Rhinoscleroma

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1. R HIN O SCLE R OM A R AA F I U L B ASH E E R

2. INTRODUCTION INCIDENCE ETIOPATHOGENESIS CLINICAL FEATURES DIAGNOSIS TREATMENT TABLE OF CONTENTS OVERVIEW

3. INTRODUCTION COINED BY:- VON HEBRA IN 1870 DEFINITION Respiratory scleroma or rhinoscleroma is a progressive granulomatous disease commencing in the nose and later extending into the nasopharynx and oropharynx, the larynx and sometimes the trachea and bronchi. Respiratory scleroma or rhinoscleroma is a progressive granulomatous disease commencing in the nose and later extending into the nasopharynx and oropharynx, the larynx and sometimes the trachea and bronchi.

4. INCIDENCE

5. INCIDENCE (INDIA)

6. ETIOPATHOGENESIS ETIOLOGY Klebsiella rhinoscleromatis Frisch bacillus Gram negative Intracellular

7. RISK FACTORS MULTIFACTORIAL LOW SOCIO ECONOMIC STATUS POOR DOMESTIC HYGIENE OVERCROWDING 1 2 3 4

8. ROUTE OF ENTRY DROPLET NUCLEI

9. ROUTE OF ENTRY DROPLET NUCLEI ENTRY INTO MUCOSA OF OROPHARYNX & LARYNGOPHARYNX

10. ROUTE OF ENTRY DROPLET NUCLEI ENTRY INTO MUCOSA OF OROPHARYNX & LARYNGOPHARYNX INTRACELLULAR SURVIVAL ENGULFED BY MACROPHAGES MUCKLICZ CELLS MUCOPOLYSACCHARIDES SUPPORT INTRACELLULAR SURVIVAL

11. C LINI C A L F E AT U RE S SUBDERMAL INFILTRATION PROGRESSES THROUGH 4 STAGES - CATARRHAL STAGE ATROPIC STAGE GRANULOMATOUS STAGE CICATRICIAL STAGE STAGE-I CATARRHAL STAGE FOUL SMELLING DISCHARGE (CARPENTER’S GLUE) DURATION-WEEKS TO MONTHS STAGE-II ATROPIC STAGE FEATURES OF ATROPHIC RHINITIS CRUST FORMATION CRUST STAGE-III GRANULOMATOUS/PROLIFERATIVE/NODULAR STAGE NODULES PAINLESS & NON-ULCERATIVE INITIALLY- BLUISH-RED & RUBBERY LATER- PALE & INDURATED TAPIR’S NOSE SHAPE AND CONTOUR OF NOSE CHANGES WOODY FEEL ` STAGE-IV CICATRICIAL STAGE ADHESIONS & STENOSIS MIGHT OCCUR TO DISTORT NORMAL ANATOMY MC SITE AFFECTED IN NOSE ANT. PART OF NASAL SEPTUM COMMONEST PRESENTATION NASAL OBSTRUCTION MC SITE AFFECTED IN LARYNX SUBGLOTTIS GOTHIC SIGN DUE TO FIBROSIS PHARYNGEAL WALL IS PULLED UP & IT RESEMBLES TOP OF A CHURCH

12. KEY POINTS STAGES CLINICAL FEATURES CATARRHAL CARPENTERS GLUE ATROPHIC CRUST FORMATION NODULAR HEBRA NOSE CICATRICIAL STENOSIS+FIBROSIS

13. INVESTIGATIONS SUBMUCOSA INFILTRATED WITH PLASMA CELLS , LYMPHOCYTES , EOSINOPHILS , MIKULICZ CELLS AND RUSSEL BODIES MIKCULICZ CELLS (DIAGNOSTIC) FOAM CELLS CONTAIN CAUSATIVE ORGANISM RUSSEL BODIES (DIAGNOSTIC) FOUND IN PLASMA CELLS ACCUMULATION OF Ig’s BIOPSY

14. D I A G N O S I S BASED ON REACTION OF PATIENT’S SERUM WITH SUSPENSION OF Klebsiella rhinoscleromatis . HIGH TITRES OF ANTIBODIES AGAINST K. rhinoscleromatis INDICATES INTACT HUMORAL IMMUNITY. COMPLEMENT FIXATION TEST ( LEVIN TEST )

15. D I A G N O S I S INTRACRANIAL EXTENSION OF RHINOSCLEROMA INTO ANTERIOR CRANIAL CAVITY VIA DESTRUCTION OF POSTERIOR BONY WALL OF LEFT SPHENOIDAL SINUS CT SCAN

16. D I A G N O S I S T1-WEIGHTED NASAL MASS EXTENDING INTO NASOPHARYNX MRI

17. D I A G N O S I S MUCOID DOME SHAPED STICKY PINK COLOURED LACTOSE FERMENTING COLONIES CULTURE ( MacConkey AGAR )

18. D I A G N O S I S PAS GIEMSA WARTHIN STARRY SILVER STAINS PAS STAIN INCLUSION BODIES CONTAIN CAUSATIVE ORGANISM

19. COMPLICATIONS EXTERNAL NOSE DEFORMITY VESTIBULAR STENOSIS CICATRIZATION OF SOFT PALATE NASAL REGURGITATION TRACHEAL STENOSIS

20. DISEASE FOLLOWS A PROTRACTED BUT USUALLY SELF LIMITING COURSE ENDING IN CICATRIZING STAGE ORGANISM CAN BE EXTREMELY DIFFICULT TO ERRADICATE BY ANTIMICROBIALS ONCE DIAGNOSIS IS CONFIRMED THE TREATMENT SHOULD BE INTENSE AND PROLONGED LINES OF TREATMENT ANTIBIOTICS STEROIDS RADIOTHERAPY SURGERY TREATMENT MEDICAL

21. DRUGS STREPTOMYCIN I.M 1GM FOR 4-6 WEEKS TETRACYCLINE 500MG Q.I.D RIFAMPICIN 400MG FOR 6 WEEKS ORALLY NASAL INSTILLATION NASAL INFILTRATIOMN COTRIMOXAZOLE+CIPROFLOXACIN ACRIFLAVIN 2% LOCALLY 8 WEEKS BACTERICIDAL ANTIBIOTICS LARGE DOSES GIVEN FOR MIN. DURATION OF 4-6 WEEKS CONTINUED TILL 2 CONSECUTIVE CULTURES FROM BIOPSY MATERIAL ARE PROVEN NEGATIVE

22. CONTENT CARBOLIC ACID(0.2ML) GLACIAL ACETIC ACID(0.2ML) GLYCERIN(0.4ML) D/W(10ML) ROUTE INJECTED LOCALLY MECHANISM CHEMICAL NECROSIS OF GRANULOMA 8-10 INJ. LEADS TO COMPLETE REGRESSION OF GRANULOMA AND RESTORATION OF NORMAL NASAL PATENCY Kalisa regimen

23. DOSE 3000-3500 GY DURATION 3 WEEKS MECHANISM DESTROYS SCLEROMA BY RADIATION CURRENTLY NOT REQUIRED IRRADIATION

24. COMBINED WITH ANTIBIOTICS TO REDUCE FIBROSIS INTRALESIONAL STEROIDS

25. REQUIRED IN 4 TH STAGE OF FIBROSIS AND STENOSIS PLASTIC RECONSTRUCTIVE SURGERY CARRIED OUT BY LASER AND NASAL ENDOSCOPY SILASTIC STENT FACILITATES RE-EPITHELISATION USES ESTABLISHES AIRWAY CORRECT THE NASAL DEFORMITY SURGERY

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