- In 1952, Schwartz coined the term atrophica idiopathica mucosa oris to describe an oral fibrosing disease he discovered in 5 Indian women from Kenya. Joshi subsequently coined the termed oral submucous fibrosis (OSF) for the condition in 1953.
- Oral submucous fibrosis is a chronic debilitating disease of the oral cavity characterized by inflammation and progressive fibrosis of the submucosal tissues (lamina propria and deeper connective tissues). Oral submucous fibrosis results in marked rigidity and an eventual inability to open the mouth. The buccal mucosa is the most commonly involved site, but any part of the oral cavity can be involved, even the pharynx.
- The condition is well recognized for its malignant potential and is particularly associated with areca nut chewing, the main component of betel quid. Betel quid chewing is a habit practiced predominately in Southeast Asia and India that dates back for thousands of years. It is similar to tobacco chewing in westernized societies. The mixture of this quid, or chew, is a combination of the areca nut (fruit of the Areca catechu palm tree, erroneously termed betel nut) and betel leaf (from the Piper betel, a pepper shrub), tobacco, slaked lime (calcium hydroxide), and catechu (extract of the Acacia catechu tree). Lime acts to keep the active ingredient in its freebase or alkaline form, enabling it to enter the bloodstream via sublingual absorption. Arecoline, an alkaloid found in the areca nut, promotes salivation, stains saliva red, and is a stimulant.
Oral submucous fibrosis has a high rate of morbidity because it causes a progressive inability to open the mouth, resulting in difficulty in eating and consequent nutritional deficiencies. Oral submucous fibrosis also has a significant mortality rate because it is a premalignant condition and malignant transformation has been noticed in 3-7.6% of cases
Oral submucous fibrosis is clinically divided into 3 stages:
- Stage 1: Stomatitis
- Stage 2: Fibrosis
- a- Early lesions, blanching of the oral mucosa
- b- Older lesions, vertical and circular palpable fibrous bands in and around the mouth or lips, resulting in a mottled, marble-like appearance of the buccal mucosa
- Stage 3: Sequelae of oral submucous fibrosis
- a- Leukoplakia
- b- Speech and hearing deficits
Histologic findings in oral submucous fibrosis are generally characterized by diffuse hyalinization of the subepithelialstroma, atrophic epithelium and intercellular edema, with or without keratosis, epithelial dysplasia, chronic inflammation and fibrosis in the minor salivary glands in the area of quid placement; and atrophy of the underlying muscle.
Staging: Khanna and Andrade in 1995 developed a group classification system for the surgical management of trismus.
- • Group I: Earliest stage without mouth opening limitations with an interincisal distance of greater than 35 mm.
- • Group II: Patients with an interincisal distance of 26-35 mm.
- • Group III: Moderately advanced cases with an interincisal distance of 15-26 mm. Fibrotic bands are visible at the soft palate, and pterygomandibular raphe and anterior pillars of fauces are present.
- • Group IVA: Trismus is severe, with an interincisal distance of less than 15 mm and extensive fibrosis of all the oral mucosa.
- • Group IVB: Disease is most advanced, with premalignant and malignant changes throughout the mucosa.
The diagnosis is usually made on history of areca nut exposure and clinical examination.
Suggested clinical criteria are one or more of the following:
- whitening of the oral mucosa
- palpable fibrous bands
- tough leathery texture to the oral mucosa
More than one fourth of affected persons may have coexisting leukoplakia. The buccal mucosa is the most commonly involved site, followed by the lip and tongue, but OSF can occur in any intraoral site.
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Mucosal biopsyshows nonspecific pathologyin early stages with swelling and acute inflammation. However in well established disease, the pathology is characteristic with chronic inflammation and the presence of thick collagen bands and reduced blood vessels. The surface epithelium is thinned and dysplasia is common. Muscle degeneration is seen in late disease.
Barium swallow may be useful to assess throat and oesophageal involvement.
Blood tests may be required to assess nutritional state and for autoimmune conditions.
Its believed that OSF is “irreversible”. Elasticity of buccal mucosa cannot be brought back. MARCEL does it with aplomb. MARCEL-OMR Therapy re-assures OSF patients for bringing back elasticity of buccal mucosa by regenerating the damaged mucosa.
Oral Submucous Fibrosis: a chronic debilitating disease of theoral cavitycharacterized by inflammation and progressivefibrosisof thesub mucosal tissues.A generalised pathological state of the oral mucosa associated with a significantly increased risk of cancer. Early OSF-Prodromal symptoms: Burning sensation in the mouth on consuming spicy food, Blisters on the palate Ulcerations or recurrent generalized inflammation of the oral mucosa, excessive salivation, defective gustatory sensation, dryness of the mouth Periods of exacerbations with appearance of small vesicles in the cheek and palate. Petichiae are observed on tongue followed by labial and buccal mucosa. Pain in area with submucosal fibrotic bands on palpation is a useful clinical test. Histologically- Hyperplastic epithelium Numerous dilated and blood filled capillaries juxtaepithelially. Inflammatory cells: lymphocytes, plasma cells. Large number of Lymphocytes, fibroblasts and plasma cells suggest tissue reaction leading to OSF Advanced OSF: Oral mucosa becomes blanched and slightly opaque and white fibrous bands appear Buccal mucosa, lips, palate and faucial pillars are affected Oral mucosa is involved symmetrically and fibrous bands in the buccal mucosa run in vertical direction Soft Palate: initially: fibrous depostis varies from slight whitish areas on soft palate, with no symptoms later: dense fibrosis, causing fixation and shortening or even deviation of uvula and soft palate. Faucial Pillars: ranges from slight sub mucosal accumulation in both pillars to a dense fibrosis extending deep into the pillars with strangulation of tonsils. Difficulty in mouth opening is due to Dense Fibrosis involving the tissues around the pterygomandibularraphae. In severe cases the Fibrosis may spread to Pharynx and down the piriform fossae Circular bands are felt around the entire Rima Oris(mouth orifice) and seen more around the lower lip. Tongue: Decreased movement and atrophy of tongue papillae. With Progressive fibrosis the following changes can be seen :
- Difficulty in mouth opening
- Inability to whistle
- Difficulty to blow out a candle
- Difficulty in swallowing
- Habitual areca Nut chewing is the main cause which leads to
- Stimulation of Fibroblast proliferation leading to excessive collagen synthesis.
- Decreased secretion of collagenase
- Very early
- Moderately advanced
- Subepithelial collagen
- Presence or absence of edema
- Physical state of mucosal collagen
- State of blood vessels
Nowadays Chewable (Pan-Masala, Gutkha, Kharra, chewable tobacco) has become a “Status Symbol”. This habit is common in all socio-economical strata and age group. Laborers, High class Executive, School Going Kid, Collegians, Housewives everybody falls prey to these temptations of so-called “freshening” his/her mouth with these “Slow Poisons”.
According to various studies carried out at national and international levels, it has been proved beyond doubt that Gutkha and Pan Masala contains ‘Mutagens’ which can change our normal tissues in to cancer tissues, and can cause a progressive disease called, “ Oral Sub Mucous Fibrosis (OSF). After some time of chewable abuse the skin loses its elasticity. Thus, the disease has been termed as Oral (meaning mouth), Sub mucous (meaning skin below the delicate skin of mouth) and Fibrosis (meaning hardening and scarring). Its acronym is OSF.
As far as clinical experience goes, a majority of cases of OSF can be directly linked to Gutkha & Pan Masala chewing as a major contributory factor among many other like Smoking, Supari chewing and consumption of spicy foodstuff. Many scientists believe that ill effects of chemicals like capsaicin (found in chilies) and arachidene and aracholine (found in Supari) is the major cause of OSF.
Oral Sub mucous Fibrosis (OSF) :-
By definition“ Oral OSF is an insidious chronic disease affecting any part of the oral cavity and sometime the pharynx. Although occasionally preceded by and/or associated with vesicle formation. It is associated with a juxtaepithelial inflammatory reaction followed by a fibro-elastic change of the lamina propria, with epithelial atrophy leading to stiffness of the oral mucosa and causing trismus and inability to eat. ”
|Dietary Component||Vitamin-B deficiency
|Food habits||Spicy, hot food (prepared with excessive use of chilies)|
|Other habits||Tabacco,Ghutka, Pan- Masala, Snuff, Quid etc.|
The aetiology is multifactorial but betel (pan), Gutkha, Pan Masala chewing is strongly suspected. Pan typically consists of areca nut, tobacco and lime wrapped in betel leaf. While pan-masala and Gutkha consists of areca nut as main ingredient.
Experimentally, aeroline, a derivative of areca, can induce fibroblast proliferation and collagen synthesis. There is significant association between areca nut consumption and sub mucous fibrosis.
Why Gutkha & Pan-Masala causes OSF: Harmful chemicals present in various ingredients of Gutkha like: Phenol, Tannin, Polycyclic Aromatic Hydro-carbons, Nitrosamines, Cancer causing Mutagens, Toxic metals such as Nickel, Cadmium, Lead along with pesticides residues. Presence of pre-formed N-nitrososarcosine, N-nitrosoazotidine-4-carboxylic acid and N-nitrosothiazolidine-4-carboxylic acid is also noticed.
SIGNS & TESTS: -
- Ulceration in mouth
- Excessive salivation
- Oral mucousa becomes blanched and opaque leading to stiffness of oral mucousa.
- Patient cannot blow out his cheek
Initial signs of OSF is burning sensation in mouth during consumption of spicy foodstuff because of scarring of delicate skin of the mouth becomes whitish and it gets irritated. The other cardinal feature is a progressive inability to open mouth.
Blanched, opaque and stiff oral mucousa in case of OSF is a result of build up of thickened layers of keratin, which is a tough protein. Reddened area in the mouth may result when lining of the mouth thins and blood vessels show through more than usual. White and red area may be non-cancerous, pre-cancerous or cancerous. The patients feel muscle pain and tightness around the jaws. A person who chews tobacco or Gutkha may develop white, ridged bumps on the insides of the cheeks. These bumps can develop into carcinoma. It is most commonly occurs in younger age group between 18-30 years of life.
It depends upon stages of OSF.
|Stage – I
Stage – II
|Prognosis is good|
|Stage – III||Prognosis is poor|
Clinically, symmetrical fibrosis of such sites as the buccal mucosa, soft palate or inner aspects of lips is characteristic. The overlying mucousa may be normal or there may be a vesiculating stomatitis. Oral Sub Mucous fibrosis typically affects the buccal mucosa, lips, retro molar areas and the soft palate. Occasional involvement of the pharynx and esophagus is seen. Early lesions present as a blanching of the mucousa, imparting a mottled, marble-like appearance. Later lesions demonstrate palpable fibrous bands running vertically in the buccal mucousa and in a circular fashion around the mouth opening or lips. As the disease progresses the mucousa becomes so stiff that it cannot be indented with fingers, causing difficulty in eating and considerably restricting the patient’s ability to open the mouth (trismus). If the tongue is involved, it becomes stiff and has a diminished size.
Mucosal petechiae are seen in more than 10% of cases and most patients of a burning sensation, often aggravated by spicy foods, Salivary flow is diminished and blotchy melanotic mucosal pigmentation is often seen. More than a fourth of affected persons develop precancerous, leukoplakia of one or more oral surfaces. Once present, oral sub mucous fibrosis does not regress, either spontaneously or with cessation of betel quid chewing.
OSF by itself is not carcinogenic but it makes the skin of the mouth prone to cancer. Patients with OSF must therefore maintain diligent oral hygiene and get a through oral examination done at regular intervals at a competent facility. Research has shown that about 5 to 6 percent of cases of OSF will progress into full-fledged oral cancer.
The subepithelial connective tissue becomes thickened, hyaline and avascular, and there may be mild chronic inflammation. The epithelium usually becomes thinned and may shoe atypia. Underlying muscle fibers undergo progressive atrophy.
Pathology and Differential Diagnosis
The early cases of OSF are present as chronic inflammatory cell infiltration of subepithelial connective tissues. This otherwise nonspecific infiltrate a reduced vascularity, reduced numbers of inflammatory cells and dense bundles and sheets of collagen immediately beneath the epithelium, The eventual thick band of hyalinized subepithelial collagen shows varying extension into submusosal tissues, typically replacing the fatty or fibro vascular tissues normal to the site.
Minor salivary in the area of habitual quid / Pan-Masala/ Gutkha placement often demonstrate a chronic inflammatory infiltrate and replacement of acinar structures by the hyalinized stroma can be distinguished from the amyloidal infiltration of amyloidosis through the use of Congo red staining and thioflavin-T staining under polarized and immunofluorescent light.
The epithelium is atrophic, with or without excess surface keratin, and demonstrates intercellular edema. A fourth of the biopsies cases will demonstrate epithelial dysplasia at the time of biopsy. When squamous cell carcinoma is seen. It has the same features of carcinoma as those seen in persons without the betel quid chewing habit.
OSF is always diagnosed based simply on person’s medical history and physical examination. Part of the examination involves pushing on the side of the face or placing the little finger in the person’s ear and gently pressing forward while person opens and closes the jaw. Also, gently palpates the muscle used for chewing to detect pain or tenderness and notes whether the jaws slides when the person bites. Special x-ray can also help in diagnosis. Though expensive, a Computed Tomography (CT) or Magnetic Resonance Imaging (MRI) scan is used in rare instances to find out why a person is not responding to treatment.
Following treatments are currently advised.
- Multivitamin and anti-oxidant Supplementation.
- Intralesional Injections of Steroids into the hard tissue.
- Excision and Skin Grafting.
- Surgical removal of hardened bands.
- Tongue in Cheek (Tongue Flap) and Nasolabial Flap Surgery.
- Physical Therapy - Electromyography, Spray & Scratch Exercise, Friction Massage, Transcutaneous Electrical Nerve Stimulation, topical steroidal cream and jelly for local application.