maintenance, usually with oral valganciclovir. Epstein-Barr Virus and Oral Hairy Leukoplakia. OHL has been encountered since the early epidemic and has been strongly linked to...

Pernicious Anemia

Pernicious anemia is associated with atrophic gastritis. The marked loss of gastric parietal cells results in a deficiency of intrinsic factor, which is required to transport Vitamin B12 across the intestinal mucosa. The resulting deficiency of Vitamin B12, which is necessary FIGURE 43 Pernicious anemia. This elderly female complained of a painful tongue. The mucosa of the anterior portion of the tongue was severely atrophic and totally devoid of lingual papillae. Posteriorly, the mucosa was...

F

FIGURE 4 Erythematous form of oral candidiasis of the palate. Source Courtesy of the International AIDS Society-U.S.A. From Refs. 3, 4, 11. FIGURE 4 Erythematous form of oral candidiasis of the palate. Source Courtesy of the International AIDS Society-U.S.A. From Refs. 3, 4, 11. FIGURE 6 Erythematous candidiasis on the dorsal tongue surface. Source Courtesy of the International AIDS Society-U.S. A. From Refs. 3, 4, 11. FIGURE 6 Erythematous candidiasis on the dorsal tongue surface. Source...

References

Arch Otolaryngol 1981 107(1) 1-11. 2. Cook SP, Macartney KK, Rose CD, et al. Lyme disease and seventh nerve paralysis in children. Am J Otolaryngol 1997 18(5) 320-323. 3. Peltomaa M, Pyykko I, Seppala I, et al. Lyme borreliosis and facial paralysis a prospective analysis of risk factors and outcome. Am J Otolaryngol 2002 23(3) 125-132. 4. Bingham PM, Galetta SL, Athreya B, et al. Neurologic manifestations in children with Lyme disease. Pediatrics 1995 96(6)...

Differential Diagnosis

Multiple diseases can present with findings similar to those seen with Adamantiades-Behget's disease and should be considered when a patient presents with recurrent oral or genital ulcers, inflammatory eye disease, or other manifestations of vasculitis. Included in the differential diagnosis are systemic lupus erythematosus (Chapter 1), seronegative spondyloarthropathies, inflammatory bowel disease (Crohn's or ulcerative colitis) (Chapter 20), herpes or other viral infections (Chapter 10),...

Myeloproliferative Disorders

PRV is frequently discovered incidentally when a complete blood count is performed for another reason. When symptoms are present, they are usually nonspecific. Fatigue, headache, and diaphoresis are common. Pruritis, often following a hot shower, is a frequent complaint. Up to 15 of patients may present with a thrombotic episode. Thrombotic cerebrovascular accidents, coronary artery thrombosis, Budd-Chiari syndrome, and pulmonary embolus all occur. Cavernous sinus thrombosis may also occur in...

Infectious Diseases

Hearing loss is an uncommon presenting symptom of a systemic infectious disease but may develop during the course of several viral and bacterial infections. Pathogens may infect the inner ear and auditory pathways in utero, resulting in congenital syndromes that often include deafness, or infection may be acquired after birth. As the fluids of the inner ear are not easily accessible for sampling, the implication of viral pathogens as the causes of sensorineural hearing loss has often depended...

Human Immunodeficiency Virus

Infection with HIV is often heralded by presenting signs and symptoms involving systems of the head and neck. Hence, otolaryngologists must remain cognizant of this when evaluating patients whose findings may constitute those attributable to HIV infection, for which there is no definitive cure. HIV is discussed in greater detail in Chapter 16. Definition. HIV infection induces a disabling of T-cell immunocompetency, resulting in a constellation of findings associated with AIDS. Infection may be...

Stomatitis

Typical Location For Intraoral Herpes

Stomatitis refers to an inflammatory process involving the mucous membrane of the mouth that may manifest itself through a variety of signs and symptoms including erythema, vesiculation, bulla formation, desquamation, sloughing, ulceration, pseudomembrane formation, and associated discomfort. Stomatitis may arise due to factors that may be of either local, isolated conditions or of systemic origin. For example, a solitary oral ulcer with a history of a recurrent pattern may be classified as...

Lymphoma Involving Specific Head and Neck Sites

Lymphoma involving the thyroid gland is rare, accounting for only 2 to 3 of all cases of lymphoma and less than 10 of thyroid malignancies. Women are affected more frequently than men are by a ratio of 2.7 1. The median age at presentation is over 60 years. The most common subtypes are DLBCL and follicular Grade-3 lymphoma, accounting for at least 80 of cases. A rare but interesting subtype is marginal zone B-cell lymphoma, which arises from MALT. In many cases, this lymphoma...

Inflammatory Autoimmune

Pemphigus is a rare disease that affects mucosal membranes. Although the term pemphigus may be erroneously used interchangeably with the condition bullous pemphigoid, it is a separate entity warranting a separate discussion. Pemphigus is characterized by vesicular lesions and bulla. There are multiple subtypes, with pemphigus vulgaris and pemphigus foliaceus being the most common (7). Other subtypes include pemphigus vegetans, pemphigus erythematosus, pemphigus herpetiformis,...

Infectious Diseases Mycobacterial Infection

The incidence of mycobacterial infections has undergone a varied course over the past 50 years. For an approximate 30-year period following 1950, the incidence in mycobacterial infection declined annually in excess of 5 . This was followed by a paradoxical sharp increase at the rate of approximately 20 during the late 1980s and early 1990s, due to multiple factors such as acquired immunodeficiency syndrome (AIDS), drug resistance, and population influx from endemic areas. With improved...

Neuromuscular Disorders

Many neuromuscular diseases cause dysphagia in fact, the presenting symptom is often dysphagia. This is not surprising, considering the complex neuromuscular coordination required to execute a normal swallow. Although stroke patients develop sudden dysphagia, patients with other degenerative and nondegenerative neuromuscular diseases have a more insidious onset of dysphagia. Motor neuron diseases causing bulbar palsy or pseudobulbar palsy usually develop a progressive dysphagia and dysarthria...

Erythema Multiforme

EM is an acute, widely distributed hypersensitivity reaction associated with circulating immune complexes that are deposited in the basement membranes of the superficial vessels of the skin and mucosa. Subsequent complement activation produces vasculitis and thrombosis, leading to tissue ischemia and necrosis of the adjacent epithelium. The intensity of the skin and mucosal reaction varies from a localized minimal erythematous FIGURE 40 Erythema multiforme. Young adult male with rapid onset of...

Allergy and Dermatitis

There is clear evidence that otitis media with effusion is highly related to an allergic diathesis. When this converts to chronic draining otitis media, the allergic component would seem to still be relevant, although direct evidence is scant (17-19). Therefore, the surgeon must consider allergy evaluation, based on a patient history of other allergic diatheses, especially of the unified respiratory epithelium. Patients with chronic draining ear and allergic rhinitis, chronic rhinosinusitis,...

Angioedema

Angioedema is an uncommon and usually self-limited swelling of the deep dermis. It can, however, be complicated by potentially serious adverse consequences, including death. Although IgE-mediated mechanisms are not the major cause of angioedema, they are among the few that can be clearly identified, and thus this topic will be discussed here. Angioedema occurs together with urticaria approximately 85 of the time in 15 of instances, it occurs alone. Angioedema (Fig. 1) is the abrupt and...

Pemphigus And Pemphigoid

Pemphigus and pemphigoid are autoimmune diseases that produce blistering of skin and or mucosa. In pemphigus, intraepithelial blistering is caused by destruction of desmogleins that connect epithelial cells. Its most common variant, pemphigus vulgaris, begins with ulcerating lesions in the mouth, as the outer epithelium of the blister sloughs. Later, skin is also involved. In pemphigoid, the basement membrane is attacked, resulting in subepithelial blisters. Mucosal involvement nearly always...

Congenital Syphilis

Fetal infection with syphilis is highly dependent on the mother's stage of disease. Mothers with untreated primary or secondary disease are extremely likely to pass the infection, while those with latent disease have rates of transmission as low as 2 . Appropriate treatment of the mother during pregnancy generally prevents fetal infection. When infection does occur, the outcome can range from spontaneous abortion to a healthy-appearing child at birth with only latent infection. Similar to...

Crohns Disease and Ulcerative Colitis

Department of Medicine, Division of Gastroenterology, Inflammatory Bowel Disease Center, Mount Sinai School of Medicine, New York, New York, U.S.A. Clinical Manifestations 299 Crohn's disease CD and ulcerative colitis, collectively part of the inflammatory bowel diseases IBD , are both diseases of the gastrointestinal tract that arise in genetically susceptible individuals. Both CD and ulcerative colitis, though clinically different entities, are thought to be part of a spectrum of diseases...

Porphyrias

Porphyrias are a group of metabolic disorders characterized by defects in the synthesis of heme, a metalloporphyrin that is the product of porphyrin metabolism. Presentation of the disorder typically involves abdominal pain, vomiting, constipation, hypertension, tachycardia, photosensitivity, psychosis, and neuropathy. Neuropathy is estimated to affect 10 to 40 of patients and is primarily motor 126 . Cranial neuropathies have been described but facial paralysis is rare 127 . Although the...

Mucous Membrane Pemphigoid

Pemphigoid Bullous Mucosa

MMP is a chronic autoimmune mucocutaneous disease in which autoantibodies directed at structural proteins of the hemidesmosome destroy the epithelial-connective tissue attachment at the level of the basement membrane, producing a subepithelial separation 24,25 . The protein targets of the autoantibodies include BP-1, BP-2, and laminin-5 epiligrin , all components of the epithelial anchoring apparatus. MMP is a generalized term for a group of closely related disease processes 26 . The term oral...

Otic Diseases

Acute otitis media is a suppurative infection of the middle-ear cavity. Facial nerve involvement is considered a complication of the infection and often occurs from direct pathogen invasion of a dehiscent portion of the facial nerve course, most commonly in the horizontal segment of the facial nerve. Treatment consists of systemic antibiotic therapy against the most common pathogens Haemophilus influenzae, Moraxella catarrhalis, and Streptococcus pneumoniae. Drainage of the...

Migraine Syndrome

Migraine Aura Fortification

Department of Neurology, David Geffen School of Medicine, University of California, Los Angeles, California, U.S.A. Clinical Manifestations 336 Complications and Prognosis 340 Migraine is a syndrome characterized by periodic headaches. Often, however, patients experience other symptoms, including hearing loss, tinnitus, and dizziness and in some cases, these can be the only symptoms. Migraine is nearly always familial and occurs in complex patterns and settings. The association of migraine and...

Mycobacterial Infections

Tuberculosis TB, from Mycobacterium tuberculosis and or Mycobacterium bovis is a major cause of morbidity and mortality in HIV disease. It usually presents as reactivation of a pulmonary primary focus, with a risk of 7 to 10 per year for HIV-infected persons regardless of CD4 lymphocyte count, versus 10 per lifetime for HIV-negative persons. There can be involvement of the lungs, central nervous system CNS , or other organs, with rhinosinusitis, diffuse or localized scrofula...

Definitions

Fungal sinusitis, when used loosely, can be a misleading term. It actually refers to a spectrum of fungal-associated diseases of the nose and paranasal sinuses, each with a unique presentation and management implications Table 1 . When communicating with TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses is used to indicate controversy TABLE 1 Fungal-Associated Diseases of the Nose and Paranasal Sinuses is used to indicate controversy topical nasal steroids, antifungals...