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RHIN OSCLE R
O M A
R A A F I U L B A S H E E R
• I N T R O D U C T I O N
• I N C I D E N C E
• E T I O PAT H O G E N E
S I S
• C L I N I C A L
F E AT U R E S
• D I A G N O S I S
• T R E AT M E N T
TABLE
OF
CONTENTS
OVERVIEW
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.
INCIDENCE
INCIDENCE
(INDIA)
ETIOPATHOGENESIS
ETIOLOGY
K l e b s i e l l a
r h i n o s c l e r o m a
t i s
• F r i s c h
b a c i l l u s
• G r a m
n e g a t i v e
• I n t r a c e l l u l a r
RISK FACTORS
MULTIFACTORIAL
LOW SOCIO ECONOMIC
STATUS
POOR DOMESTIC HYGIENE
OVERCROWDING
1
2
3
4
ROUTE OF ENTRY
• DROPLET
NUCLEI
ROUTE OF ENTRY
• DROPLET
NUCLEI
ENTRY INTO MUCOSA
• OF OROPHARYNX
&
LARYNGOPHARYN
X
ROUTE OF ENTRY
• DROPLET
NUCLEI
ENTRY INTO MUCOSA
• OF
OROPHARYNX
&
LARYNGOPHAR
YNX
INTRACELLULAR SURVIVAL
• ENGULFED BY
MACROPHAGES
• MUCKLICZ CELLS
• MUCOPOLYSACC
HARIDES
SUPPORT
INTRACELLULAR
SURVIVAL
CLINICAL FEATURES
• SUBDERMAL INFILTRATION
• PROGRESSES THROUGH 4 STAGES-
1. CATARRHAL STAGE
2. ATROPIC STAGE
3. GRANULOMATOUS STAGE
4. CICATRICIAL STAGE
STAGE-I
RPENTER’S
STAGE-II
STAGE-III
STAGE-IV
KEY POINTS
STAGES CLINICAL FEATURES
CATARRHAL CARPENTERS GLUE
ATROPHIC CRUST FORMATION
NODULAR HEBRA NOSE
CICATRICIAL STENOSIS+FIBROSIS
I N V E S T I G A T I O N S
• 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
B I O P S Y
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.
C O M P L E M E N T F I X A T I O N
T E S T ( L E V I N T E S T )
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
C T S C A N
D I A G N O S I S
• T1-WEIGHTED
• NASAL MASS
EXTENDING INTO
NASOPHARYNX
M R I
D I A G N O S I S
• MUCOID
• DOME SHAPED
• STICKY
• PINK COLOURED
• LACTOSE FERMENTING
COLONIES
C U L T U R E ( M a c C o n k e y
A G A R )
D I A G N O S I S
1. PAS
2. GIEMSA
3. WARTHIN STARRY SILVER
S T A I N S
PAS STAIN
• INCLUSION BODIES
• CONTAIN CAUSATIVE ORGANISM
C O M P L I C AT I O N S
1. EXTERNAL NOSE DEFORMITY
2. VESTIBULAR STENOSIS
3. CICATRIZATION OF SOFT PALATE
4. NASAL REGURGITATION
5. TRACHEAL STENOSIS
• 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
1. ANTIBIOTICS
2. STEROIDS
3. RADIOTHERAPY
4. SURGERY
TREATMENT
MEDICAL
• DRUGS
1. STREPTOMYCIN
• I.M 1GM FOR 4-6 WEEKS
2. TETRACYCLINE
• 500MG Q.I.D
3. RIFAMPICIN
• 400MG FOR 6 WEEKS ORALLY
• NASAL INSTILLATION
• NASAL INFILTRATIOMN
4. COTRIMOXAZOLE+CIPROFLOXACIN
5. ACRIFLAVIN
• 2% LOCALLY
• 8 WEEKS
B A C T E R I C I D A L
A N T I B I O T I C S
• LARGE DOSES GIVEN FOR
MIN. DURATION OF 4-6 WEEKS
• CONTINUED TILL 2
CONSECUTIVE CULTURES
FROM BIOPSY MATERIAL ARE
PROVEN NEGATIVE
• CONTENT
1. CARBOLIC ACID(0.2ML)
2. GLACIAL ACETIC ACID(0.2ML)
3. GLYCERIN(0.4ML)
4. D/W(10ML)
• ROUTE
• INJECTED LOCALLY
• MECHANISM
• CHEMICAL NECROSIS OF GRANULOMA
• 8-10 INJ. LEADS TO COMPLETE
REGRESSION OF GRANULOMAAND
RESTORATION OF NORMAL NASAL
PATENCY
K a l i s a r e g i m e n
• DOSE
• 3000-3500 GY
• DURATION
• 3 WEEKS
• MECHANISM
• DESTROYS SCLEROMA BY RADIATION
• CURRENTLY NOT REQUIRED
I R R A D I AT I O N
• COMBINED WITH ANTIBIOTICS TO REDUCE
FIBROSIS
I N T R A L E S I O N A L
S T E R O I D S
• REQUIRED IN 4TH 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
S U R G E R Y
Rhinoscleroma

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Rhinoscleroma

  • 1. RHIN OSCLE R O M A R A A F I U L B A S H E E R
  • 2. • I N T R O D U C T I O N • I N C I D E N C E • E T I O PAT H O G E N E S I S • C L I N I C A L F E AT U R E S • D I A G N O S I S • T R E AT M E N T 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.
  • 6. ETIOPATHOGENESIS ETIOLOGY K l e b s i e l l a r h i n o s c l e r o m a t i s • F r i s c h b a c i l l u s • G r a m n e g a t i v e • I n t r a c e l l u l a r
  • 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 & LARYNGOPHARYN X
  • 10. ROUTE OF ENTRY • DROPLET NUCLEI ENTRY INTO MUCOSA • OF OROPHARYNX & LARYNGOPHAR YNX INTRACELLULAR SURVIVAL • ENGULFED BY MACROPHAGES • MUCKLICZ CELLS • MUCOPOLYSACC HARIDES SUPPORT INTRACELLULAR SURVIVAL
  • 11. CLINICAL FEATURES • SUBDERMAL INFILTRATION • PROGRESSES THROUGH 4 STAGES- 1. CATARRHAL STAGE 2. ATROPIC STAGE 3. GRANULOMATOUS STAGE 4. CICATRICIAL STAGE STAGE-I RPENTER’S STAGE-II STAGE-III STAGE-IV
  • 12. KEY POINTS STAGES CLINICAL FEATURES CATARRHAL CARPENTERS GLUE ATROPHIC CRUST FORMATION NODULAR HEBRA NOSE CICATRICIAL STENOSIS+FIBROSIS
  • 13. I N V E S T I G A T I O N S • 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 B I O P S Y
  • 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. C O M P L E M E N T F I X A T I O N T E S T ( L E V I N T E S T )
  • 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 C T S C A N
  • 16. D I A G N O S I S • T1-WEIGHTED • NASAL MASS EXTENDING INTO NASOPHARYNX M R I
  • 17. D I A G N O S I S • MUCOID • DOME SHAPED • STICKY • PINK COLOURED • LACTOSE FERMENTING COLONIES C U L T U R E ( M a c C o n k e y A G A R )
  • 18. D I A G N O S I S 1. PAS 2. GIEMSA 3. WARTHIN STARRY SILVER S T A I N S PAS STAIN • INCLUSION BODIES • CONTAIN CAUSATIVE ORGANISM
  • 19. C O M P L I C AT I O N S 1. EXTERNAL NOSE DEFORMITY 2. VESTIBULAR STENOSIS 3. CICATRIZATION OF SOFT PALATE 4. NASAL REGURGITATION 5. 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 1. ANTIBIOTICS 2. STEROIDS 3. RADIOTHERAPY 4. SURGERY TREATMENT MEDICAL
  • 21. • DRUGS 1. STREPTOMYCIN • I.M 1GM FOR 4-6 WEEKS 2. TETRACYCLINE • 500MG Q.I.D 3. RIFAMPICIN • 400MG FOR 6 WEEKS ORALLY • NASAL INSTILLATION • NASAL INFILTRATIOMN 4. COTRIMOXAZOLE+CIPROFLOXACIN 5. ACRIFLAVIN • 2% LOCALLY • 8 WEEKS B A C T E R I C I D A L A N T I B I O T I C S • LARGE DOSES GIVEN FOR MIN. DURATION OF 4-6 WEEKS • CONTINUED TILL 2 CONSECUTIVE CULTURES FROM BIOPSY MATERIAL ARE PROVEN NEGATIVE
  • 22. • CONTENT 1. CARBOLIC ACID(0.2ML) 2. GLACIAL ACETIC ACID(0.2ML) 3. GLYCERIN(0.4ML) 4. D/W(10ML) • ROUTE • INJECTED LOCALLY • MECHANISM • CHEMICAL NECROSIS OF GRANULOMA • 8-10 INJ. LEADS TO COMPLETE REGRESSION OF GRANULOMAAND RESTORATION OF NORMAL NASAL PATENCY K a l i s a r e g i m e n
  • 23. • DOSE • 3000-3500 GY • DURATION • 3 WEEKS • MECHANISM • DESTROYS SCLEROMA BY RADIATION • CURRENTLY NOT REQUIRED I R R A D I AT I O N
  • 24. • COMBINED WITH ANTIBIOTICS TO REDUCE FIBROSIS I N T R A L E S I O N A L S T E R O I D S
  • 25. • REQUIRED IN 4TH 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 S U R G E R Y