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A CASE OF SECONDARY ATROPHIC RHINITIS WITH HANSEN’S DISEASE Nowsheen Hamdani1, M. Vijaya Kumar2

1Postgraduate Scholar, Department of Otolaryngology, Shadan Institute of Medical Sciences (SIMS), Hyderabad. 2Professor and Head, Department of ENT, Shadan Institute of Medical Sciences (SIMS), Hyderabad.

HOW TO CITE THIS ARTICLE: Hamdani N, Vijaya Kumar M. A case of secondary atrophic rhinitis with Hansen’s disease. J. Evid. Based Med. Healthc. 2018; 5(20), 1589-1591. DOI: 10.18410/jebmh/2018/334

PRESENTATION OF THE CASE significant number of cases are diagnosed every year, so it Atrophic rhinitis is an uncommon disorder in modern is important to stay well aware about the disease.10,11 This societies and its incidence varies from 0.3-7.8% of the clinical report of a case of secondary atrophic rhinitis with population.1 It can be classified as primary atrophic rhinitis Hansen’s disease is aimed at showing the nasal finding in which arises de novo or secondary atrophic rhinitis which along with its treatment and reconstruction of the may occur as a sequela of granulomatous diseases such as nose. leprosy, , etc., comprises only 1% of the cases.2 Thus In our experience, a 34-year-old male patient came to such cases are of clinical importance and rarity. Atrophic ENT outpatient department of SIMS presented with atrophic rhinitis (AR) is a debilitating chronic nasal mucosal disease rhinitis, a small septal perforation and saddle nose deformity of unknown aetiology. The condition is characterized by due to Hansen’s disease (secondary atrophic rhinitis), which progressive nasal mucosal atrophy, progressive atrophy of was diagnosed and treated, followed by nasal reconstruction the underlying bone of the turbinates, abnormal widening via open septorhinoplasty approach using an autologous (roomy) / patency of the nasal cavities (with paradoxical conchal cartilage graft with columellar reconstruction, congestion) and viscid secretions and dried crusts leading to yielding a successful postoperative result. a characteristic fetor (ozaena).3,4,5 On the basis of causes AR can be classified as; Primary Atrophic rhinitis which has DIFFERENTIAL DIAGNOSIS decreased markedly in incidence (0.3-1%) and the aetiology Sarcoidosis, , Wegener’s granulomatosis, is unknown2,3,6 and secondary atrophic rhinitis is mostly Lupus erythematosus, Long-standing purulent sinusitis, common in developed countries. The most common cause is Radiotherapy of the nose, Empty nose syndrome and sinus surgery, it alone comprises of 90% of secondary Infectious diseases like TB and syphilis. atrophic rhinitis, common procedures include partial and total turbinectomy (80%), without turbinectomy (10%) and CLINICAL DIAGNOSIS partial maxillectomy (10%), followed by radiation (2.5%), In this case, a 34-year-old male patient, generator mechanic trauma (1%) and granulomatous or infectious diseases by occupation with chief complaints of excessive unilateral (1%).2,7 The malady of “empty nose syndrome is associated left sided nasal crusting since 5 years, altered smell with extensive turbinate surgeries causing secondary sensation since 4 years, excessive bilateral crusting and Atrophic Rhinitis”.8 Little is known about this secondary form depression over the dorsum of the nose since 1 year, tingling and it remains incompletely characterized. It has occurred in sensation over the toes started 4 months back and association with chronic granulomatous diseases of the nose thickening and tingling sensation over bilateral pinna was including leprosy, sarcoidosis, rhinoscleroma, Wegener’s observed one month back. Bilateral crusting started 1 year granulomatosis and infectious diseases like TB and syphilis. back which required cleaning morning and evening, it was The onset after treatment with antiangiogenic drug accompanied by nasal obstruction which was relieved on underlines the role of the microvasculature in the clearing of crust, there is no history of bleeding. No past pathogenesis.2,9 While these causes were once common, history of similar complaints. No history of , now only comprise of 1-2% cases, thus rare.2 Secondary diabetes mellitus, Jaundice, Asthma or Allergy. Patient is atrophic rhinitis is typically seen in older population. using Betnovate N cream at night in both nostrils. History of Hansen’s disease (Leprosy) is a chronic granulomatous tobacco chewing and pan chewing since 3 yrs. No history of disease caused by Mycobacterium leprae. Leprosy has been similar complains in the family. On general examination; PR: officially eliminated in India since 31st December 2005, yet 80/min, BP: 110/80, Temp: 98.4F. No anaemia, no jaundice. Hypopigmented patch of size 5x3 cms over the right thigh. Financial or Other, Competing Interest: None. Ulnar nerve thickening felt over the left elbow. On Local Submission 17-04-2018, Peer Review 24-04-2018, Acceptance 02-05-2018, Published 14-05-2018. examination of nose, External nose skin appears normal, Corresponding Author: depression over the dorsum of the nose is seen (Figure 1). Dr. Nowsheen Hamdani, Anterior rhinoscopy shows nasal cavity is covered with Postgraduate Scholar, Department of Otolaryngology, Shadan Institute of Medical Sciences (SIMS), greenish yellow crusts, both nasal cavities appear roomy and Hyderabad. were foul smelling, atrophy of inferior turbinates seen, small E-mail: [email protected] septal perforation was seen (Figure 2A). Posterior DOI: 10.18410/jebmh/2018/334 rhinoscopy shows crusts in nasopharynx. On examining oral cavity and pharynx, teeth are tobacco stained, oral cavity,

tongue, floor of the mouth, hard palate and soft palate

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Jebmh.com Case Report appears normal. Post nasal drip with crusting is seen in the implantation of a dorsal onlay graft. An absent anterior nasal posterior nasal wall (Figure 2b). Ear examination, inspection spine was reconstructed by implantation of a caudally shows bilateral pinna thickened and shiny in appearance, extended columellar structure. An autologous costal and post and pre-auricular areas are normal. auricular cartilage was exclusively used. Wound infection, On palpation, bilateral pinna thickened and rubbery in extrusion or warping of implants was not observed in any consistency. 1 nodule of size 0.5x1 cm felt over the helix of patient. Functional and aesthetic improvement was left pinna, it is mobile adherent to the skin but not to the observed (15/17) patients. The rate of implant resorption underlying cartilage. Sensation over the pinna is reduced. was dependant on the implant site. Least resorption was DNE shows bilateral crusting, small septal perforation and observed for dorsal onlay grafts (4/17). Moderate resorption atrophied turbinates (Figure 2C). CT Scan shows atrophy of was observed for columellar structure and shield grafts inferior and middle turbinates, mucosal thickening of (7/17). In general, conchal cartilage grafts were associated maxillary and ethmoid sinuses (Figure 2D). In view of with less resorption than costal cartilage grafts overall, leonine facies and nodules the patient was advised Slit Skin advantages are dominating so that authors advocate Smear both ear lobules. Z N stain shows stout bacilli singly reconstruction of saddle nose deformity in leprosy using arranged intracellularly in cigar bundle appearance (globi), autologous cartilage grafts.13,18 Septal or Conchal cartilage with bacteriological index 4+ (Ridley Jopling) suggestive of is easily available, causes minor donor site morbidity, lepromatous leprosy (Figure 2E). suffices to compensate most of simple and complex saddle Patient was treated with Rifampicin 600mg once a nose deformities. Some patients may require more graft month, Dapsone 100mg OD, Clofazimine 300mg once a material like costal cartilage (5%) to substitute for the major month 50mg OD for 18 months and then remained quiescent structural losses.19,20,21 Also bone grafts like autologous iliac for 1 year after which nasal reconstruction surgery for the crest, calvarian bone, have been used but the rate of saddle nose deformity by augmentation (open) complications such as wound infection and graft resorption using autologous auricular cartilaginous graft and along with was 50%.21,22 This case report reinforces the importance of columellar reconstruction was done (Figure 3). Post- an otolaryngologist for early diagnosis and multi-disciplinary operative recovery was satisfactory and good cosmetic result evaluation of patients with leprosy. and functional improvement was achieved (Figure 3).

DISCUSSION OF MANAGEMENT In Leprosy (Hansen’s disease), the nose is an important portal of entry for this bacterium and is therefore frequently affected.10 Depending upon the host immune response, nasal symptoms of leprosy may include epistaxis, gross nasal deformity and destruction. Patient may complain of anosmia and nasal obstruction. On nasal endoscopy friable granulomatous intranasal lesion involving the septum with associated crusting. In the early stage, yellowish discolouration of the mucosa, nodules or pale plaques may be visualized involving the septum and inferior turbinates. As the disease progresses the mucosa becomes thickened resulting in purulent nasal discharge that contains a high Figure 1 concentration of the organisms. This disease has a predilection for nasal invasion resulting in decreased nasal sensation. Late stage leprosy is characterized by dryness, crusting and septal cartilage destruction. This disease is very slowly progressive but once the diagnosis is confirmed, broad spectrum antibiotic treatment should be initiated to prevent disfigurement.12 Sinonasal symptoms can occur and can be refractory to systemic treatment.13,14,15 The clinical spectrum ranges from localized involvement of the external nose and extensive destruction of the septal cartilage and bone resulting in nasal septal perforation and saddle nose deformity.13,16,17,18 Principally, systemic treatment should precede any local surgical procedure.13 Menger and coworkers reconstructed 24 saddle nose deformities of Figure 2 different severity caused by leprosy.13,18 In all cases external approach was used. A inverted V procedure was used in 4 patients in order to lengthen a retracted columella. The nasal septum and upper lateral cartilages were reconstructed by

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[10] Srinivasan S, Nehru VI, Bapuraj JR, et al. CT findings in involvement of paranasal sinuses by lepromatous leprosy. Br J Radiol 1999;72(855):271-273. [11] National leprosy eradication programme. M/O H & FW, Govt. of India. Annual report 2011-2012. [12] Gupta A, Seiden AM. Nasal leprosy: a case study. Otolaryngol Head and Neck Surgery 2003;129(5):608- 610. [13] Sachse F, Stoll W. Nasal surgery in patients with systemic disorders. GMS Curr Top Otorhinolaryngol Head Neck Surg 2010;9:Doc02. [14] Gupta SC, Tiwari M, Singh KG. Radiological and Figure 3 atroscopic study of maxillary antrum in multibacillary leprosy patients. Indian J Lepr 2004;76(4):305-309. REFERENCES [15] Kiris A, Karlidag T, Kocakoc E, et al. Paranasal sinus [1] Sreedharan SS, Prasad V, Shenoy VS, et al. A clinical computed tomography findings in patients treated for study on Atrophic rhinitis, its management and surgical lepromatous leprosy. J Laryngol Otol 2007;121(1):15- outcomes. General Med 2015;3:1. 18. [2] Moore EJ, Kern EB. Atrophic rhinitis: a review of 242 [16] Inamadar AC, Palit A, Kulkarni NH, et al. Nodulo- cases. Am J Rhinol 2001;15(6):355-361. ulcerative lesions over the nose. Indian J Dermatol [3] Dutt SN1, Kameswaran M. The aetiology and Venereol Leprol 2004;70(3):197-198. management of atrophic rhinitis. J Laryngol Otol [17] Torres-Larrosa MT, Perez-Perez LJ, Quitana Ginestar 2005;119(11):843-852. MV, et al. Nasal leprosy: impact of multitherapy in the [4] Zohar Y, Talmi YP, Strauss M, et al. Ozaena revisited. morphology and physiology of the nose. Acta J Otolaryngol 1990;19:345-349. Otorrinolaringol Esp 2007;58(5):182-186. [5] Weir N, Golding-Wood DG. Infective rhinitis and [18] Menger DJ, Fokkens WJ, Lohuis PJ, et al. sinusitis. In: Mackay IS, Bull TR, eds. Scott-Brown’s Reconstructive surgery of the leprosy nose: a new otolaryngology. 6th edn. Oxford: Butterworth- approach. J Plast Reconstr Aesthet Surg Heinemann 1997;4: p. 4,8,26-28. 2007;60(2):152-162. [6] Schoss P. Diagnosis and control of progressive atrophic [19] Riechelmann H, Rettinger G. Three step reconstruction rhinitis. ALTEX 1994;11(5):355-361. of complex saddle nose deformities. Arch Otolaryngol [7] Alan C, Mathew WR, Francis BQ, et al. Atrophic rhinitis. Head Neck Surg 2004;130(3):334-338. UTMB, Dept of Otolaryngology March 30, 2005. [20] Toriumi DM. Autogenous grafts are worth extra time. [8] Wang Y, Liu T, Qu Y, et al. Empty nose syndrome. Arch Otolaryngol Head Neck Surg 2000;126(4):562- Zhonghua Er Bi Yan Hou Ke Za Zhi 2001;36(3):203- 564. 205. [21] Adamson PA. Grafts in rhinoplasty: autogenous grafts [9] Prutiere-Escabasse V, Escudier E, Balheda R, et al. are superior to alloplastic. Arch Otolaryngol Head Neck Rhinitis and expistaxis in patients treated by Surg 2000;126(4):561-562. antiangiogenic therapy. Invst New Drugs [22] Schwarz RJ, Macdonald M. A rational approach to nasal 2009;27:285-286. reconstruction in leprosy. Plastic Reconstr Surg 2004;114(4):876-882.

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