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Table of Contents    
Year : 2016  |  Volume : 64  |  Issue : 3  |  Page : 549-552

Rhinoscleroma with intracranial extension: A rare case

1 Department of Neurosurgery, Bangur Institute of Neurosciences, Kolkata, West Bengal, India
2 Department of ENT, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

Date of Web Publication3-May-2016

Correspondence Address:
Aditya Kesharwani
Department of Neurosurgery, Bangur Institute of Neurosciences, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.181553

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How to cite this article:
Ghosh SN, Kesharwani A, Chaudhuri S, Ghosh D, Sengupta A. Rhinoscleroma with intracranial extension: A rare case. Neurol India 2016;64:549-52

How to cite this URL:
Ghosh SN, Kesharwani A, Chaudhuri S, Ghosh D, Sengupta A. Rhinoscleroma with intracranial extension: A rare case. Neurol India [serial online] 2016 [cited 2022 Aug 19];64:549-52. Available from: https://www.neurologyindia.com/text.asp?2016/64/3/549/181553


A 48-year-old male patient came to our hospital, with the chief compliant of proptosis of the right eye for 3 years, headache for 2 years, and loss of vision in the right eye for 1 year. The patient had a history of left orbital exentration and left maxillectomy for nasal obstruction and left eye proptosis in 2007, and right orbital decompression by a lateral orbitotomy in 2010 in another college by an ENT Surgeon. The histopathology was suggestive of rhinoscleroma. One year after the operation, the patient again developed proptosis of the right eye followed by headache and loss of vision in the right eye.

On examination, the patient was conscious, cooperative, well-oriented to time, place, and person. His Glasgow Coma Scale was E4V5M6, right eye proptosis was present, and the eye was chemosed. The perception of light and projection of rays were absent in the right eye, and corneal ulceration was present [Figure 1]. Routine blood investigations were within normal limits. Computed tomography (CT) scan (December 20, 2014) of the paranasal sinuses and orbit showed an enhancing lesion involving the right paranasal sinus with extension to the skull base, orbits, and anterior cranial fossa [Figure 2]. The magnetic resonance imaging scan (December 27, 2014) showed a large retroocular enhancing lesion in the right orbit with posterior extension of the lesion in the basifrontal region bilaterally [Figure 3]. The patient was started on tetracycline and antiepileptics. This was followed by combined surgery (January 10, 2015) by the Neurosurgical unit and the ENT surgeons. In the first step, the patient underwent a right maxillectomy and removal of the mass from the maxillary sinus and ethmoid sinus along with right orbital extentration. In the second step, for intracranial extension, a bicoronal incision was made and bifrontal craniotomy and excision of the lesion via a subfrontal approach was done [Figure 4] and [Figure 5]. The patient improved postoperatively and his post-surgery hospital stay was uneventful. His histopathology showed a multinodular and multilobulated encapsulated tumor composed of extensive lymphohistocytic proliferation with foamy macrophages, plasma cells (Russel's bodies), and numerous Mikulicz's cells. The diagnosis was consistent with rhinoscleroma [Figure 6]. The follow-up CT scan on February 07, 2015 confirmed complete removal of the intracranial portion of the lesion with no evidence of residual lesion or recurrence of the lesion [Figure 7] and [Figure 8].
Figure 1: Preoperative appearance of the patient

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Figure 2: Computed tomography scan showing an enchancing lesion involving the right paranasal sinus with extension to the skull base, orbits, and anterior cranial fossa

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Figure 3: Magnetic resonance imaging scan showing a large retro-ocular mass with extension into the base of skull

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Figure 4: Intraoperative picture of a patient undergoing a right maxillectomy and removal of the mass from the maxillary and ethmoid sinuses along with right orbital extentration

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Figure 5: Intraoperative picture showing the intracranial portion of the mass

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Figure 6: Multinodular and multilobulated encapsulated tumor nodule composed of extensive lympho- - histocytic proliferation with foamy macrophages, plasma cells (Russel's bodies), and numerous Mikulicz's cells

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Figure 7: Postoperative contrast computed tomography scan of the patient showed near total removal of the intracranial mass

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Figure 8: Postoperative appearance of the patient

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Rhinoscleroma is a chronic granulomatous disease affecting the upper respiratory airway. The causative organism is Klebsiella rhinoscleromatis. Rhinoscleroma is rarely seen in the orbit, middle ear, and rarely involves the cervical lymph nodes. The disease evolves through three stages: (1) Catarrhal stage: This is characterized by foul smelling purulent nasal discharge and crusting resembling atrophic rhinitis. (2) Granulomatous stage: In this stage, painless and nonulcerative granulomatous nodules form in the nasal mucosa. There is also subdermal infiltration of the external nose and upper lip giving a “woody” feel. (3) Cicatricial stage: This causes stenosis of the nares, distortion of the upper lip, and adhesions in the nose, nasopharynx, and oropharynx.

The lesion usually starts as small granulomas on the nasal septum or lower turbinates, which eventually coalesce to form obstructing masses in the air passages of the nose, pharynx, larynx. It may extend downwards to the trachea and bronchi, often impairing swallowing and requiring a tracheostomy. Infiltration upwards through the sinuses and the lacrimal apparatus is unusual but has being reported with increasing frequency.[1] Relentless progression of the process, while it does not invade the bone itself, causes pressure atrophy of facial bones and may invade the base of the skull and proceed to the orbits, causing serious complications such as meningitis and blindness by pressure on the optic nerve. Infection and hemorrhage are usually the cause of death in fatal cases of this sclerotic process.

The patient in our case had presented with a lesion in the nose with involvement of the paranasal sinuses and both orbits with extension to the base of the skull. Invasion into the orbit and skull base is extremely rare and only three cases exist in literature with intracranial involvement. Intracranial involvement was first reported by Bahri et al., who found that scleroma may invade the brain through the cribriform plate and produce a tumor-like lesion at the base of the brain.[2] de Pontual et al., reported a case that showed intracranial extension of the rhinoscleroma into the anterior cranial cavity via destruction of the posterior bony wall of the left sphenoidal sinus with thickening of the left cavernous sinus and infarction in the territory of the left middle cerebral artery, with vascular occlusion of the M2 segment.[3] Hara published his observations on seven cases of rhinoscleroma in a single family over 10 years, and in another case who developed intracranial complications after 5 years.[4]

We report this case of a rhinoscleroma with intracranial and orbital extension. This entity is very rare. The histopathology of the specimen showed the characteristic inflammatory infiltrate and Mikulicz cells. The Mikulicz cells are characteristic but not pathognomonic of the presence of a rhinoscleroma. They are also seen in leprosy and bubonic plague and are thought to represent the cells' nonspecific response to toxins produced by the causative organism. A large number of the rhinoscleroma bacillus, K. rhinoscleromatis, can sometimes be found within the Mikulicz cells. They stain faintly with hematoxylin and eosin, but may require Gram's stain or Giemsa stain to be seen. The differential diagnosis of the lesion in this area could be an esthesioneuroblastoma that also presents as a nasal mass with intracranial extension. Amongst the other malignant nasal cavity tumors, 80% include squamous cell carcinomas, which can extend into the orbit as well as intracranially and present in a similar manner; however, in literature, a case of nasal dermoid with intracranial extension and sinonasal sarcoidosis with intracranial extension have also been reported.[5],[6]

The goals of pharmacotherapy are to eradicate the infection, reduce morbidity, and prevent complications. Antibiotics are the basis of treatment and tetracycline is the drug of choice. Other antibiotics including ciprofloxacin, rifampicin, cephalosporins, and trimethoprim-sulfamethoxazole have also been used earlier. The antibiotics should be continued for several months (3–6 months) until the biopsy and culture results are negative.

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There are no conflicts of interest.

  References Top

Shaw HJ, Martin H. Rhinoscleroma – A clinical perspective. J Laryngol Otol 1961;75:1011-39.  Back to cited text no. 1
Bahri HC, Bassi NK, Rohatgi MS. Scleroma with intracranial extension. Ann Otol Rhinol Laryngol 1972;81:856-9.  Back to cited text no. 2
de Pontual L, Ovetchkine P, Rodriguez D, Grant A, Puel A, Bustamante J, et al. Rhinoscleroma: A French national retrospective study of epidemiological and clinical features. Clin Infect Dis 2008; 47:1396-402.  Back to cited text no. 3
Hara HJ. Rhinoscleroma: Observations on seven cases in one family for 10 years and another case which developed intracranial complications after five years. Trans Am Laryngol Rhinol Otol Soc 1957;59:326-43.  Back to cited text no. 4
Meher R, Singh I, Aggarwal S. Nasal dermoid with intracranial extension. J Postgrad Med 2005;51:39-40.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
Dessouky OY. Isolated sinonasal sarcoidosis with intracranial extension: Case report. Acta Otorhinolaryngol Ital 2008;28:306-8.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]

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2 Rhinoscleroma Masquerading as a Nasopharyngeal Malignancy
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