Case presentation

65 Years old female patient named Zakia Ezz EldeinComes 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 forcepsNasal examination was free

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)

Then post operative antibiotics adminstration combined with steroid tappering dose

Post operative CT was done showing no air way obstruction

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

• 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

Diagnosis is recurrent laryngoscleroma

laryngoscleroma

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

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:FrequencyIt 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

RacePatients of all races can be affected.Sexscleroma tends to affect females somewhat

more than it does males.AgeTypically, rhinoscleroma appears in patients

aged 10-30 years.

Pathophysiologyscleroma 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.

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

Clinical stages:There are four stages of this disease: catarrhal, atrophic,

granulomatousand cicatricial.1. Catarrhal: Foul smelling purulent nasal discharge for

weeksto months.2. Atrophic stage: This stage presents with crusting, whichresembles 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 andupper lip gives “woody” feel. These painless nodules arenon-ulcerative and can be found in pharynx, larynx, tracheaand bronchi.4. Cicatricial stage: Fibrosis leads to stenosis of nares,

distortionof upper lip and adhesions in the nose, nasopharynx,oropharynx and larynx. The subglottic stenosis manifestsas respiratory distress.

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)

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 crusts2-fibrosis and subglottic stenosis (webbing)Trachea showing mulitlevel webs & scattered

granulations

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

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

Imaging Studies (MRI)In the hypertrophic stage of rhinoscleroma, both

T1- and T2-weighted images show characteristic mild-to-marked high signal intensity.

Treatment: Antibiotic agents Tetracycline is the drug of choice (2 g/day) for 4–6 weeks Other medication1. 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

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