HIV AND ENT HIV and ENT

It has been well documented that 70 - 90% of patients with HIV will at some stage present with an ENT manifestation of the disease.

According to World Health Organisation (WHO) statistics for the year 2002, there are an estimated 5.3 million South Africans living with HIV. Although south- ern Africa is home only to 2% of the world’s population, the region accounts for 30% of people living with HIV worldwide.1

It has been well documented that 70 - 90% of patients with HIV will at some stage present with an ENT manifestation of the disease. Most of them will initially present to the general practitioner and it is important that the ENT manifestations of HIV are recognised early and that appropriate management is instituted. Antiretroviral therapy can prevent and cure most of these diseases, but as this option is not readily available to the majority of South Africans we need to look D E LUBBE at alternative treatment. MB ChB, FCORL (SA) HIV-positive patients will be included, knowingly or unknowingly, in all general Senior Registrar practices and may present with a complaint totally unrelated to their underlying Division of Otorhinolaryngology disease or with an AIDS-defining or malignancy. Routine Groote Schuur Hospital organisms predominate when it comes to infections of the ear, nose and throat, Cape Town which means that HIV-positive patients should be treated with the same antibiotic as HIV-negative patients, based on the most likely organism responsible for that specific infection. The most common ENT manifestations of HIV are listed in Dr Lubbe qualified as an ENT surgeon at Table I. the University of Cape Town in 2003 and is currently working as a senior registrar in ORAL CAVITY MANIFESTATIONS the Department of Otorhinolaryngology at Contrary to medical training in the pre-HIV era, multiple can be present Groote Schuur Hospital. Her current MMed simultaneously in those infected with HIV. Recent studies have proved that there is project includes the investigation of otitis a decreased appearance in the occurrence of buccal lesions in those on triple 2 media with effusion in the HIV-positive pop- antiretroviral therapy. ulation to determine the natural progres- is one of the earliest and most common findings suggesting sion of the disease and to establish a treat- HIV infection and is characterised by a typical cottage cheese inflammatory ment protocol. appearance. These patients usually still appear healthy, but if a known HIV-posi- tive patient develops thrush, long-term survival appears to be diminished. Four different types of oral candidiasis can be identified: • Pseudomembranous candidiasis. This is the classic form identified by a white plaque that can be scraped off, leaving an erythematous, bleeding base. • Hyperplastic candidiasis. These lesions can easily be confused with leuko- plakia or oral hairy since they appear as thick white plaques that cannot be scraped off. If there is any doubt regarding the diagnosis, a is indicated. • Atrophic candidiasis. This presents as flat atrophic lesions involving the oral and oropharyngeal mucosa. • Angular . These are oral commissure erythematous lesions that can involve the adjacent skin.

Topical agents are usually effective in the early stages of HIV infection, but with advanced disease and increasing immune deficiency, systemic therapy (with flu- conazole and rarely amphotericin B) may be indicated. Oesophageal or pharyn- geal candidiasis is more common in this patient population and should be sus- pected in patients with oral candidiasis who develop a severe sore throat or diffi- culty in swallowing.

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Table I. ENT manifestations of HIV

Oral Nose and paranasal Otological and sinuses neurotological Head and neck • Recurrent Cutaneous lesions • Otitis externa • Generalised lymphadenopathy aphthous ulcers • Kaposi’s sarcoma • Otitis media with • Neck mass • Candidiasis • effusion Mycobacterium tuberculosis • Herpes simplex • Herpes zoster • Eustachian tube Mycobacterium avium • • Seborrhoeic dermatitis dysfunction Cryptococcosis • Non-cutaneous • Kaposi’s sarcoma Histoplasmosis • Periodontitis • Adenoid hypertrophy • Sensorineural Coccidioidomycosis • Condylomata • Eustachian tube hearing loss • Parotid gland cyst • Hairy leukoplakia obstruction • Bell’s palsy • Kaposi’s sarcoma • Allergic rhinitis • Non-Hodgkin’s • Acute AND chronic sinusitis

Oral hairy leukoplakia (Fig. 1) is Herpes simplex infections are viduals practise good oral hygiene a condition almost pathognomonic of more common in those infected with and that even a mild gingivitis be HIV infection and often indicates pro- HIV, with being the treated early and adequately. gression to AIDS. The most fre- most common manifestation. These Treatment consists of rinsing the mouth quently appears on the lateral aspect ‘fever blisters’ are larger, more numer- with chlorhexidine solution, antibiotics of the with a thick, vertically ous, recur more often and persist for against Gram-negative anaerobes and correlated (‘hairy’) whitish plaque, longer periods. Herpes simplex early referral to an oral surgeon if the very similar in appearance to the type 1 (HSV-1) is most commonly condition progresses. hyperplastic type of oral candidiasis. involved and usually begins as small Potassium hydroxide (KOH) prepara- bullae that rupture and then coalesce. Recurrent aphthous ulcers are tions of surface scrapings will identify It can extend onto the adjacent facial frequently encountered in the HIV-posi- the mycelia or hyphae seen in candidi- skin and form giant herpetic lesions. If tive patient and present as painful asis and can therefore be used to dif- the diagnosis is in doubt then smears ulcers on the non-keratinised unat- ferentiate between these two condi- or scrapings should be taken before tached . They vary in size tions. A biopsy of the lesion will be starting treatment. Mild oral herpes from 0.2 mm to > 6 mm. Treatment is diagnostic of oral hairy leukoplakia. infections can usually be treated con- aimed at symptomatic relief using anti- The Epstein-Barr virus has been identi- servatively, but high-dose oral septic mouthwashes, topical steroids fied as the most likely causative agent. (up to 4 g/day) should be used for and topical anaesthetics. In cases of Oral hairy leukoplakia is typically more severe lesions. Aciclovir will only large ulcerative lesions and frequent asymptomatic and does not usually be effective in eradicating the lesions recurrences a biopsy should be consid- require any treatment, but it is a signif- if started within the first few days of ered if lesions do not respond to treat- icant finding because of its diagnostic infection. Prophylactic aciclovir can be ment. and prognostic implications. The used in the severely immunocompro- lesions have been successfully treated mised patient with frequent recur- AIDS patients have a much higher inci- with aciclovir (2 g/day), sulpha drugs, rences. dence of developing oral cavity or topical retinoic acid Kaposi’s sarcoma and non-Hodgkin’s (Fig. 1). , gingivitis lymphoma. and necrotising gingivitis are much more severe in the HIV-positive Kaposi’s sarcoma (Fig. 2) is the patient and can produce significant most common oral tumour in AIDS morbidity with extensive tissue destruc- patients and one of the AIDS-defining tion. HIV-associated gingivitis usually illnesses. These dark, purple/pink begins with erythematous, bleeding macular lesions can be found on after minor trauma and can then any oral mucosal surface, but the rapidly progress to a necrotising is involved in 95% of cases. process known as acute necrotising Although the diagnosis is usually easi- ulcerative gingivitis (ANUG), a condi- ly made based on clinical findings, an tion that has once again resurfaced in excisional biopsy is indicated, prefer- Fig. 1. Oral hairy leukoplakia (repro- the HIV-positive population. It is there- ably from a cutaneous lesion, as these duced with permission from Professor fore imperative that HIV-infected indi- lesions are vascular and can bleed Jos Hille).

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controversial. It has been noted, how- infection must be entertained. In HIV- ever, that there are many young HIV positive patients enlarged adenoids patients with no or moderate risk fac- are most commonly due to lymphoid tors for SCC who present with this and generalised lym- condition of the oral cavity.3 SCC phadenopathy and enlarged tonsils seems to present at an earlier age and are frequently part of the clinical pic- tends to be more aggressive in HIV- ture. The aetiology is thought to be positive patients compared with HIV- related to B-cell activation by HIV, negative patients in the same age Epstein-Barr virus or cytomegalovirus. Fig. 2. Kaposi’s sarcoma of the group. This suggests that there might Otitis media with effusion (OME), palate (reproduced with permission be a relationship between SCC and causing a conductive hearing loss, from Professor Jos Hille). HIV infection, although there is as yet often follows owing to obstruction of little evidence to support this. The lat- the Eustachian tubes by the lymphoid profusely. Treatment is mostly pallia- eral tongue is most frequently involved mass in the postnasal space. tive, as HIV-positive patients with and the lesion can be either infiltrative Lymphoma, especially high-grade B- Kaposi’s sarcoma are already severely or exophytic. Diagnosis is made on cell lymphoma, needs to be excluded. immunocompromised and the eventual biopsy and management of the HIV- The diagnosis of a postnasal mass can cause of death is usually opportunistic positive patient with SCC should be be made by doing a posterior infections and not tumour effects. no different from that of an HIV-nega- rhinoscopy using an angled mirror, a Treatment also depends on whether tive patient. Unless the patient is surgi- lateral radiograph of the neck or a there is a solitary tumour or dissemi- cally unfit or severely immunocompro- flexible nasopharyngoscope, the last nated disease. Although Kaposi’s sar- mised, s/he will benefit from surgical being the method of choice used by coma is very radiosensitive and resection and neck dissection, as con- ENT surgeons. A biopsy of the mass localised tumours respond well to low- ventional radiotherapy is poorly toler- can then be taken either under local dose radiotherapy with a ated. or general anaesthesia. For lymphoid good palliative result, HIV-positive hyperplasia, an adenoidectomy is fre- patients appear to be more susceptible HIV SINONASAL quently done to relieve symptoms of to radiation-induced mucositis and MANIFESTATIONS nasal obstruction and OME. cannot tolerate wide-field radiation. Between 40% and 70% of patients Lymphoma is treated with systemic Alternative treatment options include with AIDS will present with allergic chemotherapy, and radiotherapy is intralesional chemotherapy for solitary rhinitis, sinusitis, a blocked nose or given if the tumour causes any mass lesions and systemic chemotherapy for symptoms suggesting a possible effect. disseminated disease. Systemic thera- sinonasal malignancy. The most com- py causes further immunosuppression, mon sinonasal complaint is that of a Allergic rhinitis is very common in with an increased risk of opportunistic blocked nose, the most likely causes the HIV-positive population. Although infections. Laser and cryotherapy have being adenoidal hypertrophy and cellular immunity is depressed, there also been used for localised tumours. allergic rhinitis. HIV-infected individu- seems to be increased polyclonal B- als often suffer from other common ill- cell activation with increased circulat- Non-Hodgkin’s lymphoma is the nesses, such as TB, which can present ing immune complexes and increased second most common AIDS-associated with unusual manifestations of the levels of IgE. These high levels of IgE malignancy. The tumours are exophytic disease (Fig. 3). then lead to increased IgE-mediated and can involve the alveolar ridge, allergic symptoms. Patients present palate, gingiva, sinonasal cavity, with sneezing, watery rhinorrhoea and orbit, neck and nearly any other site. a blocked nose. Treatment consists of Most patients have extranodal disease allergen avoidance, topical steroids and 70% of cases have high-grade and oral antihistamines. disease, with a poor prognosis and systemic chemotherapy options limited Acute, recurrent and chronic rhinosi- by the already compromised nusitis occurs in 20 - 68% of HIV- . The diagnosis must be infected patients. Predisposing factors made by tissue biopsy if from an include allergic rhinitis, with the oede- extranodal site or by doing a com- matous mucosa leading to obstruction plete excisional biopsy from the low- Fig. 3. An old disease (TB) with an of the sinus ostia, and decreased local est, largest involved cervical lymph unusual presentation. and systemic immunity. The microbiolo- node in the neck. gy is similar to that found in the HIV- Adenoidal hypertrophy will pres- negative population, with the excep- The association between squamous ent as persistent nasal obstruction. In tion of two atypical opportunistic cell carcinoma (SCC) of the head the adult patient this demands further organisms, Pseudomonas aeruginosa and neck and HIV infection is still investigation and the possibility of HIV and Aspergillus fumigatus. Initial man-

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agement should be restricted to the completely asymptomatic. The natural Acknowledgements use of first-line drugs such as progression of the disease is unknown. I would like to thank Professor Jos amoxicillin or amoxiclav. Management of these lesions includes Hille of the National Health Mucormycosis, although found in the confirming the diagnosis with ultra- Laboratory Services/University of the neutropenic immunocompromised host, sound or FNAC. Ultrasound clearly Western Cape (NHLS/UWC) for pro- is not a common disease in the HIV- shows multiple cystic lesions within the viding two illustrations. infected patient. The reason for this is parotid gland. Treatment is guided by that the HIV-positive patient does not the severity of the cosmetic deformity References available on request. become neutropenic until the final and includes antiretroviral therapy, stages of the disease process. aspiration of the cysts with tetracycline Further reading sclerosis and low-dose radiotherapy. Moazzez AH, Alvi A. Head and neck manifes- NECK MANIFESTATIONS tations of AIDS in adults. Am Fam Physician 1998, 57: 1813-1821. Enlarging neck masses frequently Infectious processes causing cervi- Tami TA. Otolaryngologic manifestations of acquired syndrome. occur in the HIV-infected population cal lymphadenopathy include tubercu- Otolaryngol Clin North Am 1992; 25: 1147- and up to 90% of patients with an losis, non-tuberculous mycobacterial 1367. ENT manifestation will present with a infections, toxoplasmosis, bacterial neck mass as well. The diagnostic lymphadenitis and fungal infections. approach differs slightly from that of OTOLOGICAL the HIV-negative patient where investi- IN A NUTSHELL MANIFESTATIONS gations are mostly aimed at excluding Routine organisms predominate a malignancy. The differential diagno- Ear manifestations occur less frequent- when it comes to infections of the sis of a neck mass in the HIV-positive ly than the abovementioned ENT mani- ear, nose and throat. patient can be broadly divided into festations. HIV-infected individuals do HIV-positive patients should initially the following categories: not have a higher incidence of otitis be treated with the same antibiotic • HIV lymphadenopathy externa and they are not more prone as HIV-negative patients. • parotid disease to develop necrotising otitis externa • infections when compared with other immuno- Multiple oral lesions can be present • neoplasms. compromised patients such as diabet- simultaneously. ics. Oral candidiasis and rhinosinusitis Persistent generalised lympha- are the commonest ENT manifesta- denopathy is present in up to 70% The incidence of OME is higher in the tions of HIV. of patients in the initial period after HIV population than in the general seroconversion and can be defined in population, and if an adult patient Oesophageal candidiasis must be adults as lymphadenopathy presents with bilateral middle ear effu- suspected in patients with increas- greater than 1 cm, of unexplained sions the possibility of HIV infection ing dysphagia and odynophagia. aetiology and involving 2 or more must be considered. Patients with Oral hairy leukoplakia is a condi- extra-inguinal sites for longer than 3 OME must be referred to an ENT sur- tion almost pathognomonic of HIV months.4 Indications for further investi- geon for further investigations and infection and often indicates pro- gations and fine-needle aspiration exclusion of a postnasal lesion gression to AIDS. cytology (FNAC) include the following: obstructing the eustachian tubes. rapidly enlarging lymph nodes, a Treatment is still controversial, but Kaposi’s sarcoma is the most com- dominant node, asymmetrical lym- includes the insertion of ventilation mon HIV-associated oral tumour phadenopathy, firm non-mobile nodes tubes and an adenoidectomy if indi- and predominantly occurs on the and recent weight loss. cated. palate.

HIV-associated cystic lympho-epithe- Parotid gland enlargement is Sensorineural hearing loss lial disease of the parotid is a dis- commonly encountered in HIV-infected occurs in the HIV-infected patient and ease process unique to HIV-infected adults and children who are not on the causes include a direct effect of individuals. antiretroviral therapy. HIV-associated the neurotropic virus on the cochlear cystic lympho-epithelial disease nerve, syphilis, and cryptococcal OME in the adult patient must be (benign lympho-epithelial cysts) is a meningitis. Investigations include a investigated and the HIV status of disease process unique to HIV-infected complete audiology work-up, ENT the patient ascertained. individuals and the aetiology is examination, laboratory tests to thought to be related to a lymphoid exclude syphilis, and imaging if indi- response to HIV infection. Patients cated. Cerebrospinal fluid is sampled usually present with bilateral, painless in some instances to detect Treponema parotomegaly and, except for the pallidum and the cryptococcal anti- cosmetic deformity, these lesions are gen.

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