case presentaion of laryngoscleroma by dr/ AMR KHOLIEF ass. lecturer E.N.T department alex. uni. egypt

2.  65 Years old female patient named Zakia Ezz Eldein
 Comes to emergency to E.N.T department with severe stridor
with history of laryngoscleroma
 patient is intubated and admitted to our ICU unit and prepared
for DL under general anathesia planned for debulking of air way
obstruction by microdebrider without tracheostomy
 Medical history patient is diabetic & hypertensive
 O/E ( intra operative)
 Revealed bulge of both ventricular bands
 Debulking was done for this bulge under general anathesia
using microdebrider and also by conventional laryngeal forceps
 Nasal examination was free

4.  After recovery patient extubated without stridor
 Biobsy was sent for histopathalogical examination&
revealed hyperplastic acanthotic squamous epithelium
with moderate chronic non-specific inflamation
 C&S  revealed klebsilla ( g –ve cocco bacilli)

5.  Then post operative antibiotics adminstration
combined with steroid tappering dose
 Post operative CT was done showing no air way
obstruction

8. The follow up laryngoscopy was done :
It show greenish crusts only in larynx without subglottic
stenosis

10. • Recently patient come with mild dyspnea and
hoarsness of voice after years
• Vediostropscopy was done and showing only mild
odema of the larynx and greenish crusts in subglottic
region and over the true cords

12. Diagnosis is recurrent laryngoscleroma

14. History:
Von Hebra termed the rhinoscleroma in 1870.
Rhinoscleroma is endemic in several parts of the world.
In India, northern parts are affected more than the
southern states

15. Definition:
is a chronic granulomatous condition of the nose and other
structures of the upper respiratory tract. Rhinoscleroma is a
result of infection by the bacterium Klebsiella
rhinoscleromatis.
Epidemiology:
 Frequency
It is endemic to regions of Africa (Egypt, tropical areas), South
east Asia, Mexico, Central and South America, and Central and
Eastern Europe, but it has been infrequent in the United States

16.  Race
Patients of all races can be affected.
 Sex
scleroma tends to affect females somewhat more than it
does males.
 Age
Typically, rhinoscleroma appears in patients aged 10-30
years.

17. Pathophysiology
scleroma is transmitted by means of the direct
inhalation of droplets or contaminated material. The
disease probably begins in areas of epithelial transition
such as the vestibule of the nose, the subglottic area of
the larynx, or the area between the nasopharynx and
oropharynx.
scleroma usually affects the nasal cavity, but lesions
associated with rhinoscleroma may also affect the
larynx; nasopharynx; oral cavity; paranasal sinuses; or
soft tissues of the lips, nose, trachea, and bronchi.

18. The CD4/CD8 cell ratio in the lesion is altered with
decreased levels of CD4 lymphocytes; this change
possibly induces a diminished T-cell response.
Macrophages are not fully activated.
Mucopolysaccharides in the bacterial capsule probably
contribute to the inhibition of phagocytosis

19. Clinical stages:
There are four stages of this disease: catarrhal, atrophic, granulomatous
and cicatricial.
1. Catarrhal: Foul smelling purulent nasal discharge for weeks
to months.
2. Atrophic stage: This stage presents with crusting, which
resembles atrophic rhinitis.
3. Granulomatous stage: Multiple granulomatous nodules,
which enlarge and coalesce, are seen in nasal mucosa.
Subdermal infiltration of lower part of external nose and
upper lip gives “woody” feel. These painless nodules are
non-ulcerative and can be found in pharynx, larynx, trachea
and bronchi.
4. Cicatricial stage: Fibrosis leads to stenosis of nares, distortion
of upper lip and adhesions in the nose, nasopharynx,
oropharynx and larynx. The subglottic stenosis manifests
as respiratory distress.

20. Clinically:
Symptoms
 Nasal obstruction (most common complaint)
 Rhinorrhea
 Epistaxis
 Dysphagia
 Nasal deformity
 Anesthesia of the soft palate
 Difficulty breathing that progresses to stridor( biphasic )
 Dysphonia
 Anosmia
 Cogh and expectoration of (greenish crusts)

21. Signs:
Nasal exam The initial nodule is often intra-nasal and
small in size. Rarely, if neglected, it can grow into an
exophytic giant tumor, which may obstruct the entire
respiratory tract.
Larynx  showing
1-pale pinkish smooth swelling on both sides of subglottis
covered by greenish crusts
2-fibrosis and subglottic stenosis (webbing)
Trachea showing mulitlevel webs & scattered granulations

22.  Laboratory Studies:
o A positive result with culturing in MacConkey agar is
diagnostic of rhinoscleroma. However, culture
results are positive in only 50-60% of patients
o Mikulicz cells are large foam cells with a central
nucleus and vacuolated cytoplasm containing
causative Bacilli .Russell bodies are homogenous
eosinophilic inclusion bodies found in the plasma
cells

23.  Imaging Studies (CT findings)
1. The subglottic area is involved in laryngeal and
tracheal scleroma. The lesions primarily cause
concentric irregular narrowing of the airway. In the
trachea, cryptlike irregularities are diagnostic of
scleroma
2. The lesions are characteristically homogeneous and
nonenhancing, and they have well defined edges
3. CT findings in primary nasal and nasopharyngeal
rhinoscleroma include soft-tissue masses of variable
sizes

24.  Imaging Studies (MRI)
In the hypertrophic stage of rhinoscleroma, both T1- and T2-
weighted images show characteristic mild-to-marked high
signal intensity.

25.  Treatment:
 Antibiotic agents Tetracycline is the drug of choice
(2 g/day) for 4–6 weeks
Other medication
1. Ciprofloxacin is a fluoroquinolone with activity
against Pseudomonas species, streptococci,
MRSA, Staphylococcus
2. Rifampin inhibits DNA-dependent bacteria by binding to the
beta subunit of DNA-dependent RNA polymerase, blocking
RNA transcription.
3. Cefixime is a third-generation cephalosporin. It arrests
bacterial cell wall synthesis
N.B Repeat if necessary after 1 month.Treatment is stopped only
when two consecutive cultures are negative

26. Corticosteroid agents : (Prednisone (Deltasone,
Meticorten, Orasone)
They can be combined to reduce fibrosis
Surgical Care:
A. Tracheotomy should be considered in patients with
laryngeal obstruction of the third degree
(granulomatous stage)
B. Extensive granulomatous lesions are treated by
means of open excision by using the laryngofissure
approach
C. Surgery and laser therapy are required to treat airway
compromise and tissue deformity.
D. Bronchoscopy has a role in the initial treatment of
the disease