Authors: Menachem Gross, Ron Eliashar
Categories: Article
Source: Orofacial Pain and Headache
Authors: Menachem Gross, Ron Eliashar
Orofacial pain is a relatively common complaint in both general medical and dental practice. Otolaryngologists are often involved when primary disorders of the ear, nose and throat, or head and neck are the source of pain. This chapter deals with the differential diagnosis of common painful disorders affecting the ears, sinonasal and facial area and the throat (see Table 6.1, Table 6.2, Table 6.3 ).Table 6.1Causes of Primary OtalgiaClassificationConditionComments for Entities Not Covered in This ChapterAuricularAuricular cellulitisdisordersAuricular traumaInjury to the ear may cause hyperaemia, abrasions, lacerations and auricular haematoma in cases of blunt trauma. The haematoma should be drained to relieve pain and to prevent abscess formation and auricular deformity such as ‘cauliflower ear’.Relapsing polychondritisDisorders of the external earImpacted cerumenWax may obstruct the ear canal and may cause pain, itching and temporary hearing loss. Wax is removed by irrigation or by rolling it with a blunt curette or loop.Foreign object in the external earInsertion of foreign bodies such as beads, erasers and beans, is often by children. Foreign body should be removed by raking it out with a blunt hook.Furuncle of the external ear canal Otitis externa Necrotizing otitis externa Ramsay Hunt syndrome (herpes zoster oticus)Disorders of the middle earOtitis mediaMyringitis bullosaTraumatic tympanic membrane ruptureThe tympanic membrane may be perforated by objects placed in the external canal, by sudden overpressure, such as an explosion, a slap or diving, or by sudden negative pressure. This results in sudden severe pain followed by bleeding from the ear. Spontaneous closure of the perforation is usual. Persistent perforation is an indication for myringoplasty.Eustachian tube dysfunction BarotraumaTable 6.2Causes of Secondary OtalgiaClassificationConditionCommentDental problemsInfant teethingSee Chapter 5Pain of eruptionImpacted third molars (wisdom teeth)Dental infections in the upper back teethFractured toothDry socket following dental extractionGingivitis and other periodontal diseaseInfectionsUpper respiratory tract infectionAn acute usually viral infection of the respiratory tract with inflammation in the nose, throat, larynx and trachea. It is associated with watery nasal secretions, sore throat, cough and referred otalgia through glossopharyngeal nerve.SinusitisSee section on facial pain in this chapterInfection of the tonsillitis, pharyngitis, peritonsillar abscessCovered under throat pain in this chapterLaryngitisAcute viral inflammation of the larynx is associated with unnatural change of voice, throat pain and referred otalgia via the vagus nerveSalivary gland infectionSee Chapter 5Diseases of the joints and muscles of the lower jawMasticatory muscles disordersSee Chapter 7Temporomandibular joint (TMJ) dysfunctionSee Chapter 8Head and neck muscle spasmsSee Chapter 7The removal of tonsilsPost-tonsillectomy painFollowing tonsillectomy patients have throat pain and may feel a referred pain in one or both ears via the glossopharyngeal nerve. This pain vanishes within a few weeks.Cervical spine problemsSee Chapter 13Inflammation of the blood vessels in the temporal arteritisSee Chapter 13Neuralgic disordersTrigeminal neuralgiaSee Chapter 11Glossopharyngeal neuralgiaArnold's nerve cough syndromeReflex cough, caused by chronic irritation of auricular branch of vagus (X) nerve. It is associated with attack of suboccipital stabbing or burning pain and auricular pain.Cancer of the head or neckSee Chapters 13 and 14chapter 13chapter 14Table 6.3Differential Diagnosis of Facial Pain: Otolaryngologist's ViewpointClassificationEntityComment for Entities Not Covered in This ChapterSinonasal painRhinosinusitisMucosal contact point headacheIntranasal tumourNasal obstruction and local nasal pain may occur as the intranasal tumour enlarges, especially if it invades nerves.Granulomatous diseases of the noseWegener's granulomatosis and sarcoidosis may affect the nasal cavity causing congestion, nasal secretions and local pain due to destructive lesions of soft tissue cartilage, and bone.Midfacial segment painAtypical facial pain(recently renamed as persistent idiopathic facial pain)See also Chapter 11Scuba diving causing painSinus painTension-type headache or muscle painCarbon dioxide toxicityDecompression sickness headacheNeuralgiasTrigeminalSee Chapter 11SphenopalatinePrimary headachesTension headacheSee Chapter 7MigraineSee Chapter 9Cluster headacheSee Chapter 10Chronic paroxysmal hemicraniaSee Chapter 10
Earache or otalgia is quite common among both children and adults. It can vary from mild pain to an excruciating severe, dull, aching, or lancinating pain. Otalgia may be associated with sensations such as a sense of fullness in the ear, burning, throbbing, tenderness or itching. The exact incidence of otalgia is not known. Adults tend to suffer from fewer ear problems and less otalgia than children. Children are mostly affected, since acute otitis media (which is the most common cause of otalgia) occurs mainly in young people (Murtagh 1991).
Otalgia may be caused by several different medical conditions. Detailed history and physical examination with directed studies as indicated can clarify the source of the pain. Based on the resultant findings, the disease is classified as primary or secondary (referred) otalgia (Table 6.1, Table 6.2).
Primary otalgia is due to a disease in the ear itself (a direct cause of earache) and the most serious problems are usually caused by infection. The areas most commonly involved in causing pain, or becoming infected, are the external ear and the middle ear. Pain in the external ear radiates most often to the vertex and to the temple, but may sometimes spread towards other areas of the head. Often patients cannot differentiate between pain originating in the inner ear and pain whose source is in the external or middle ear (Harvey 1992).
Secondary or referred otalgia (Table 6.2) is pain which stems from another region or location in the body and radiates to the ear (an indirect cause of earache). Because of the nature of the ear's sensory innervation a wide variety of disorders can produce referred otalgia. Branches of the trigeminal, facial, glossopharyngeal and vagus cranial nerves all participate, as well as the lesser occipital and the great auricular cervical nerve roots. The ear thus shares its sensory innervation with other head and neck structures, including the face, eyes, jaws, teeth, pharynx and larynx.
The incidence of referred otalgia increases with age. Up to 50% of cases suffering from otalgia are caused by referred pain from non-ear-related problems, whereas the other half are due to dental disorders (Yanagisawa and Kveton 1992). Diseased molars are the most common dental cause of secondary otalgia and cause severe unremitting pain which often worsens when cold fluids enter the oral cavity (see Chapter 5).
Muscular pain originating in the muscles, tendons or fascia of the head or neck such as myofascial pain and tension-type headache (Chapter 7) can also produce ear pain. The pain is constant, dull and aching and usually with no throbbing quality (Wazen 1989). Movement of the jaw or the head worsens the pain. Prolonged clenching the teeth, abnormal jaw movements and dental disease may cause a type of muscle spasm known as protective muscle splinting (Chapter 7).
Pharyngeal and laryngeal diseases cause referred otalgia via the glossopharyngeal nerve (Yanagisawa and Kveton 1992). Associated symptoms may include dysphagia, throat pain and breathing difficulty. Following recent tonsillectomy, patients virtually always complain of postoperative otalgia (Wazen 1989).
Persistent earache with a normal ear examination increases the suspicion of carcinoma, especially when associated with haemoptysis, weight loss, tooth pain or difficulty in swallowing (Thaller and DeSilva 1987). Otalgia is one of the earliest symptoms of carcinoma of the pyriform sinus, although neoplasia-producing otalgia may also be located in the larynx, oesophagus, nasopharynx, lungs, tonsils or tongue (Wazen 1989, Harvey 1992, Nestor 1994, Morgan 1995). Amundson (1990) stresses that unilateral ear pain in an adult who is a heavy smoker or a heavy drinker is probably caused by cancer until ruled out.
Auricular cellulitis is a diffuse, spreading, acute infection of the auricular skin or subcutaneous structures, characterized by hyperaemia and oedema with no cellular necrosis or suppuration. The most common pathogen is Streptococcus pyogenes (Group-A, β-hemolytic streptococcus). Staphylococcus aureus occasionally causes superficial auricular cellulitis, which is less extensive than that of streptococcal origin. The infective process involves the entire auricle including the auricular lobule. The infection can occur spontaneously or following acute external otitis, chronic auricular dermatitis or trauma (such as auricular piercing). Primary treatment includes parenteral antibiotic therapy such as oxacillin or cefazolin.
Relapsing polychondritis (RP) is an episodic inflammatory condition of the cartilaginous and non-cartilaginous tissues that causes progressive destruction of the head and neck cartilages, predominantly those of the ear, nose and laryngotracheobronchial tree (Gergely et al 2004). Other affected structures may include the eye, the cardiovascular system, small and large peripheral joints and the middle and inner ear (Lucente 1995, Staats 2002, Gergely 2004). The aetiology is unknown; however, the pathogenesis is most likely autoimmune (Gergely et al 2004). Signs and symptoms of RP of the auricle include auricular cellulitis, typically with a sudden onset of unilateral or bilateral auricular pain, tenderness, swelling and redness, with sparing of the lobules. The pain and redness usually disappear within 2–4 weeks but may recur. A definitive diagnosis of RP is made by biopsy of the affected cartilaginous tissue that demonstrates infiltration of the cartilage and perichondrial tissues with neutrophils and lymphocytes and loss of cartilaginous matrix. Mild cases may respond to symptomatic treatment with aspirin, indomethacin or other nonsteroidal anti-inflammatory drugs (NSAIDs). Severe cases are initially treated with systemic corticosteroids with tapering of the dose in accordance with the clinical response. In very severe cases addition of an immunosuppressive agent such as cyclophosphamide is required.
Furuncle of the external ear canal is an acute, tender, perifollicular inflammatory nodule resulting from infection by S. aureus. The initial nodule evolves into a pustule with central necrosis, which later discharges a sanguineous purulent exudate. A furuncle causes localized pain within the external auditory meatus, which is intensified by local pressure. Treatment includes surgical drainage of the furuncle, systemic antibiotic therapy with oxacillin or cefazolin.
Acute otitis externa (AOE) is an infective condition involving the external ear canal usually caused by a Gram-negative rod such as Pseudomonas aeruginosa or Escherichia coli; however, S. aureus or, rarely, a fungus may also be causative agents (Sander 2001). Often this infection is predisposed by moisture in the external ear canal such as in warm-moist climates or after bathing. Injury caused by attempts to clean or scratch an itching ear as in patients suffering from contact dermatitis (from earrings or earphones), allergic dermatitis or seborrheic dermatitis is another common cause (Hirsch 1992). A variant of AOE is ‘swimmer's ear’, a condition caused by a combination of external ear canal trauma and humidity during the swimming season (Agius et al 1992). Signs and symptoms of AOE include mild to severe otalgia associated with purulent discharge from the external ear canal. Auricular movement or pressure on the tragus intensifies the pain. The otalgia can become extreme as the canal swells and becomes blocked. Appropriate treatment includes topical application of a combination of antibiotic and corticosteroid eardrops, instilled directly into the ear canal. Irrigation of the ear canal with either hydrogen peroxide or 70% alcohol can temporarily stop the pain and itching.
Necrotizing external otitis (NEO) is an infection which starts as an external otitis caused by P. aeruginosa and progresses into an osteomyelitis of the temporal bone (Sander 2001). This disease commonly occurs in elderly diabetic patients and occasionally in immunocompromised patients (Handzel and Halperin 2003). The disease spreads outside the external ear canal through the fissures of Santorini which are two slits located at the anterior cartilaginous external canal wall and the osseocartilaginous junction into the skull base. It is characterized by persistent severe otalgia, purulent otorrhea and granulation tissue in the external ear canal (Handzel and Halperin 2003). Pre-auricular and temporomandibular joint pain intensified by opening the mouth or by chewing may be present. Severe headache in the temporal or occipital areas may accompany the earache. The severity of the pain can give a clue to the diagnosis. In severe cases, facial nerve involvement may occur, indicating an invasive infection. Other cranial nerve palsies, such as IX, X, XI and XII, may also occur. Imaging evaluation by computed tomography (CT) usually demonstrates soft tissue infiltration and bone destruction (Guy et al 1991). Technetium^99^ bone-scan is usually positive and is highly sensitive to NEO. Treatment includes good control of the diabetes and prolonged therapy with an anti-pseudomonal antibiotic such as ciprofloxacin for at least 6 weeks in addition to local therapy for the otitis externa. Surgical debridement through a mastoidectomy approach is occasionally required to control the spread of the infection.
Ramsay Hunt Syndrome (RHS), also called herpes zoster oticus, is a herpes virus-3 infection (varicella-zoster virus infection which causes chickenpox and shingles) of the geniculate ganglion (facial nerve ganglion) (Kuhweide et al 2002). The virus spreads from the geniculate ganglion to the facial nerve and to the adjacent vestibulocochlear (XIII) nerve (Kuhweide et al 2002). The syndrome consists of unilateral facial paralysis, severe ear pain on the same side as the infection, tinnitus, vertigo and herpetic blistering rash or vesicles on the pinna, external canal and sometimes the roof of the mouth, in the distribution of the sensory branches of the facial nerve. Other cranial nerves may be involved and some degree of meningeal inflammation may occur (Ko et al 2000). RHS is usually more painful than Bell's palsy. It is rare in healthy people and occurs more commonly in people with a weakened immune system, such as the elderly (Burke et al 1982). Diagnosis of RHS is based on the clinical symptoms and signs. Prompt treatment with corticosteroids and antiviral medications such as acyclovir and famciclovir reduces the symptoms and improves recovery (Morrow 2000, Kinishi 2001). Overall, chances of recovery are better if treatment is started within 3 days of the onset of symptoms. Certain degrees of hearing loss or facial paralysis may become permanent (Morrow 2000). Recovery may be complicated if the nerve grows back to the wrong areas (synkinesis); this may cause inappropriate responses, such as tearing when laughing or chewing (crocodile's tears). Some may start blinking when talking or eating.
Acute otitis media (AOM) is an infection of the middle ear cavity. AOM occurs most frequently in infants and children, particularly between the ages of 3 months and 3 years, although it can occur at any age. AOM usually follows or accompanies an upper respiratory infection (URI) or a reduced immune response with no special reason. AOM is the second most common childhood disease after URI. The first complaint is usually persistent, severe otalgia, which is often caused by an accumulation of fluid and pressure behind the tympanic membrane (TM). Other symptoms include hearing loss, fever, chills, irritability and a feeling of fullness and pressure in the affected ear. Spontaneous drainage of pus or a clear or bloody fluid from the middle ear is common, and may indicate perforation of the TM. An otoscopic examination reveals abnormal findings of the TM, including an erythematous and bulging TM, middle ear effusion, non-distinct anatomical middle ear landmarks, displaced or absent TM light reflex and decreased TM mobility on pneumatic otoscopy. The most common bacterial pathogen in AOM is Streptococcus pneumoniae, followed by Haemophilus influenzae and Moraxella catarrhalis. These three organisms are responsible for more than 95% of all AOM cases with a bacterial aetiology. The most important factors in the pathogenesis of a middle ear infection are eustachian tube dysfunction and direct extension of infectious processes from the nasopharynx into the middle ear cleft. AOM is treated with painkillers and systemic antibiotics such as oral amoxicillin. Topical analgesics such as tetracaine applied by eardrops and nasal decongestants such as xylometazoline can be helpful. Antibiotic therapy relieves the symptoms, hastens resolution of the infection and reduces the chance for developing complications (such as mastoiditis or meningitis). Myringotomy (small incision of the TM) should be considered in cases of a bulging TM or a persistent earache.
Myringitis bullosa (MB) is an inflammatory infection of the TM caused by a viral or a bacterial infection. Mycoplasma pneumoniae and S. pneumoniae are the most common bacteria responsible for MB. The disease is characterized by very painful vesicles on the tympanic membrane that may be full with a serous or haemorrhagic fluid. An upper respiratory tract infection can precede the ear manifestations. Pain is severe, starts suddenly, persists for 24–72 hours and is due to the TM blisters, which appear between the richly innervated outer epithelium and the middle fibrous layers of the TM (Woo 1992, Marais 1997). Bullae involving the TM may also extend towards portions of the external auditory canal immediately adjacent to the TM. Objective cochlear and vestibular involvement manifested by sensorineural or mixed-type hearing loss and vertigo have been reported in patients suffering from MB (Eliashar et al 2004). Treatment includes analgetic and antibiotic therapy such as azithromycin which is efficacious against the major known pathogens causing MB.
The middle ear is connected to the nasopharynx by the eustachian tube (ET). The ET enables normal fluids to drain from the middle ear, and has an important role in equalizing the pressure in the middle ear when atmospheric pressure of ambient air shifts. Acute obstruction of the ET, mainly in cases of URI, may cause otalgia. This is especially common in small children, whose ET is naturally shorter and more horizontal. ET obstruction also occurs when there is an increase of postnasal drainage (PND) or allergy (Derebery 1997, Grimmer 2005). As the PND drains posteriorly into the nasopharynx around the opening of the ET, it gets irritated and swollen and eventually becomes blocked on one or both sides. When this happens, surrounding tissue absorbs the air in the affected tube, creating a vacuum, which causes the differences in pressure to pull the tympanic membrane inward, the result being a sensation of fullness and pain. Hearing can be slightly impaired and the ears can feel blocked or stuffed. To improve ET function, topical vasoconstrictors such as xylometazoline may be instilled into each nasal cavity.
Barotitis media (BM), also known as ‘aerotitis’, represents damage to the middle ear and tympanic membrane due to ambient pressure changes. When a sudden increase in the ambient pressure occurs, air must move from the nasopharynx into the middle ear in order to maintain equilibrium on both sides of the TM. In cases of eustachian tube dysfunction, the pressure in the middle ear is below the ambient pressure, and the relative negative pressure in the middle ear results in retraction of the TM and transudation of blood from vessels. Very severe and sudden pressure differences may rupture the TM, causing bleeding in the middle ear with severe earache and conductive hearing loss. A perilymphatic fistula through the oval or round windows can occur, causing sensorineural hearing loss and vertigo. Barotrauma commonly occurs with altitude changes, such as in descent of an airplane, deep sea diving, scuba diving or driving in the mountains (Mirza and Richardson 2005). Otalgia is the most common complaint of scuba divers and is experienced at some point by almost every diver (Clenney and Lassen 1996). Some divers call it the ‘ear squeeze’. A person with an acute URI or allergic rhinitis should be advised to abstain from diving. Topical application of a nasal vasoconstrictor such as xylometazoline before any activity associated with descent and pressure changes can prevent barotrauma.
Facial and sinus pain complaints are commonly encountered by physicians. Facial pain is usually referred to otolaryngology, dental, and neurologic clinics, and often needs a multidisciplinary evaluation. Facial pain is located in the area below the eyebrows or the supraorbital rims, extending along the course of one or more trigeminal dermatomes. Headaches are located in the forehead and temporal regions. Facial pain may be due to either local disease of any one of the major facial structures or a condition affecting their innervation. The latter can occur anywhere between the posterior cranial fossa and the distal ends of the trigeminal nerve. The prevalence of facial pain of paranasal sinus origin has probably been overestimated. Patients' expectations are often tempered by their own prior diagnosis of ‘sinusitis’ guided by their knowledge of the location of the sinuses, or by their primary-care physician. However, many of these patients are found to have no evidence of sinonasal disease and there is an increasing awareness among general physicians and otolaryngologists that neurological causes are responsible for most of the suffering experienced by patients with facial pain or headache. The ‘gold standard’ in establishing a sinonasal aetiology is a diagnosis made after a personal history, a careful physical examination, imaging studies, response to medical and surgical treatment and an extended follow-up period. Patients who undergo normal nasal endoscopy or CT scan are often found to suffer from a range of neurological causes (West and Jones 2001). When the ostia of the sinuses are found to be patent and the mucosa healthy, the probability that a sinonasal disease or an anatomic variation is the cause of pain is eliminated (West and Jones 2001).
The trigeminal nerve is the main facial sensory supply. The ophthalmic (V1) and maxillary (V2) divisions of the trigeminal nerve provide sensation to the mucosa of the sinonasal cavity. These nerve branches terminate as extensive uncovered nerve terminal endings next to the basal cells of the nasal epithelium (Baraniuk 2001). Nasal pain is mediated by Aδ fibres—the fast-responding, primarily mechanoreceptive pain fibres—and by C fibres—the slower, unmyelinated fibres associated with a duller pain from mechanothermal and chemosensory stimulation (Baraniuk 2001). Recent theories of sinonasal pain from ‘contact points’ or pressure from sinus inflammation are based upon substance P (SP) release from trigeminal sensory neurons located in the nasal mucosa (Stammberger 1988, Chow 1994, Clerico 1995, Clerico 1997). This may be accompanied by the release of various other neuropeptides such as calcitonin gene-related peptide (CGRP) and vasoactive intestinal peptide (VIP), which appear to be involved in the inflammatory cascade (Baraniuk 2001). The local stimulation of the trigeminal fibres leads to both a painful orthodromic and an antidromic response, typified by vasodilatation and hypersecretion (Stammberger 1988, Baraniuk 2001). Despite this frequently cited mechanism for contact point sinonasal pain, it is poorly documented and highly controversial (Abu-Bakra 2001a, Abu-Bakra 2001b, Jones 2004).
Rhinosinusitis is a group of disorders characterized by inflammation of the mucosa of the nose and paranasal sinuses. The term ‘rhinosinusitis’ is used instead of ‘sinusitis’ due to the fact that sinusitis is almost always accompanied by concurrent nasal airway inflammation, and in many cases, sinusitis is preceded by rhinitis. Rhinosinusitis is a leading healthcare problem which may affect up to 14% of the adult population and costs more than $2 billion in direct medical costs (Anand et al 1997). Rhinosinusitis is increasing in prevalence and incidence and has been estimated to affect approximately 31 million patients in the United States each year (International Rhinosinusitis Advisory Board 1997).
A common presenting symptom of acute rhinosinusitis is facial pain or headache. The pain is usually accompanied by other symptoms such as nasal congestion, anterior and posterior purulent nasal drainage and hyposmia or anosmia. The pain is a subjective complaint; however, tenderness on percussion is a function of spinal cord pain processing (hyperalgesia). People with acute rhinosinusitis have a significantly lower pain and sensory detection threshold in their sinus regions when compared to a healthy control group (Meltzer 2004, Benoliel 2006). Sinus pain caused by inflammation induced by infection (bacterial or viral) or allergic rhinosinusitis occurs when exudate blocks the sinus ostium and exerts pressure which stimulates local trigeminal nerve fibres. The local release of proinflammatory and proalgesic mediators is an early mechanism (this is probably as important as the pressure).
The development of rhinosinusitis depends on a variety of environmental and host factors and is considered to be a disease with multifactorial causes. Host factors include genetic or congenital conditions (e.g. cystic fibrosis, immotile cilia syndrome), allergic rhinitis, sinonasal anatomic abnormalities and systemic diseases. Environmental factors include infectious agents, trauma, noxious chemicals and iatrogenic causes.
There are two principal systems of classification and diagnostic criteria relating headaches and sinus the working definitions for acute rhinosinusitis (ARS), subacute rhinosinusitis (SRS) and chronic rhinosinusitis (CRS) recommended by the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS), and the International Headache Society (IHS) criteria (Lanza 1997, Benninger 2003, Meltzer 2004, Olesen 2004). As expected the IHS classification relates to diseases that may induce facial pain or headache whilst the AAO-HNS classification is concerned primarily with the disease process itself.
The different types of rhinosinusitis can be diagnosed clinically in most patients based on the history and physical examination. Physical examination includes otoscopy, anterior rhinoscopy, percussion over the areas of the paranasal sinuses and oropharyngeal and neck examination. Nasal endoscopy and imaging evaluation are not required for an initial diagnosis of any form of rhinosinusitis. However, these modalities may be very helpful in definitive diagnosis of rhinosinusitis. Patients with recurrent or complicated sinus disease may require imaging. Computerized tomography is superior to radiography because the latter are imprecise at determining the extent of the disease and the patency of the sinus ostium (Lanza 1997, Sinus and Allergy Health Partnership 2004). CT scanning of the sinonasal region has two major roles in 1To define the anatomy of the sinuses before surgery; and2To aid in the diagnosis and management of CRS or recurrent rhinosinusitis.
Acute rhinosinusitis is an inflammatory condition involving the paranasal sinuses and the lining of the nasal passages, where the symptoms and signs last up to 4 weeks (Lanza and Kennedy 1997) (see Table 6.4, Table 6.5). The symptoms resolve completely, and after the disease has been treated, antibiotics are no longer required.
The most common cause of ARS is a community-acquired viral infection leading to a self-limiting period of upper respiratory symptoms, such as nasal discharge, nasal congestion and coughing (Meltzer et al 2004). Human rhinovirus is the most common cause of viral ARS. Other viruses include coronavirus, influenza A and B virus, parainfluenza virus, respiratory syncytial virus and adenovirus. Occasionally, a secondary bacterial infection of the paranasal sinuses occurs and requires specific antimicrobial therapy (Meltzer et al 2004). Allergic rhinitis, nasal polyposis, foreign body in the nasal cavity, trauma, dental infection and other factors leading to inflammation of the nose and paranasal sinuses can also predispose individuals to develop ARS.
Commonly isolated bacteria in patients with ARS include S. pneumonia, non-typeable H. influenzae and M. catarrhalis (Sinus and Allergy Health Partnership 2004). Streptococcus pneumonia and non-typeable H. influenzae account for more than 75% of bacterial isolates (Meltzer et al 2004). Other streptococcal species, anaerobic bacteria and S. aureus are responsible for a small percentage of cases. Nosocomial rhinosinusitis often occurs in patients who require extended periods of intensive care, which involve prolonged nasotracheal intubation or a nasogastric tube. Isolates from hospitalized patients usually contain Gram-negative enterics such as P. aeruginosa, Klebsiella pneumoniae, Enterobacter spp., Proteus mirabilis, Serratia marcescens, and coagulase-negative S. aureus (Meltzer et al 2004).
The diagnosis of ARS focuses on clinical history and physical examination with radiographic diagnosis playing a supportive role (Sinus and Allergy Health Partnership 2004). The clinical diagnostic criteria for ARS as defined by the Task Force on Rhinosinusitis of the AAO-HNS include major and minor symptoms or signs, with a disease duration of less than 4 weeks (Table 6.4 ). According to this classification, facial pain or pressure is regarded as a major symptom, whereas headache is considered to be a minor symptom (Lanza 1997, Meltzer 2004). This classification was designed to be used by both primary care physicians and specialists. A diagnosis of rhinosinusitis is possible if two or more major symptoms or one major symptom and two or more minor symptoms are present. However, nasal purulence is a strong indicator of an accurate diagnosis. Facial pain or pressure alone does not constitute a suggestive history in the absence of another major nasal symptom or sign. Fever in itself does not constitute a strongly suggestive history in the absence of another major nasal symptom or sign. The IHS diagnostic criteria for sinusitis-related headache reiterate the requirement of clinical findings of acute sinusitis, along with a reversible ‘sinus headache’; see Table 6.5 (Olesen et al 2004).Table 6.4Clinical Criteria for the Diagnosis of Acute Rhinosinusitis (ARS)CriteriaCommentsMajor Purulent anterior nasal dischargePurulent-discoloured posterior nasal drainageNasal obstruction or blockageFacial pain/pressure/congestion/fullnessHyposmia or anosmiaFeverA diagnosis of rhinosinusitis is possible (a) 2 or more major symptoms or(b) 1 major symptom and 2 or more minor symptoms are present.Minor HeadacheEar pain/pressure/fullnessHalitosisDental painCoughFever (all non-acute)FatigueNasal purulence in itself is a strong indicator of an accurate diagnosis.Headache/facial pain or pressure as sole symptoms is not indicative of ARS.Similarly fever as a sole symptom is not indicative of ARS.Table 6.5The IHS Criteria for the Diagnosis of Headache/Facial Pain due to RhinosinusitisDiagnostic CriteriaCommentAFrontal headache accompanied by pain in one or more regions of the face, ears or teeth and fulfilling criteria C and DBClinical, nasal endoscopy, computerized tomography and/or magnetic resonance imaging, and/or laboratory evidence of acute or acute-on-chronic rhinosinusitisClinical evidence includes purulent nasal discharge, nasal obstruction, hyposmia or anosmia and/or feverCHeadache and facial pain develop simultaneously with onset or acute exacerbation of rhinosinusitisDHeadache and/or facial pain resolve within 7 days after remission or successful treatment of acute or acute-on-chronic rhinosinusitisChronic sinusitis is not validated as a cause of headache or facial pain unless there is re-acutization.
ARS has four basic clinical resolution, development of adverse sequelae, development of a symptomatic CRS and development of a silent CRS.
In the immunocompetent person living in the general community, ARS is typically believed to be induced by viruses and does not require antibiotics for the first 10–14 days. Symptomatic relief may be obtained by the use of topical application of a nasal decongestant such as xylometazoline and oral acetaminophen. The presence of severe headache or facial pain, high fever, and brain, eye or lung complications indicate secondary bacterial infection and antibiotics are indicated (Meltzer et al 2004).
In cases where no complications occur but the symptoms and signs persist for more than 10–14 days, bacteria are presumed to predominate and the patient can benefit from initiating antibiotic therapy as described below (Meltzer et al 2004).
Expert recommendation is divided about the appropriate therapy for ARS and ranges from symptomatic treatment alone (Snow et al 2001) to a prolonged course of antibiotic therapy effective against β-lactamase-producing organisms (Winther and Gwaltney 1990). Primary care and specialty physicians demonstrate variability in treatment approaches (Piccirillo et al 2001). No benefit for antibiotic therapy has been demonstrated for the treatment of ARS diagnosed by clinical criteria alone (Stalman et al 1997) and one study showed no overall benefit for seven days of penicillin V compared to placebo (Hansen et al 2000). However, there are indications that early antibiotics are of benefit for patients with the most severe maxillofacial pain symptoms at baseline (Williams et al 2003). Success rates for amoxicillin, trimethoprim-sulfamethoxazole or erythromycin compared to newer, broader spectrum antibiotics are similar (Piccirillo et al 2001). Therefore, current data do not support the use of newer, more expensive, non-penicillin antibiotics for first-line empiric therapy (Williams et al 2003). Among the newer, wider-spectrum antibiotics efficacy was similar but amoxicillin-clavulanate had significantly more adverse effects than cephalosporins (Williams et al 2003). Therefore, differences in adverse effects and costs should be considered when choosing a second-line antimicrobial therapy. Based on a meta-analysis, ARS should be treated with amoxicillin 500 mg three times daily for 10 days (Williams et al 2003). For penicillin-allergic patients, several cephalosporins and macrolides have been shown to be equivalent to amoxicillin (Williams et al 2003). Based on limited data, there is no convincing evidence that adjuvant treatment such as topical nasal decongestants or nasal corticosteroids improve clinical outcomes.
Acute maxillary rhinosinusitis is the most common affliction of all paranasal sinuses and the most common sinus disease causing facial pain (Fig. 6.1 ). The pain is usually related to the affected maxillary antrum, but is often referred to the upper teeth (whose roots are intimately related to the floor of the maxillary sinus, see Fig. 6.3 ) or to the forehead. Purulent nasal discharge in the middle nasal meatus and sensitivity to percussion over the cheek or teeth confirm the diagnosis.Fig. 6.1• Plain film demonstrating left acute maxillary rhinosinusitis with air-fluid level in the left maxillary sinus (arrow).Fig. 6.3• Coronal CT scan demonstrating right chronic maxillary rhinosinusitis with complete opacification of the right maxillary sinus and obstruction of the sinus ostium (arrow).
Diseases in the maxillary sinus mucosa may refer pain to the upper teeth. The pain is usually felt in several teeth as dull, aching or throbbing. Occasionally, the pain is associated with pressure below the eyes. It increases when bending the head, applying pressure over the sinuses, coughing or sneezing. Tests performed on the teeth, such as applying ice, chewing and percussion, may increase pain from a sinonasal origin. A history of URI, nasal congestion or other sinus problem is suspicious of a sinus toothache. Thorough dental examination (clinical and radiographic) excludes a primary dental cause.
Acute ethmoiditis causes pain at the root of the nose or behind the eye. Seldom does ethmoiditis occur as an isolated infection. More often it is part of an acute pansinusitis involving the maxillary and frontal sinuses as well. Purulent anterior and posterior nasal discharge and tenderness over the inner canthus of the eye are characteristic. The pain may spread laterally into the orbit or radiate to the temporal region. Occasionally, an orbital complication (such as periorbital cellulitis or abscess) may occur due to spreading of the infection into the orbit through the thin lamina papyracea.
Acute suppurative frontal rhinosinusitis is not very common, apparently because of the vertical nature of the frontal sinus and its natural advantage of a dependent drainage through the nasofrontal duct (Fig. 6.2 ). Thus, the common forehead pain is seldom due to an underlying frontal rhinosinusitis.Fig. 6.2• Coronal CT scan demonstrating right acute frontal rhinosinusitis with opacification of the right frontal sinus (arrow).
The characteristic pain is over the affected sinus and often along the upper orbital rim. The pain may radiate to the vertex and behind the eye. Tenderness is usually felt in the frontal sinus or along its floor. When frontal sinusitis is complicated by osteomyelitis of the frontal bone, the pain is prominent, diffuse and intense, often worsening at night keeping the patient awake.
Acute sphenoiditis is rare and is characterized by a wide variety of types and distributions of pain. These include severe occipital headache, retro-orbital dull and aching pain and a stabbing pain at the vertex. Definitive diagnosis is by CT scan.
Subacute rhinosinusitis represents a continuum of the natural progression of ARS which has not resolved. SRS is diagnosed after 4 weeks duration of symptoms or signs of rhinosinusitis, and lasts up to 12 weeks (Lanza and Kennedy 1997).
Chronic rhinosinusitis is divided into two major categories, including CRS with nasal polyposis (CRSwNP) and CRS with no nasal polyposis. The clinical diagnostic criteria suggested by the AAO-HNS for CRS include major and minor symptoms or signs, with disease duration of 12 weeks or more; see Table 6.6 (Lanza 1997, Meltzer 2004).Table 6.6Clinical Criteria for the Diagnosis of Chronic Rhinosinusitis (CRS)CriteriaCommentsMajor factorsNasal obstructionFacial congestionFacial pain/pressure/fullnessNasal discharge (anterior /posterior purulent discharge)Loss of smellSymptoms of CRS are milder than in the acute form.CRS may present with only one symptom such as postnasal drip, headache/facial pain or nasal obstruction.Minor factorsFatigueHeadacheEar pain/pressureCoughHalitosisDental painFeverNote, however, that according to the IHS CRS has not been validated as a cause of headache/facial pain.
Symptoms of CRS are generally the same as those of ARS. However, they are usually milder and include only one symptom such as postnasal drip, headache, nasal obstruction or facial pain. CRS has not been defined as a cause of headache or facial pain according to the IHS diagnostic criteria, unless relapsing into an acute stage (Olesen et al 2004). The most recent otolaryngologic literature regarding CRS includes ‘facial pain and pressure’ as a major factor in CRS, with ‘headache’ as a minor factor, but suggests that facial pain and pressure or headache alone are not suggestive of CRS in the absence of other major nasal symptoms or signs (Lanza 2004). Other causes of headache or facial pain should be considered in the differential diagnosis of such cases.
Bacterial infection as a causative factor in patients with CRS is controversial, but is regarded as a relatively important factor. A comparatively new hypothesis regarding the pathogenesis of CRS is related to colonization of the sinonasal mucosa with microorganisms such as superantigen-producing S. aureus, colonizing fungi, or biofilms, and the host response to their presence (Meltzer et al 2004). A recent study has shown increased sensory thresholds in the skin over sinuses diagnosed with CRS, suggesting some form of peripheral nerve damage (Benoliel et al 2006).
CRS has four basic clinical resolution, persistence, development of adverse sequelae and progression to generalized airway reactivity.
CRS is predominantly a medical condition where surgery can relieve symptoms and sometimes bring about a reversal in the course of the disease.
The most characteristic symptom of this condition is persistent purulent rhinorrhea with localization of pus in the middle meatus (Fig. 6.3). CRS in the maxillary sinus seldom gives rise to facial pain or headache, except during an episode of acute exacerbation or with progression towards maxillary pyocele or maxillary osteomyelitis.
Pain is seldom a symptom of CRS of the frontal sinus. This condition is usually part of chronic pansinusitis which involves the ethmoid and maxillary sinuses, the most common symptom being chronic purulent nasal discharge.
Stammberger and Wolf (1988) postulated that variations in the anatomy of the nasal cavity result in mucus stasis, infection and ultimately facial pain (Stammberger and Wolf 1988). They also stated that mucosal contact points could result in the release of substance P, a recognized neuropeptide and transmitter in nociceptive fibres. Other authors have adopted this concept to explain how pain might be induced by anatomical variants such as a concha bullosa (a pneumatized middle turbinate) or a pneumatized superior turbinate touching the septum (Morgenstein 1980, Blaugrund 1989, Goldsmith 1993, Clerico 1994, Clerico 1996). According to the IHS, the assumptions related to certain otolaryngologic conditions often considered to induce headaches have not been sufficiently validated. These include deviation of the nasal septum, hypertrophy of turbinates and atrophy of sinus membranes. However, mucosal contact point pain is defined in the IHS appendix; see Table 6.7 (Olesen et al 2004).Table 6.7Proposed IHS Diagnostic Criteria for Mucosal Contact Point HeadacheCriteriaCommentsAIntermittent pain localized to the periorbital and medial canthal or temporozygomatic regions and fulfilling criteria C and D.This is a new addition to the IHS appendix and is not completely validated.BClinical, endoscopic and/or CT imaging evidence of mucosal contact points without acute rhinosinusitis.CEvidence that the pain can be attributed to mucosal contact based on at least one of the 1The pain corresponds to gravitational changes in mucosal congestion as the patient moves between upright and recumbent postures.2Abolition of pain within 5 minutes after diagnostic topical application of local anaesthesia to the middle turbinate using placebo or other controls.Abolition as defined in criteria C2 means complete pain relief with a visual analogue score of 0.DPain resolves within 7 days, and does not recur, after surgical removal of mucosal contact points.
Midfacial segment pain (MSP) may have all the same pain characteristics as tension headache, but affects the face and may involve the nasion, under the bridge of the nose, either side of the nose, the periorbital region, the retro-orbital region or across the cheeks (Jones 2004). The forehead is often affected as well. It is described as a dull ache, a feeling of pressure or tightness. Some patients may feel that their nose is blocked although they have no nasal airway obstruction. The forehead and occipital region are affected simultaneously in approximately 60% of patients (Jones 2004). There are no consistent exacerbating or relieving factors. The pain can be chronic or episodic, and the skin and soft tissues over the forehead or cheek may be sensitive. Nasal endoscopy and CT scan of the paranasal sinuses are normal. MSP is the commonest cause of non-rhinologic facial pain seen in otolaryngological practice.
The aetiology of MSP is uncertain and may be myofascial or neurovascular in origin. However, Olesen's theory, which integrates the effects of myofascial afferents, the activation of peripheral nociceptors and their convergence on the caudal nucleus of the trigeminal nerve, along with qualitative changes in the central nervous system, provides one of the best models (Olesen 1991, Jensen 2000). The treatment of choice in this condition is amitriptyline for a period of 6 months (Jones 2004).
Atypical facial pain (AFP) is characterized by deep, achy, ill-defined, pulling or crushing pain, involving diffuse areas of the face in the territory of the trigeminal nerve. This term has recently been changed by the IHS to persistent idiopathic facial pain; see also Chapter 11. The pain fluctuates in intensity and severity. Occasionally, patients use terms such as ‘sharp’ or ‘knifelike’ to describe the pain. The pain often worsens at night, and can be aggravated by activity. Sometimes pain is continuous. In the majority of cases, only one side of the face is affected, but pain on both sides is also possible. The pain may move from one part of the face to another, and may be accompanied by complaints such as ‘mucus moving’ in the sinuses (Jones 2004). Interestingly, the pain often crosses the recognized neurological dermatomes. AFP is more common in women over the age of 40.
In some AFP cases the symptoms may initially appear similar to trigeminal neuralgia, but they progress towards an AFP pattern. Whereas trigeminal neuralgia is characterized by quick episodes of jabbing or lancinating pain, AFP attacks always last longer than a few seconds, usually minutes or hours (see Chapters 11 and 13chapter 11chapter 13). Moreover, no trigger points on the face are detected in AFP. Many patients suffering from AFP show a significant psychological disturbance or a history of depression, and are unable to function normally as a result of the pains (Jones 2004). AFP usually has no specific cause. However, injury of any peripheral or proximal branch of the trigeminal nerve due to facial trauma or skull base fracture can produce this disorder. This possibility is extensively discussed in Chapter 13.
Medical treatment of AFP is often difficult and less satisfactory than treatment of trigeminal neuralgia. The treatment of choice is amitriptyline for a period of 6 months (see Chapters 11 and 16chapter 13chapter 17). Conventional analgesic drugs, including opioids, can also be effective with selected individuals, often accompanied by a comprehensive pain management programme.
Diving pain has spoiled the diving vacation of many scuba divers. Several causes have been associated with pain and diving.
Sinus barotraumas, also known as ‘sinus squeeze’, is due to failure to equalize pressure during descent. Pressurized air cannot be forced into the sinuses. The air within the sinus contracts and causes the walls of the sinus to bleed. This is accompanied by intense, sharp pain. Common causes of sinus barotraumas include allergic rhinitis, vasomotor rhinitis, nasal deformity, nasal polyposis, acute URI and chronic irritation (such as smoking, diesel fumes, chemicals and prolonged use of decongestant nasal drops). Symptoms include pain in the area of the affected sinus or in the upper teeth (when the maxillary sinus is involved) and nose bleeding. The frontal sinuses are the most frequently affected, because the nasofrontal duct draining the frontal sinus is longer and more tortuous. A ‘reverse squeeze’ occurs during ascent, when air in the sinus expands without any ability to escape and is extremely painful. The situation is self-limiting, because air is gradually absorbed into the tissues lining the sinuses. When a sinus squeeze occurs, a slow ascent to the surface that generally alleviates most of the pain is recommended. Because the sinus may be filled with blood, the patient is at high risk of developing consequent sinusitis. Antibiotic therapy along with oral and nasal topical decongestants to promote drainage is recommended. Persistence of severe pain with no signs of acute rhinosinusitis may be treated by a short course of steroids. Diving is not recommended for patients with URI.
Symptoms of a tension-type headache include headache and pain in the nape (see Chapter 7). Tension headaches may be caused by muscle strain due to anxiety and muscular rigidity. To prevent the development of muscle strain, and consequently a tension headache, divers must learn to adopt a proper posture and relax in the water.
A dull throbbing headache after diving is usually caused by carbon dioxide toxicity. This type of pain is common in divers and is caused by an accumulation of carbon dioxide in the body. Hypoventilation usually happens when a scuba diver does not inhale long deep breaths from the air tank, or not often enough. The best therapy for this type of headache is slow, deep breaths in order to reduce the carbon dioxide retention. Carbon dioxide-induced headaches do not respond well to pain relievers.
Headaches can be a symptom of decompression sickness (DCS). DCS is caused by the formation of bubbles when dissolved nitrogen is discharged from the tissues on ascent. Associated symptoms include joint pain and swelling, skin rash, itching, dizziness, nausea, vomiting, tinnitus (ringing inside the ears) and extreme exhaustion. A scuba diver is at a higher risk of DCS when he does not decompress after a long or deep dive, before surfacing, or when he ascends too quickly or makes a panic ascent.
Sphenopalatine neuralgia (SPN) also known as ‘Sluder's neuralgia’ is characterized by a unilateral headache behind the eyes with pain in the upper jaw or soft palate (Sluder 1910, Sluder 1913, Ahamed 2003). Pain in the back of the nose, teeth, temple, occiput or neck may also occur. The pain is associated with sinonasal congestion, swelling of the nasal mucous membranes, tearing and redness of the face (Ahamed and Jones 2003). SPN is more common in women, with the ratio being 1. SPN should be distinguished from a cluster headache, although both are characterized by similar symptoms. However, SPN pain lasts longer and is associated with inflamed nasal mucosa on the involved side. SPN is evidently caused by an irritation of the sphenopalatine ganglion from intranasal infection, deformity or scarring. A topical decongestant may occasionally alleviate the nasal symptoms. Ganglion blocks (either by intranasal application or by direct injection) are effective for controlling the pain (Puig 1998, Day 1999).
The IHS classification system lists operational diagnostic criteria for migraine as shown in Chapter 9 (Olesen et al 2004). Migraine is a common cause of headache or facial pain. Despite the high prevalence of migraine, over 50% of migraineurs remain undiagnosed by their physicians (Lipton et al 2001). Recent publications have stated that ‘sinus’ headache patients, at least those with no obvious signs of infection, have a 58–88% incidence of a migraine-type headache (Perry 2004, Schreiber 2004). Migraine can cause similar symptoms to rhinosinusitis, such as facial pain, nasal congestion and rhinorrhea. This form of migraine has been termed ‘facial migraine’ and is discussed further in Chapter 9.
A certain proportion of the patients attending an ENT clinic suffer from some facial pain resulting from migraine. In some clinical studies, 90% of self- or physician-diagnosed sinus headaches met the criteria of IHS migraine-type headache and were treated satisfactorily by anti-migraine treatments (Cady and Schreiber 2004). A recent prospective evaluation of 100 sequential patients who complained of sinus headaches demonstrated that 86% of patients had migraine or ‘probable migraine’, and only 3% had actual ARS-related headaches (Mudgil et al 2002).
The reason for the high rate of sinus headache patients misdiagnosed by their physicians is related to the pathophysiology of migraine and to the nature of nasal pain. The neurovascular theory of migraine implicates the nervous system as being the initiator of the process, and the blood vessel involvement occurring as a consequence of the neuronal process. This migraine model identifies the starting point in the central nervous system followed by a sensitization of the peripheral neurons of the trigeminal nerve, including those which supply sensation to the meninges. When the peripheral early phase is not treated in time, central sensitization at the level of the caudal nucleus of the trigeminal nerve occurs, with concomitant repetitive firing of the involved neurons, and pain in the distribution of the ophthalmic (V1) and/or maxillary branch (V2) of the trigeminal nerve. These nerves also provide sensation to the mucosa of the sinonasal cavity and are involved in the neurobiology of primary sinonasal pain. The early sensitization phase is accompanied by cutaneous allodynia (pain induced by stimulation which is normally non-painful) in the majority of patients (80%), and often occurs in the distribution of V1 and V2 (Cady and Schreiber 2004). This may include stimulation of the nose, such as while breathing cold air. A migraine attack may include secondary nasal symptoms, which are probably mediated by stimulation of the parasympathetic nervous system via the superior salivatory nucleus of the facial nerve (VII); this reflex activation is extensively discussed in Chapters 9 and 10chapter 9chapter 10. A report on patients with sinus headaches which fulfil the migraine criteria demonstrated that 84% reported sinus pressure, 82% sinus pain, 63% nasal congestion, 40% rhinorrhea, 38% watery eyes and 27% ‘itchy’ nose (Schreiber et al 2004). Occasionally, sinonasal disease or nasal allergy can play a role in triggering migraine which may respond to nasal steroids or to leukotriene antagonists (Cady and Schreiber 2004). The appearance of ‘neurogenic rhinitis’ along with a sinus headache in patients with migraine is often misunderstood and contributes to misdiagnosis.
The duration of a sinus headache and sinus symptoms is important for differentiating ARS from a migraine attack. In contrast to ARS, a migraine headache typically resolves within 72 hours. Bacterial ARS pain typically worsens after 5–7 days and is usually associated with hyposmia or anosmia, fever, cough, maxillary dental pain and ear fullness or pressure. The diagnostic features of facial migraine are primarily based on the patient's history and on the features reminiscent of typical migraine headaches as defined by the IHS (see Chapter 9).
Throat pain or a sore throat is a common symptom. It is described as pain, discomfort or raw feeling of the throat, especially when swallowing. The vast majority of sore throats are caused by viral infections of the pharynx or tonsils. Many people experience a mild sore throat at the beginning of a common cold. The differential diagnosis of sore throat also includes various diseases of the oropharynx.
Acute pharyngitis (AP) is an infection of the pharynx, which may involve the tonsils. AP may be a part of a generalized URI, or it may be a specific localized infection inside the pharynx. The most common cause of AP is a viral infection, but it can also be caused by a bacterial infection, such as group A Streptococcus, M. pneumoniae and Chlamydia pneumoniae. The viruses causing viral pharyngitis are highly contagious and tend to spread quickly, especially during the winter season. Throat symptoms include soreness, scratchiness or irritation. Associated symptoms include fever and discomfort with swallowing or odynophagia. Fever is more prominent in young children than in adults. Most viral pharyngitis cases are accompanied by a flu or cold, along with a stuffy runny nose, sneezing and generalized aches such as arthralgia and muscle pain. Nonproductive cough and hoarseness may also be present. Oedema and erythema of the pharyngeal walls are the typical findings on physical examination. Pharyngeal exudation occurs infrequently and is generally less effusive than in patients suffering from bacterial pharyngitis.
The most common viruses causing viral pharyngitis are rhinovirus and adenovirus. Other less common viruses include Epstein-Barr virus (EBV), herpes simplex virus (HSV), influenza virus, parainfluenza virus, coronavirus, coxsackievirus, echovirus, respiratory syncytial virus (RSV) and cytomegalovirus (CMV).
Rhinovirus, which is responsible for the common cold, is one of the most common causes of viral pharyngitis. The virus does not invade the pharyngeal mucosa but causes oedema and hyperaemia of the mucous membranes, and increases the secretory activity of the mucous glands. Bradykinin and lysyl-bradykinin are generated in the nasal mucous membranes and stimulate pain nerve endings.
Adenoviral pharyngitis is common among young children and military recruits. The most common adenovirus types causing pharyngitis are adenovirus types 1–3 and 5, which directly invade the pharyngeal mucosa. Specific symptoms include fever, sore throat (more intense than that of the common cold) and conjunctivitis.
EBV pharyngitis is usually associated with infectious mononucleosis (IM).
Oedema and hyperaemia of the pharyngeal mucosa and uvula with enlargement of the tonsils are characteristic signs. Approximately 50% of patients with IM have grey-white thick patches of exudates distributed over the tonsils and pharyngeal walls, thus mimicking a streptococcal pharyngitis. Palatal petechiae associated with IM tend to be confined to the soft palate. An inflammatory exudate and nasopharyngeal lymphoid hyperplasia (adenoiditis) may also develop. Body temperature is usually high and may reach up to 104°F/40°C. Generalized lymphadenopathy is common in IM and is usually prominent in the anterior and posterior cervical triangles. Splenomegaly and hepatomegaly are present in approximately 50% and 10–15% of patients respectively. Patients suffering from IM who are treated with ampicillin may develop a diffuse, pruritic maculopapular eruption.
Herpetic pharyngitis is caused by herpes simplex virus types 1 and 2. This infection is commonly observed in children and young adults. HSV may cause gingivitis or stomatitis. A sore throat with associated gingivostomatitis is the typical presenting symptom. Other associated symptoms include fever, odynophagia, myalgia and malaise.
Enteroviral pharyngitis is usually caused by coxsackievirus or echovirus. Enteroviral lesions in the oropharyngeal mucosa are usually a result of secondary infection of the endothelial cells in small mucosal vessels, occurring during viraemia. Coxsackievirus infection is common in the late summer and early fall and may cause the pathologies described below.
Herpangina is usually caused by group-A coxsackieviruses and tends to occur in epidemics, most commonly in infants and children. It is characterized by multiple, small (1–2 mm), greyish papulovesicular lesions on the tonsils, tonsillar pillars, uvula or soft palate. The lesions may occasionally grow up to 4 mm or have an erythematous ring as large as 10 mm. The vesicles become shallow ulcers in about 3 days and heal after a few days. The pharynx is usually not involved. Associated symptoms include a sudden onset of fever with a sore throat, headache, anorexia and frequently pain in the neck, abdomen and extremities.
Acute lymphonodular pharyngitis is caused by coxsackievirus A10 and is characterized by a distribution of oral and pharyngeal lesions similar to those seen in herpangina. However, the lesions are protruding, whitish to yellowish nodules, and do not evolve into vesicles or ulcers. They remain papular and become greyish-white and nodular, secondary to infiltration by lymphocytes.
Hand-foot-and-mouth disease is usually caused by coxsackievirus A16 and is characterized by 4–8 mm ulcers on the tongue, buccal mucosa and occasionally the tonsillar pillars. Vesicular exanthems develop on the hands and feet and in infants occasionally in the diaper area.
Differentiating viral from bacterial pharyngitis solely on the basis of physical examination is not easy. In viral pharyngitis the total white blood cell count may initially be slightly high, followed by a decrease to fewer than 5000 cells after 4–7 days of illness in around 50% of the cases. Atypical lymphocytosis is frequently associated with viral pharyngitis. In IM pharyngitis, the peripheral blood smear reveals relative and absolute lymphocytosis, with more than 10% atypical lymphocytes. Liver function test results are abnormal in 90% of IM cases. A mononucleosis spot test (Monospot) is usually positive and allows rapid screening for heterophile antibodies. IgM antibody to EBV capsid antigen and antibody to early antigen are useful for diagnosing an acute infection, particularly in heterophile negative cases. Rapid streptococcal antigen test and bacterial culture of a throat swab are negative. Leukopenia and proteinuria may be seen in influenza virus pharyngitis.
Treatment of viral pharyngitis includes bed rest, salt-water gargling and fluid intake. Analgesics and antipyretics are used to relieve pain and fever.
Acute tonsillitis is inflammation of the palatine tonsils, usually due to group A β-hemolytic streptococcus (GABHS), or less commonly to a viral infection. Acute tonsillitis caused by Streptococcus species usually occurs in children aged 5–15 years. Approximately 10–20% of sore throats in adults are caused by streptococcal infection. Fever, chills, sore throat, foul breath, dysphagia, odynophagia and tenderness in the angle of the jaw characterize acute tonsillitis. The pain is frequently referred to the ears. Physical examination reveals hyperaemic and swollen palatine tonsils, uvula, tongue base and pharyngeal walls, with irregular, thin, nonconfluent patches of white exudates on the tonsils, forming the typical appearance of follicular tonsillitis. Submandibular lymph nodes are often enlarged and tender. Leukocytosis with an increased neutrophil count (shift to the left) is commonly present in bacterial infections. Mere reliance on clinical criteria, such as the presence of follicular exudate, erythema, fever and lymphadenopathy, is not an accurate means for distinguishing streptococcal from viral tonsillitis. Throat culture is the gold standard for detecting GABHS tonsillitis with a sensitivity of 80–95%. Rapid antigen detection test (RADT) detects the presence of GABHS cell wall carbohydrate from swabbed material and is considered less sensitive than throat cultures. However, RADT has a high specificity and produces results in significantly less time than a throat culture.
Chronic tonsillitis is characterized by chronic sore throat, halitosis, recurrent tonsillitis and persistent tender cervical lymph nodes. A polymicrobial bacterial population is observed in most cases, with α- and β-hemolytic streptococcal species, S. aureus, H. influenzae and Bacteroides species identified.
Complications of GABHS tonsillitis are classified into suppurative and nonsuppurative. The nonsuppurative complications include scarlet fever, acute rheumatic fever and post-streptococcal glomerulonephritis. Suppurative complications include peritonsillar, parapharyngeal and retropharyngeal cellulites and/or abscess.
Treatment of acute streptococcal tonsillitis includes adequate hydration and caloric intake. Analgesics and antipyretics are used to relieve pain and fever. GABHS infection obligates antibiotic therapy. Antibiotic therapy is directed 1Prevention of acute rheumatic fever;2Prevention of suppurative complications;3Abatement of clinical symptoms and signs; and4Reduction in transmission of GABHS to close contacts.
Penicillin is the treatment of choice in streptococcal tonsillitis.
Peritonsillar infection, also known as ‘Quincy's angina’, is an infection located between the tonsil and the superior pharyngeal constrictor muscle. The most common pathogen causing peritonsillar cellulitis/abscess is GABHS. Anaerobic Bacteroides can also cause this type of infection. Presenting symptoms include severe throat pain, especially on swallowing, fever, drooling, foul breath, trismus, hot potato voice and unilateral referred otalgia. Limited mouth opening (trismus) is always present in varying severity. The tonsil and uvula are displaced medially by the peritonsillar infection. The soft palate and the anterior pillar are swollen and hyperaemic. Unilateral enlarged and tender submandibular lymph nodes are present. Peritonsillar cellulitis without pus formation usually responds to intravenous penicillin therapy. Drainage along with intravenous penicillin is required in peritonsillar abscess. Peritonsillar abscess tends to recur and tonsillectomy is usually indicated.
Lingual tonsillitis is an infection of the lymphatic tissue located in the base of the tongue. Most patients with lingual tonsillitis have already had palatine tonsillectomy. Lingual tonsillitis presents with fever, sore throat, glossal pain, dysphagia, muffled voice and pain at the level of the hyoid bone during swallowing. Lingual tonsillitis is visible only by means of a laryngeal mirror or fibre-optic examination. The base of the tongue is enlarged and oedematous and covered by exudates. The pharynx may appear normal or mildly hyperaemic. The anterior neck may be tender at the level of the hyoid bone, and cervical and submandibular adenopathy may be observed. Treatment includes intravenous penicillin.
The parapharyngeal space (PPS) (i.e. lateral pharyngeal space, pharyngomaxillary space, pterygomaxillary space, pterygopharyngeal space) occupies an inverted pyramidal area lateral to the superior constrictor muscles, and bounded by multiple components of the fascial system. The styloid process divides the PPS into an anterior or prestyloid compartment and a neurovascular or post-styloid compartment.
Parapharyngeal space infections (PPSI) may follow an infection in the pharynx, tonsils, adenoids, teeth, parotid gland, peritonsillar area, submandibular space, retropharyngeal space, Bezold abscess (mastoid abscess on the inner aspect of the mastoid tip along the digastric ridge) and adjacent lymph nodes. Despite the multitude of well-defined potential sources, in nearly half of the cases, the aetiology cannot be defined. Signs and symptoms of PPSI differ depending on whether the prestyloid or post-styloid compartment is involved. Anterior PPSI is characterized by pain in the angle of the jaw, preauricular area, ear and adjacent upper neck. Rotating the head and neck to the contralateral side intensifies the pain. Other symptoms include dysphagia, odynophagia, drooling, trismus (due to medial pterygoid muscle irritation), fever, chills and malaise. Oedema and medial displacement or bulging of the lateral pharyngeal wall and tonsil are hallmarks of PPSI. Swelling, induration and tenderness at the angle of the mandible are also commonly observed. Dyspnea and other symptoms of airway obstruction may occur in severe cases.
Posterior PPSI is not associated with trismus or tonsillar displacement and may have no localizing signs on examination. Despite this, the patients appear to be toxic with parotid space swelling. Involvement of the neurovascular structures may lead to complications such as cranial neuropathies, Horner's syndrome, septic internal jugular thrombosis and carotid artery rupture.
CT scan of the neck (with contrast medium) facilitates the diagnosis and assessment of the extent of PPSI. A recent manuscript redefines PPSI into two different disorders that are clinically and therapeutically relevant (Sichel et al 2006):1Parapharyngeal lymphadenitis—infection located in the posterior part of the PPS with no invasion into the parapharyngeal fat and with no extensions into other cervical spaces except the adjacent retropharyngeal space. This condition is relatively benign, and intravenous antibiotics and nonsurgical treatment are recommended.2Parapharyngeal abscess—infection in the anterior part of the PPS involving the parapharyngeal fat. May also be termed deep neck abscess. Diffusion into the mediastinum and other severe complications are frequent. Early diagnosis, aggressive intravenous antibiotics and urgent surgical drainage are recommended.
The retropharyngeal space (RPS) lies between the visceral division of the middle layer of the deep cervical fascia behind the pharyngeal constrictors and the alar division of the deep layer of the deep cervical fascia posteriorly. Retropharyngeal space infection (RPSI) may follow infection in the nasopharynx, oropharynx, sinonasal region and rarely mastoiditis. RPSI may also occur directly after a traumatic perforation of the posterior pharyngeal wall or oesophagus, or indirectly, from the parapharyngeal space. Most RPSIs in children are secondary to URI, whereas trauma or foreign bodies cause most RPSIs in adults. Retropharyngeal lymph nodes tend to regress by the age of 5, causing infection in this area to be much more common in children than in adults. RPSI may drain into the prevertebral space and through this space into the chest, thus causing mediastinitis. Symptoms of RPSI include sore throat, dysphagia, stiff neck, fever and rarely posterior neck and shoulder pain aggravated by swallowing. Examination reveals anterior displacement or bulging of one or both sides of the posterior pharyngeal wall due to involvement of lymph nodes, which are distributed lateral to the midline fascial raphe. Lateral neck radiograph (demonstrating widening of the retropharyngeal space) and CT scan with contrast of the neck facilitate diagnosis and assessment of the extent of RPSI. Early diagnosis is important and treatment with aggressive intravenous antibiotics is mandatory. Transoral incision and drainage is recommended in cases with abscess formation.
Ludwig's angina is a potentially life-threatening, rapidly expanding and spreading, gangrenous cellulitis of the submandibular space. Swelling of this region can compromise the airway. Most Ludwig's angina infections are odontogenic, usually from the second or third mandibular molar. Other causes include peritonsillar or parapharyngeal abscesses, mandibular fracture, oral lacerations or piercing, and rarely submandibular sialadenitis. Predisposing factors include dental caries, recent dental treatment, systemic illnesses (such as diabetes mellitus), malnutrition, alcoholism, compromised immune system (such as AIDS) and organ transplantation. The term ‘Ludwig's angina’ is reserved for infections meeting the following five 1A cellulitic process (not an abscess) of the submandibular space;2Involvement of only the submandibular space, although this might be bilateral and spread into secondary spaces;3The finding of gangrene with foul serosanguineous fluid on incision, but no frank purulence;4Involvement of the fascia, muscle and connective tissue, with sparing of the glandular tissue; and5Direct spread of infection rather than spread by lymphatics.
The most common microbes involved are usually Streptococcus species and oral anaerobic bacteria. Symptoms include painful neck swelling, tooth pain, dysphagia, odynophagia, dyspnea, fever and malaise. Ludwig's angina is characterized by a brawny induration of the mouth floor and suprahyoid region (bilaterally), with elevation or protrusion of the tongue, thus potentially obstructing the airway. Other signs include a tender, firm swelling in the submental and anterior neck without fluctuance, tachypnea, stridor, trismus, muffled or ‘hot potato’ voice and drooling. The white blood cell count is high with a ‘shift to the left’. Airway management is the primary therapeutic concern. Airway control by endotracheal intubation is mandatory. Therapy includes intravenous broad-spectrum antibiotics and occasionally drainage of the swelling through a cervical incision with placement of drains. Dental treatment may be needed to treat the initiating tooth infection. Complications such as sepsis and descending necrotizing mediastinitis may occur through the retropharyngeal space and carotid sheath.