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J Oral Maxillofac Surg70:135-140, 2012

Rhinoscleroma With Involvement of theMaxillary Sinus, Orbital Floor, and

Temporomandibular Joint: A Case ReportRahul R. Bhowate, MDS,* Shirish Degwekar, MDS,†

Shivlal Rawlani, MDS,‡ and Surwarna Dangore, MDS§

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Rhinoscleroma is caused by Klebsiella rhinosclero-matis, a gram-negative aerobic coccobacillus. Rhino-scleroma is a worldwide disease that occurs under allclimatic conditions. Most cases are from tropical andtemperate zones. The disease has been widely re-ported from countries in the Middle East, India, andSoutheast Asia.1-3 A large endemic area has been re-ported in Eastern Europe extending into Ukraine andaround the Black Sea and Caspian Sea.2 A high inci-

ence of scleroma has been reported from Egypt,preading throughout Africa, accounting for 5% of allorldwide cases.4 Most cases in Central and South

American are from Guatemala.2,3 Rare sporadic casesccur in the United States, usually in immigrant pop-lations arriving from countries in which the disease

s endemic.5-8 In the United States, Shum et al8 re-orted rhinoscleroma in immigrants from Mexico andentral America and agreed that physicians along theouthwestern border of United States should knowlinical manifestation of rhinoscleroma. It is mini-ally contagious, requiring a long period of contact

or transmission, and is associated with crowding andoor sanitation. Involvement of the nasal cavity andpper respiratory tract is most common, but exten-ion into the soft and hard palate, upper lip, andaxillary sinuses have been reported.9

Rhinoscleroma affects all ages without gender pref-erence. It is most frequently recognized in youngadults who may have symptoms longer than 10 years

Received from the Department of Oral Medicine and Radiology,

Sharad Pawar Dental College and Hospital, Sawangi (M), Wardha,

Maharashtra, India.

*Professor and CMS.

†Professor and Head.

‡Senior Lecturer.

§Reader.

Address correspondence and reprint requests to Prof Bhowate:

Department of Oral Medicine and Radiology, Sharad Pawar Dental

College and Hospital, Sawangi (M), Wardha 442004, Maharashtra,

India; e-mail: dr_bhowate@yahoo.com

© 2012 American Association of Oral and Maxillofacial Surgeons

278-2391/12/7001-0$36.00/0

oi:10.1016/j.joms.2011.02.004

135

before diagnosis. The incidence of nasal extension tothe oral cavity and pharynx varies from 18% to 43% indifferent areas.5 The initial complaint is sore throat,

hich is associated with nonspecific pharyngitis, pro-ressive nodular infiltration of the soft and hard pal-te, tonsillar fossa, and oropharynx. Bone destructionith subsequent defect in the hard palate may occur.

ibrosis of the soft palate and tonsillar pillars results intenosis of the oral airway.10

There is a wide variation in clinical presentationdepending on the organ involved and the duration ofthe disease. It usually affects the sinonasal cavities butall respiratory passages can be involved.8,9 The dis-ase manifests as 3 overlapping phases: exudative,roliferative, and fibrotic (cicatricial). The exudativehase is characterized by watery nasal discharge, fron-

FIGURE 1. Clinical photograph showing proptosis in the patient’sright eye.

Bhowate et al. Rhinoscleroma. J Oral Maxillofac Surg 2012.

136 RHINOSCLEROMA

toethmoidal headache, and difficulty in breathing,with nasal obstruction and epistaxis. The nasal mu-cosa and nasal septum may be hyperemic, edematous,or hypertrophic, with yellowish crusting. Sore throatand hoarseness may be present if the nasopharynx isinvolved. The exudative phase usually mimics a com-mon cold, which mandates a culture study of nasalmucosal secretions if the patient resides in an en-demic area of rhinoscleroma. The proliferative phaseincludes progressive infiltration of the nasal mucosaand septum, leading to formation of granulomatouswaxy nodules. This leads to nasal obstruction, expan-sion of the anterior naris, and deformity of the upperlip. Proliferative granulomatous infiltration of the softpalate and uvula leads to dysphonia and dysphasia.Proliferative granulomatous infiltration of nasal skinleads to destruction of the nasal cartilage, causing“Hebra nose.” The fibrotic or cicatricial phase showsscarring or stenosis of the airway, leading to obstruc-tion, and further involvement of an adjacent vitalorgan, eg, the maxillary sinus, orbit, and temporoman-dibular joint causes frontoparietal headache, epi-phora, proptosis, and trismus. When a patient residesin an endemic area of rhinoscleroma presenting withthe described signs and symptoms, thorough exami-nation of the nasal mucosa, nasal septum, soft palate,uvula, oropharynx, nasopharynx, and paranasal si-nuses followed by culture study for K rhinoscleroma-tis should be strictly followed.5-9

FIGURE 2. Panoramic open-mouth view shows the left condyle heaglenoid fossa. Involvement of the right maxillary sinus, loss of coevident.

Bhowate et al. Rhinoscleroma. J Oral Maxillofac Surg 2012.

Report of a Case

A 14-year-old girl with low socioeconomic status, a resi-dent of an urban slum area, was referred by a private ear,nose, and throat surgeon to Sharad Pawar Dental Collegeand Hospital (Wardha, India) for progressive difficulty inopening her mouth during the previous 6 months. Thepatient’s medical history as told by her mother disclosed aloss of hearing acuity of the right ear and bleeding from theright nostril from 9 years of age. Also, since 11 years of age,the patient had a right-side headache with deep burningpain in the right midfacial and temporal regions whileopening and closing the jaw. Her mother stated that shewas undergoing treatment for loss of hearing and nasalbleeding during the previous 6 years, but without any sig-nificant improvement. Because she developed difficulty inopening her jaw, the ear, nose, and throat surgeon referredthe patient for dental evaluation.

Extraoral examination showed diffuse tender swellingover the right midfacial, zygomatic buttress, preauricular,and temporal regions. Proptosis of the right eye was alsopresent (Fig 1). Temporomandibular joint movement wasrestricted on the right side. Both nostrils showed atrophicmucosa without polypoid growth. The right jugulodigastriclymph node was mobile, tender, and 1 � 1 cm. Intraoralexamination was difficult because the interincisal distancewas only 5 mm. Forceful maximum possible opening didnot show any intraoral ulceration or swelling on the palatalor maxillary buccal sulcus region on the right side. Onpalpation, the right maxillary tuberosity region was tender.

Panoramic, Water view, and lateral cephalograms wereobtained to study the maxillofacial skeleton. Panoramicradiograph with an open-mouth position showed destruc-tion of the lateral and inferior walls of the maxillary sinus

nst the articularis eminence and the right condyle head against then of the lateral pterygoid plate, and pterygomaxillary fissure are

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BHOWATE ET AL 137

and inferior orbital rim on the right side. The left condylehead was against the articular eminence, but the right con-dyle head remained in close contact to glenoid fossa, whichsuggested involvement of the right temporomandibularjoint region. Panoramic radiograph also showed a missingright pterygoid plate with an indistinct pterygomaxillaryfissure and a displaced, inverted, developing maxillary thirdmolar (Fig 2). The Water view showed a loss of corticationof the inferior and posterolateral walls of the maxillarysinus. Involvement of the right coronoid process, zygomaticbone, and inferior orbital rim with a hazy maxillary sinus

FIGURE 3. Water view showing a radiopaque right maxillarysinus involving cortication of the maxillary sinus, orbital floor, andextension into the ipsilateral nasal cavity (arrows).

Bhowate et al. Rhinoscleroma. J Oral Maxillofac Surg 2012.

FIGURE 4. Lateral cephalogram showing chara

howate et al. Rhinoscleroma. J Oral Maxillofac Surg 2012.

was evident (Fig 3). Lateral cephalogram showed the char-acteristic V-shaped thickening of the soft palate at its attach-ment to the hard palate (Fig 4). Chest radiograph did notdisplay any obvious parenchymal or pleural pathology. He-matologic and biochemical results were within normallimits.

Considering the clinical course of the disease and theradiologic findings, chronic granulomatous diseases such astuberculosis, actinomycosis, histoplasmosis, malignancy ofthe maxillary sinus (lymphoma), and nasopharyngeal tumorwere included in the differential diagnosis. Aspiration fromthe right maxillary sinus yielded scanty material, whichshowed the growth of K rhinoscleromatis. This culture wassensitive to tetracycline, ciprofloxacin, trimethoprim-sulfa-methoxazole, and cephalosporin. Because of the presenceof severe trismus, the patient was referred to the ear, nose,and throat department for nasal endoscopic biopsy. Subse-quent staining with hematoxylin and eosin showed a mixedinflammatory cell infiltrate and a collection of large, foamy,vacuolated Mikulicz cells beneath the nasal squamous epi-thelium (Fig 5).

The patient was prescribed ciprofloxacin 500 mg orallytwice daily. After 1 month of therapy, the frequency andseverity of burning pain and headache decreased, withoccasional nasal bleeding. Lateral cephalogram showed de-creased V-shaped thickening of the soft palate (Fig 6). Dur-ing the second month of therapy, the patient developedchicken pox, which was subsequently treated symptomat-ically. After 2 months, the patient reported complete relieffrom burning pain, headache, and nasal bleeding, but therewas no improvement in trismus, and lateral cephalogramshowed a greater decrease in V-shaped thickening of thesoft palate compared with the previous cephalogram (Fig7). Throughout therapy with ciprofloxacin, the patient didnot present with adverse gastrointestinal discomfort or anyother allergic reaction.

At this point, aspiration from the maxillary sinus regionwas negative for K rhinoscleromatis. After 2 months, cip-rofloxacin was discontinued and the patient was referred

V-shaped thickening of the soft palate (arrows).

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138 RHINOSCLEROMA

to the department of maxillofacial surgery for correctionof trismus. The patient was unwilling to undergo anoperative procedure, and 1 year later the patient was lostto follow-up.

Discussion

Rhinoscleroma is a disabling disease because of itspersistent, specific, granulomatous inflammatory na-ture resulting in fibrosis and sclerosis. Nasal or naso-pharyngeal involvement is seen in 95% to 100% ofpatients. Extension from the nose or primary diseaseto the oral cavity and oropharynx is seen in 18% to43% patients.8 In rhinoscleroma with paranasal sinus

isease, 40% to 60% show involvement of the maxil-

FIGURE 5. Photomicrograph displays foamy cells beneath thenasal squamous epithelium (hematoxylin and eosin stain; magnifi-cation, �200).

Bhowate et al. Rhinoscleroma. J Oral Maxillofac Surg 2012.

FIGURE 6. Lateral cephalogram after 1 month shows decre

Bhowate et al. Rhinoscleroma. J Oral Maxillofac Surg 2012.

ary sinus.8,10 Scleroma has been reported in manyunusual sites in the head and neck, including theorbit, lacrimal system, facial skin, and anterior cranialfossa.10-14 Involvement of the maxillary sinus in scle-roma may act as a reservoir of infection, thus a patientmay take a longer time to respond to antibiotic ther-apy.10 In the present case, the patient was put onantibiotic therapy for 2 months because the maxillarysinus was involved.

Klebsiella rhinoscleromatis is not found in normalnasal secretions; therefore, a positive culture is con-sidered diagnostic of scleroma.9 The presence ofgram-negative diplobacillus in Mikulicz cells or inter-cellular spaces is considered pathognomic of rhino-scleroma.4,5,8 Immunochemistry adds great specificityn the diagnosis of scleroma when histology is notiagnostic.5,8 An immunoperoxidase technique may

also be helpful in identification of K rhinoscleromatiswhen cultures are negative.5,15

Cellular immunity is impaired (immunocellular de-ficiency) in patients with rhinoscleroma, which pre-disposes them to infection.16 The CD4/CD8 cell ratioin the lesion is altered, with decreased levels of CD4lymphocytes, which possibly induces a decreased T-cell response.17 In the present case, the patient hadan episode of chicken pox, which could be attributedto impaired immunocellular deficiency.

Antibiotics constitute standard treatment in theearly stage of the disease. Surgery and laser ablationcan be used only after the disease is under controlwith antibiotics and the cultures are negative.18 Anti-biotics do not reach an effective level in relativelyavascular scar tissue; therefore, patients presentingwith areas of fibrosis and scarring require surgery as

haracteristic V-shaped thickening of the soft palate (arrows).

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BHOWATE ET AL 139

well as antibiotic therapy.18,19 Dawlatly et al20 re-ported 3 cases of surgical iatrogenic complications innasopharyngeal rhinoscleroma and emphasized mini-mal surgical intervention with appropriate antimicro-bial therapy. Direct implantation of microorganismsor alteration of hemodynamics contributes to subse-quent extensive local spread of the lesion, culminat-ing in pharyngeal and nasal obstruction with atten-dant difficulty in swallowing and hearing impairmentin these patients.20 Antibiotics used in the treatment

f rhinoscleroma include streptomycin, tetracycline,6

ciprofloxacin,21 trimethoprim-sulfamethoxazole,9

rifampicin,22 clofazimine,23 cephalosporins,24 and sul-amethoxazole.9 Ciprofloxacin is preferred over other

antibiotics because it has excellent tissue penetrationand is concentrated within macrophages. Adverse ef-fects are comparatively few and include gastrointesti-nal symptoms in 3% to 6% of patients. Central nervoussystem and allergic symptoms are uncommon.25

In the present case, involvement of the maxillarysinus, inferior orbital rim, condyle head, coronoidprocess, and lateral pterygoid plate was suggestive ofa spread of a granulomatous lesion along the tissueplanes, resulting in trismus. Invasion of the orbitalfloor from the neighboring maxillary sinus may lead toproptosis. Destruction of the lateral pterygoid plateand pterygomaxillary fissure pointed toward involve-ment of the pterygopalatine and infratemporal fossa,

FIGURE 7. Lateral cephalogram after 2 months shows d

howate et al. Rhinoscleroma. J Oral Maxillofac Surg 2012.

which could be correlated to the deep burning pain

in the present case. The V-shaped thickening of thesoft palate also helped determine the diagnosis. The“palatal sign” was previously used as a diagnostic aidfor the radiologic diagnosis of rhinoscleroma.26 Withthe administration of antibiotics, the V-shaped thick-ening decreased, indicating a response to treatment.Antibiotics helped control the infection, thus reliev-ing the burning pain, headache, and nasal bleeding,but did not correct the trismus.

In the present case, the patient had the character-istic signs and symptoms of sinonasal tract infectionover many years, although she sought medical treat-ment without relief. Diagnosis of the disease is basedon clinical, bacteriologic, and histopathologic find-ings and supported by radiologic examination. Immi-grants from endemic areas of rhinoscleroma to a re-gion without infection with K rhinoscleromatis posea serious problem because of transfer of disease.Hence, such immigrant patients with chronic respira-tory disease should be thoroughly screened for rhino-scleromatis.

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140 RHINOSCLEROMA

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