Atrophic rhinitis (AR) is a chronic nasal disease characterized by the progressive atrophy of the mucosa and underlying bone, leading to the enlargement of nasal cavities and a foul odor (ozena). The condition can be classified into primary and secondary types, with primary AR being rare in the U.S. and most commonly seen in countries like China, Egypt, and India. Secondary AR often results from complications of sinus surgery, radiation, or trauma, as well as diseases like tuberculosis and leprosy. The disease presents with symptoms like anosmia, nasal crusting, congestion, and atrophy of nasal structures, particularly turbinates. Radiographic findings typically show thickening of the paranasal sinuses and bony resorption. Treatment aims to restore nasal hydration and minimize crusting, using both topical and systemic therapies, including saline nasal douches, antibiotics, and surgical options to address nasal volume loss and obstruction.
The condition’s microbiology commonly involves Klebsiella ozenae in primary AR, whereas secondary AR may present with various bacteria such as Staphylococcus aureus and Proteus mirabilis. Management options for AR include both non-surgical and surgical approaches, such as nasal douching, implants, and submucosal injections. Surgical procedures like Young’s operation and volume reduction techniques aim to close or reduce the nasal cavity size to provide symptom relief. These surgeries can be challenging, with risks like recurrence and complications such as nasal stenosis. Non-surgical options, including nasal obturators and silastic implants, offer reversible solutions with less morbidity, although they may cause discomfort and limit cleaning.
In addition to AR, rhinocleroma is another chronic granulomatous disease of the upper respiratory tract, often involving the nasal cavity. It is caused by the bacterium Klebsiella rhinoscleromatis, and it affects individuals in endemic regions like Southeast Asia, Mexico, and Eastern Europe. The disease progresses through three stages: catarrhal (or atrophic), granulomatous, and sclerotic. It primarily causes nasal obstruction, rhinorrhea, and epistaxis, with deformities like the "Hebra nose" in later stages. Treatment for rhinocleroma involves long-term antibiotics, surgical interventions for airway obstruction, and sometimes radiotherapy. Early diagnosis and prolonged therapy are key to managing this condition, as relapses can occur, and without proper treatment, complications like upper airway obstruction and asphyxia may arise.
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ATROPHIC RHINITIS AND RHINOSCLEROMA
Atrophic Rhinitis
Common Terms
Ozena
Dry Rhinitis
Rhinitis Sicca
Open-nose syndrome
HISTORY
Dr. Spencer Watson. Diseases of the nose and its associated cavities. London, 1875.
1) Accidental or Simple Ozoena
“due to the retention of mucous.”
“easily dealt with by the frequent employment of the nasal douche …”
2) Idiopathic or constitutional
“commences in early childhood ... And remains during the early years or throughout the whole adult life.”
“The patient is generally anosmic … and he is, therefore, unaware of the offensive odor of his breath.”
“The nature of the inflammatory process is very probably allied to that of lupus erythematosus of the face.”
3) Syphilitic Ozoena
“the most common form”
“These ulcers may be preceded or followed by caries or necrosis of the bones, and the stench is then more horribly sickening than in any other form of this disgusting malady.”
HISTORY Cont..
Described in 1876 by Dr. Bernhard Fraenkel as a triad of:
Fetor
Crusting
Atrophy of nasal structures
Dr. Francke Bosworth. A Manual of Diseases of the Nose and Throat. 1881.
“the breath is often so penetrating as to render the near presence of the sufferer not only unpleasant but almost unendurable.”
Atrophic Rhinitis
Chronic nasal disease characterized by progressive atrophy of the mucosa and the underlying bone of the turbinates.
Associated with a characteristic foul odour-ozaena
Types-1) Primary
Secondary
2)Type 1(there is endarteritis)- more common
Type 2(there is vasodilation)
Atrophic rhinitis
Primary
History of prior sinus surgery, radiation, granulomatous disease, or nasal trauma are exclusions.
Primary AR is rare in the US
Most cases are reported in China, Egypt, and India
Microbiology of primary AR is almost uniformly Klebsiella ozenae.
Radiographic and clinical features similar to secondary AR.
Atrophic rhinitis
Secondary
Complication of sinus surgery (89%)
Complication of radiation (2.5%)
Following nasal trauma (1%)
Sequela of granulomatous diseases (1%)
Sarcoid
Leprosy
Rhinoscleroma
Sequlae of other infectious processes
Tuberculosis
Syphilis
Surgical causes
Based on review of 242 cases from Mayo Clinic.
Procedures per patient
2.3
Partial middle or inferior turbinectomy
56%
Total middle and inferior turbinectomy
24%
No turbinectomy
10%
Partial maxillectomy
6%
Other suggested causes
Infectious (Ssali)
Case report of AR developed in 7 children of one family after contact with another known AR child.
Dietary
(Bernat) Iron therapy found to benefit 50% of patients treated
(Han-Sen) Hypocholesterolemia present in 50% of patients.
(Han-Sen) Vitamin A therapy showed symptomatic improvement in 84%.
Hereditary (Barton, Sibert)
Proposed autosomal dominant disease due to father and 8 of 15 children contracting the disease.
Hormonal
Symptoms known to worsen with menstraution or pregnancy.
Developmental (Hagrass)
Radiologic evidence of poor maxillary antrum pneumatization and short nasal lengths
Vascular (Ruskin)
Postulated overactivation of sympathetic activity.
Environmental (Mickiewicz)
Chronic exposure to phosphorite and apatide dust
Autoimmune (Ricci)
Atrophic Rhinitis
Clinical Features
Anosmia
Ozena, i.e. foul odor
Extensive nasal crusting
Subjective nasal congestion
Enlargement of the nasal cavity
Resorption or absence of turbinates
Squamous metaplasia of nasal mucosa
Depression
Physical findings
Crusting
100% Present
Inferior Turbinates
62% Partial absence
37% Total absence
Middle Turbinates
57% Absent
Discharge
52% Present
Septum
10% Perforations
Radiographic Findings
Mucoperiosteal thickening of the paranasal sinuses.
Loss of definition of the OMC secondary to resorption of the ethmoid bulla and uncinate process.
Hypoplasia of the maxillary sinuses.
Enlargement of the nasal cavities with erosion and bowing of the lateral nasal wall.
Bony resorption and mucosal atrophy of the inferior and middle turbinates.
Biopsy Findings
Normal Mucosa
Pseudostratified Columnar
Presence of serous and mucous glands
Atrophic Rhinitis
Squamous metaplasia
Atrophy of mucous glands
Scarce or absent cilia
Endarteritis obliterans
Microbiology
Klebsiella ozenae
May be found in almost 100% of primary AR
No predominance in secondary AR
Staphylococcus aureus
Bacillous mucosus
Coccobacillus foetidus ozaena
Proteus mirabilis
Escherichia coli
Corynebacterium diphtheriae
Management
Aims
Restore nasal hydration
Minimize crusting and debris
Management Options
Topical therapy
Alkaline Nasal douching
Antiozaena solution
Systemic Therapy
Surgical Therapy
Submucosal injections
Implants to fill nasal volume
Closure of the nostrils
Topical Therapies
Alkaline N. douching
Sodium bicarbonate and Sodium diborate each 28.4 gm and NaCl 56.8gm(1:1:2) in 280 ml of warm water
Followed by application of 25% glucose in glycerine- inhibits proteolytic organism, crusting and drying
Antiozaena solution
Contains Chloramphanicol, Vit D, Oestrogen, Propylene glycol
Antibiotic solution
Gentamycin solution 80mg/L
Anti-drying agents
Glycerine
Mineral Oil
Paraffin with 2% Menthol
Other
Acetylcholine
Pilocarpine
Systemic Therapy
Avoidance of Vasoconstrictors ,Topical steroids
Systemic antibiotics -Tetracycline ,Ciprofloxacin , Aminoglycosides , Rifampicin, Streptomycin injections
Others
Vitamin A
Oral Potassium Iodide-increases nasal secretions
Vasodilators-increase blood flow to atrophic mucosa
Iron therapy
Oestrogen- in type 1 only
Placental extracts
Vaccines
Surgeries
Submucosal injection of paraffins, teflon in 50% glycerine, human placental extracts
Young operation
Modified Young operation
Volume reduction procedures – cartilage, bone , teflon, plastipore etc
Denervating operations –Stellate gangion blocks
Nasal Closure
Young’s procedure
Circumferential flap elevation 1 cm cephalic to the alar rim.
Sutures placed in center of elevated flap to close the nostril
Staged second side in 3 months
Advantages
Often provided relief of symptoms
Disadvantages
Difficult to elevate circumferential flap
Breakdown of central suture area common
Does not allow for cleaning
Did not allow for periodic examination
Recurrence after flap takedown
Nasal Closure
Modified Young’s
Elevation of extended perichondrial flap through contralateral hemitransfixion incision.
Short skin flap elevated from the intercartilaginous line on the ipsilateral side.
Suture lateral and medial flaps with vicryl.
Staged second side with first side takedown in 6 mon.
Advantages
Technically easier than Young procedure
No suture line breakdown
No vestibular stenosis on takedown
Disadvantages
Not possible with large septal defects
Does not allow for cleaning
Does not allow for periodic examination
Recurrence after flap takedown
Modified Young
Modified Young’s
Partial nostril closure leaving a 3 mm hole by placing a two cm. long thin polythene tube in the naris.
Advantages
If both the nasal cavities are closed, the patients will have to adapt to mouth breathing immediately and there is a change in their voice, developing a nasal tone.
If the patient's occupation is lifting of heavy objects, it becomes difficult to fix the diaphragm without nasal breathing.
It is also difficult for the patient for prolonged lip kissing with both the nostrils closed.
Over the period of time, all successful cases returned to normal sexual life after the successful bilateral closure. Social boycotting of the patients also ceased
Volume reduction
Plastipore implantation
Porus material allows tissue ingrowth.
Implants shaped then fenestrated for ingrowth.
Implants placed submucosally along the septum and nasal floor.
Advantages
Easier than other surgical options (Young’s)
Plastipore has low extrusion/complication rate
May be done under local anesthesia
Disadvantages
Possibility of extrusion (occurred in 1/8 pts)
Requires septal mucosa (not discussed)
Plastipore
Other Therapies
Non-surgical nasal closure
Nasal vestibule impressions taken similar to hearing aid moulds.
Impressions are used to create a silastic obturator.
Advantages
Reversible
Easily removed
Allows for irrigations
Allows for serial clinical exams
Avoids surgical morbidity
Disadvantages
May be uncomfortable
May cause sore throat due to obligate mouth breathing.
Nasal Obturator
Other Therapies
Other Implants
Acrylic
Silicone
Teflon
Silastic
Boplant
Denervation
Cervical sympathectomy (Bertein)
Stellate ganglion block (Bahl)
Sphenopalatine ganglion block (Girgis)
Parasympathectomy, i.e. GSPN section (Krmptotic)
Salivary Irrigation
Involves reimplantation of parotid duct into the maxillary sinus
Accupuncture
Time
Disease often resolves spontaneously after age 40
RHINOSCLEROMA
RHINOSCLEROMA
chronic granulomatous condition of the nose and other structures of the upper respiratory tract.
Better name respiratory scleroma
Other names- Hebra nose
- Tapir nose
HISTORICAL BACKROUNDG
Johann von Mikulich 1877;1st described histology
von Frisch identified the organism in 1882
In 1932, Belinov proposed the use of the term scleroma respiratorium
In 1961, Steffen and Smith demonstrated that K rhinoscleromatis conformed to Koch's postulates
EPIDEMIOLOGY
Endemic regions
Egypt, Southeast Asia, Mexico, Central and South America, and Central and Eastern Europe,
Infrequent in the United States, incidence increasing ,sporadic ,usually in immigrants
Rare in Saudi Arabia and Bahrain.
Five percent of all cases in Africa
Rarely lethal
All races affected.
Females somewhat more often than males.
Patients aged 10-30 years.
ETIOLOGY
Caused by the gram-negative coccobacillus k rhinoscleromatis.
Crowded conditions, poor hygiene, and poor nutrition necessary for transmission of the infectious agent
Mode of infection :droplets from exudates
Actual pathogenesis of infection
remains elusive
PATHOPHYSIOLOGY
direct inhalation of droplets or contaminated material
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
Cellular immunity impaired
Humoral immunity preserved
CD4/CD8 cell ratio altered
Decreased levels of CD4 lymphocytes - diminished T-cell response.
Macrophages not fully activated.
Mucopolysaccharides : inhibition of phagocytosis.
Patients immunocompetent in every regard except for the ineffective phagocytosis
Clinical Features
affects
nasal cavity (95-100%
nasopharynx (18-43%),
larynx (15-40%),
trachea (12%),
bronchi (2-7%).
oral cavity, paranasal sinuses, and soft tissues of the lips and nose can be affected .
spreads to the orbit rarely
presentation : nonspecific.
Nasal obstruction (most common complaint)
Rhinorrhea
Epistaxis
Dysphagia
Nasal deformity
Anesthesia of the soft palate
Difficulty breathing that progresses to stridor
Dysphonia
Anosmia
3 stages:
(1) the catarrhal, or atrophic stage
(2) the granulomatous stage
(3) the sclerotic stage
Catarrhal, or atrophic, stage
nonspecific rhinitis
purulent fetid rhinorrhea
crusting
last for weeks or months.
Granulomatous or hypertrophic stage
mucosa becomes bluish red and granular,
rubbery nodules or polyps in the nose.
Epistaxis , nasal deformity and destruction of the nasal cartilage (Hebra nose)
anosmia, anesthesia of the soft palate, enlargement of the uvula, dysphonia, and various degrees of airway obstruction early diagnosis of the condition.
Lesions
atrophic changes granulomas,
fibrotic, thick, healed stage.
The anterior-inferior part of the antrum and its medial wall more affected than other structures.
Involvement of the maxillary antrum is suggested in scleroma
maxillary antrum reservoir of infection.
Soft palate markedly thickened at its attachment to the hard palate
tapers off toward its free edge.
This sign can help in the early diagnosis of the condition.
Physical examination
erythematous granular or nodular swellings covered with crusts.
pseudotumoral rhinoscleromas
located in a variety of sites, including the septum and in the rhinopharynx.
Sclerotic stage
characterized by sclerosis and fibrosis.
nodules replaced by fibrous tissue
extensive scarring
possible stenosis
Laboratory Studies
1 culture
MacConkey agar ; diagnostic
results positive in only 50-60% of patients.
2 Bacterial identification
periodic acid-Schiff, Giemsa, Gram, and silver stains
Imaging
CT scan
soft-tissue masses of variable sizes. homogeneous and nonenhancing, distinct edge definition.
Adjacent fascial not invaded.
subglottic involvement in laryngeal and tracheal scleroma.
concentric irregular narrowing of the airway.
In the trachea, cryptlike irregularities :diagnostic
calcifications, luminal stenosis, wall thickening, and nodules.
MRI
Nasal masses obstructing the ostiomeatal units
retained secretions in the related sinuses.
In the hypertrophic stage
both T1- and T2-weighted images show characteristic mild-to-marked high signal intensity.
Other investigations
cytologic methods
Cytologic analysis of specimens of a lesion
characteristic Mikulicz type cell in the smear.
Nasal endoscopy
reveals signs of all 3 stages of scleroma: catarrhal, granulomatous, and sclerotic.
Histopathology
Plasma cells , lymphocytes and a sprinkling of eosinophils
Russell bodies
Clusters or sheets of large Mikulicz cells
Readily demonstrated by silver impregnation Warthin-Starry stains.
Electron microscopy
large phagosomes filled with bacilli and surrounded by a finely granular or fibrillar material arranged in a radial pattern.
Type A granules
type B granules
Most commonly diagnosed during the proliferative phase,
The atrophic stage 1 squamous metaplasia
2 subepithelial infiltrate of pmns
3 granulation tissue
The granulomatous stage,
1 chronic inflammatory cells,
2 Russels bodies
3 pseudoepitheliomatous hyperplasia
4 mikulicz cells
TREATMENT
Long-term antimicrobial therapy and surgical intervention in patients with symptoms of obstruction.
The goals of pharmacotherapy
eradicate the infection,
reduce morbidity, and
prevent complications.
Polymixin B ,most effective parenterally
Acriflavine :2% curative without much adverse effects
Radiotherapy :3000-3500 cGy in 3 divided doses
Surgical Care
granulomatous disease and
nasal or pharyngeal obstruction or
nasal sinus involvement due to the proliferation of lesions.
Tracheotomy : granulomatous and sclerotic stage
Plastic surgery : cicatricial stenosis
imperforation of nasal cavity, pharynx, larynx, trachea.
Surgery and laser therapy required to treat airway compromise and tissue deformity.
carbon dioxide laser vaporization
Extensively fibrotic lesions
Uvulopalatopharyngoplasty
involvement of palate with intractable symptoms
Further Outpatient Care
High rate of recurrence : prolonged antibiotic therapy (months to years) necessary.
Nasal cytology
easy and noninvasive investigation on outpatient follow up
adjuvant to clinical and histopathologic studies
a simple, reliable, and timesaving procedure that can be used with further therapy
Nasal endoscopy
Complications
upper airway obstruction.
progressive asphyxia.
Prognosis
The course is usually chronic.
Relapses can occur
( close observation is the key to the long-term follow-up care of the patient)
