Unusual Presentation of Rhinoscleroma Dehadaray A1, Patel M2, Kaushik M3, Agrawal D4

Case Report Unusual presentation of Rhinoscleroma Dehadaray A1, Patel M2, Kaushik M3, Agrawal D4

ABSTRACT Rhinoscleroma or respiratory scleroma is a chronic, slowly progressive, inflammatory disease of the upper respiratory tract. Here we present a 35 year old male presenting with unilateral orbital complaints and nonspecific findings on radiological examination, diagnosed only by histopathology as Rhinoscleroma. Due to the low incidence of this disease and its rare presentation in this case, diagnosis was a challenge, but outcome was successful. Keywords- Rhinoscleroma, Orbit

1 MS ENT, Professor, 2 MS DNB ENT, Assistant Professor, 3 MS ENT, Professor and Head, 4 PG student, Department of ENT and HNS, Bharati Vidyapeeth Deemed University Medical College, Pune-411043. Corresponding Author: Dr. Monika Patel, Assistant Professor, Bharati Vidyapeeth Deemed University Medical College, Pune, Maharashtra. Mob: +919028738326 Email: [email protected]

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Introduction hinoscleroma is a chronic progressive, specific granulomatous infectious disease affecting the Rupper respiratory tract associated with Klebsiella rhinoscleromatis infection. It is endemic in Central and South America, Central Africa, Middle East, parts of Europe, India, and Indonesia [1]. It most commonly af- fects the nose [2]. Cases of Rhinoscleroma with invasion into the orbits are rare, with very few cases reported in litera- ture. Ophthalmologists should also be aware of the Fig 1a Fig 1b disease and the problems in its management. We present a case of Rhinoscleroma which posed difficulty in with reduced movements in all directions, and vision diagnosing due to its clinical resemblance with other of 6/18. Anterior rhinoscopy revealed mucopurulent chronic granulomatous conditions, neoplastic diseas- discharge in the right nasal cavity. Rest of the cranial es and its unusual site of presentation. nerves were normal.(Fig. 1a) Case Report Microbiological examination of nasal discharge revealed Coagulase negative Staphylococcus, but no AFB A 35 year old male presented to ENT OPD with or fungus. Routine blood investigations were normal; chief complaints of excessive watering, redness and with cANCA negative (2.39 RU/ml). reduced movements of right eye since 4-5 months. He did not complain of nasal obstruction or epi- HRCT PNS and Orbit with contrast showed bilat- staxis. Patient was treated for pulmonary tuberculo- eral pansinusitis with destruction of medial wall of sis 3 years back. On examination, right eye propto- right maxillary sinus and a soft tissue mass in the right sis (4mm) and conjunctival congestion were present retrobulbar region, involving the inferior and medial recti muscles, suggestive of pseudo- tumor. (Fig. 2a, b, c, d, e) Biopsy taken from the hyper- trophied mucosa of right maxillary sinus showed polyps with inflammatory cell infiltrate, suggestive of inflammatory polyp. Patient was given various intravenous antibiot- ics, like cefotaxime, metronidazole, piperacillin+tazobactam, gentamycin and levofloxacin during the course of Fig 2c hospitalisation but showed no symp- Fig 2a tomatic improvement. Excisional biopsy of the right peri-orbital mass

Fig 2b Fig 2d Fig 2e

The Indian Practitioner q Vol.70 No.11. November 2017 33 Case Report was done by lateral rhinotomy ap- serious complications like men- proach, where a soft, greyish-white ingitis and blindness by pressure mass was seen extending up to the on the optic nerve. Infection and orbital apex and was adherent to the haemorrhage are usually the cause nasolacrimal duct. Biopsy showed Mikuliez cell of death in fatal cases of this scle- chronic inflammatory cells, Russell rotic process. bodies and Mikulicz cells with no Russell body The first case of orbital involve- evidence of fungus or malignancy, ment by rhinoscleroma was re- using PAS and Silver methena- ported by Hara [5]. He postulated mine stains (Fig. 3). A diagnosis that the route of extension was of Rhinoscleroma was made and either through the lamina papy- patient was started on oral chlor- racea or by way of the nasolacri- amphenicol and prednisolone and mal tract into the orbit. Shaw and then septran and doxycycline. On 6 Martin [4] reported a case in their week follow up, patient’s right eye series which showed invasion of movements have improved, with both orbits and the skull base with no watering of eyes and reduced Fig 3 pressure atrophy of adjacent bone conjunctival congestion. (Fig. 1b) leading to proptosis and blindness. In our case nasolacrimal duct appeared normal, hence the exact route Discussion of spread could not be found; although lamina papy- The route of transmission of this disease is not racea was found to be eroded intraoperatively; it could known as the organisms cannot survive outside, and have spread via the sinuses, as shown by computer- humans are the only identified host. Environmental ised axial tomography scan. Hsiung-Hsiang reported factors like overcrowding, poor hygiene and malnu- a similar case in 1958 as cited by Lubin JR in 1981[6]. trition is commonly associated with the acquisition of The mode of extension in that case was also through this disease. Rhinoscleroma is a diagnostic challenge, the sinuses. The possibility of intracranial spread of as it is an uncommon disease. infection is emphasised in these cases because of dif- The diagnosis can be established on the basis of his- ficulty in eradicating the organism since they are topathological, bacteriological and genetic examina- present within the macrophages are protected from tions. The disease can be divided into three sequential the host’s immune system, and hence remain viable but overlapping morphological stages: catarrhal or ex- even through prolonged courses of antibiotic therapy. udative, granulomatous or proliferative and sclerotic Hence prompt suspicion and treatment is required or cicatrical phase. During the catarrhal stage, there is when rare sites are involved. inflammation of the nasal mucosa giving rise to per- The presenting features and imaging tech- sistent rhinitis, mucopurulent discharge and atrophic niques usually provide a presumptive diagnosis of changes start occurring. In the second stage, inflamed Rhinoscleroma but histopathological examination mucosa coalesces to form granulomas. These granu- remains the mainstay for the definitive diagnosis. lomas may infiltrate other portions of the airway and Catarrhal stage shows a nonspecific sub-epithelial in- [3] cause scarring, giving rise to the cicatrical stage . In filtrate of polymorphonuclear leukocytes with granu- many cases of rhinoscleroma, the presence of all three lation tissue. The diagnostic histological changes of stages can be found at the time of diagnosis. rhinoscleroma are only seen during the proliferative Cases of rhinoscleroma with invasion into the or- phase, i.e. lymphocytes, plasma cells, Russell bodies bits are rare. The lesion usually starts as small granu- and the pathognomonic large Mikulicz cells; foamy lomas, which eventually coalesce to form obstructing macrophages with numerous cytoplasmic vacuoles masses and cause respiratory symptoms according containing viable and non-viable Klebsiella bacilli. to the site involved. Infiltration upwards through the Mikulicz cells are sparse or absent in the initial ca- sinuses and lacrimal apparatus is unusual but is be- tarrhal and final sclerotic stages. In the sclerotic phase, ing reported with increasing frequency [4]. It does not extensive fibrosis and few inflammatory cells are seen invade bone itself, but continuous progression of the [7]. The Mikulicz cells [8] are characteristic of rhinoscle- process causes pressure atrophy of facial bones and roma but not pathognomonic. They are also seen in may invade the base of the skull and orbits, causing leprosy and bubonic plague and are thought to rep-

34 The Indian Practitioner q Vol.70 No.11. November 2017 Case Report resent the cells’ nonspecific response to toxins- pro challenging when Mikulicz cells are deficient. Our duced by the causative organism. Large numbers of case serves to highlight that rhinoscleroma can pres- Klebsiella rhinoscleromatis can sometimes be found ent with only orbital manifestations, and we hope to within the Mikulicz cells. They stain faintly with hae- raise awareness of this disease among physicians. matoxylin and eosin, but may require Gram’s stain or Giemsa to be seen. Although no bacilli were seen in References our specimen with special stains. 1. Kim NR, Han J, Kwon TY. Nasal rhinoscleroma in a nonen- demic area: A case report. J Korean Med Sci 2003;18:455-8. Treatment is challenging, as it is difficult to eradi- 2. Bailhache A, Dehesdin D, Francois A, Marie J P, Choussy cate the disease and recurrences are common. K. O.Rhinoscleroma of the sinuses. Rhinology 2008; 46: 338-341. rhinoscleromatis is an intracellular bacterium, hence 3. Stiernberg CM, Clark WD: Rhinoscleroma--a diagnostic Fluoroquinolones are recommended for their excellent challenge. Laryngoscope. 1983; 93(7): 866–70. activity against Gram negative bacilli, intracellular ef- 4. Shaw HJ, Martin H. Rhinoscleroma: A clinical perspective. J [9] ficacy and low toxicity profile . Recommended treat- Laryngol Otol 1961; 75: 1011-39. ment regime includes a combination of ciprofloxacin 5. Hara HJ. Scleroma. Laryngoscope 1957; 67: 695-708. and doxycycline for at least six months. Combination 6. Lubin JR, Jallow SE, Wilson WR, Grove AS, Albert DM. therapy is preferred due to the high relapse rates of Rhinoscleroma with exophthalmos: a case report. British the disease. Surgical debridement could also be con- Journal of Ophthalmology. 1981; 65(1):14-17. sidered if there is significant airway obstruction or 7. Zhong Q, Guo W, Chen X, Ni X, Fang J, Huang Z, Zhang cosmetic deformity S. Rhinoscleroma: a retrospective study of pathologic and clinical features. J Otolaryngol Head Neck Surg 2011; 40: 167- Conclusion 74. Rhinoscleroma is a diagnostic and therapeutic chal- 8. Mikulicz J. Ueber das Rhinosclerom. Arch Klin Chir 1877; 20: lenge due to its chronic course, need for prolonged 485-8. treatment and repeated relapses. Early diagnosis and 9. de Pontual L, Ovetchkine P, Rodriguez D, et al. treatment of RS are necessary to avoid progressive and Rhinoscleroma: a French national retrospective study of epidemiological and clinical features. Clin Infect Dis 2008; destructive complications of the disease. Diagnosis is 47:1396-402. based predominantly on detection of Mikulicz cells. Hence, histological confirmation of rhinoscleroma is 9

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