A 11-year-old boy with oral submucous fibrosis : A rare case report

Oral submucous fi brosis is a chronic debilitating disorder, which is usually seen in adults with areca nut quid chewing habit. A rapid upsurge in the popularity of commercial areca nut quid products, especially among the younger generation, is a cause of grave concern, as satisfactory management is still lacking. A case of oral submucous fi brosis in an 11-year-old boy is presented along with histopathological fi ndings, highlighting strong association of areca nut chewing as the potential factor in the etiology of this condition.


INTRODUCTION
Oral submucous fi brosis (OSMF) is a chronic disorder characterized by progressive fi brosis of the lining mucosa of the oral cavity, oropharynx, and frequently the upper third of the esophagus, resulting in intolerance to spicy foods, a burning sensation in the mouth, xerostomia, and limitation of mouth opening, tongue mobility, swallowing or phonation.
It mostly occurs in Asian countries, where chewing Areca nut quid (AQ: contains areca nut, tobacco, slaked lime, and other spices) and other commercial preparations of areca nut quid in attractive pouches is popular. [1]Epidemiologic surveys in India show the rate of prevalence to be about 0.5%.Persons between 20 and 40 years of age are most commonly affected with a female: male ratio of 3:1. [2]Morbidity of OSMF among AQ users is about 3.2%, and it has a malignant transformation rate in the range of 7-13%. [3]e pathogenesis of the disease is not well established, but is believed to be multifactorial.Various mechanisms suggested include: a. Clonal selection of fi broblasts with a high amount of collagen production during long-term exposure to areca nut, b.Stabilization of collagen structure by catechin and tannins from AQ, c.Production of stable collagen (type I) by OSF fi broblasts, d.Increase in collagen cross-linking by upregulation of lysyl oxidase, e. Defi cient collagen phagocytosis, and f. micronutrient and vitamin defi ciencies. [4]MF is uncommon in the pediatric age group.Only limited number of pediatric cases have been reported in the literature, youngest being a 4-year-old girl [5] and in other two reports 9 and 11 years old girls have been reported. [6,7]A Pediatric case of OSMF is reported, highlighting the potential danger of involvement of the pediatric age group from "pouch culture".
General physical examination was normal.On Oral examination, there was reduced inter-incisor distance (IID) of 1.5 cm, there were generalized tobacco stains on teeth and hard palate with decreased elasticity and generalized blanched oral mucosa [Figure 1].A punch biopsy from the oral mucosa was taken and sent for histopathological examination.Microscopic picture with H&E stain shows chronic infl ammatory reaction and sub-epidermal fi brosis with focal atrophy to hyperplasia with fi broblastic change of lamina propria [Figure 2, Low and High Power).On MTS stain, there was fi brosis involving muscles, which were replaced by blue colored collagen [Figure 3].PAS stain was negative for glycogen [Figure 4a] and Von Gleisson stain was negative for muscle [Figure 4b].So, a diagnosis of OSMF was established on clinical basis, which was confi rmed by different histopathological examination stains.
Patient along with his parents were counseled to immediately stop the usage of areca nut quid and its preparation, and was given triamcinolone paste for local application, Oral pentoxyphylline 100 mg TDS for fi ve weeks and oral multivitamin -antioxidants preparation and mouth-opening exercise for six months.At six month, though there was minimal improvement in mouth opening but there was marked improvement in complaint of burning sensation in mouth.

DISCUSSION
Oral submucous fi brosis is regarded as a collagen metabolic disorder with an overall increased collagen production and decreased collagen degradation resulting in increased collagen deposition in the oral tissues, and fi brosis due to alkaloid exposure as a result of exposure to AQ. Quid is defi ned as "a substance, or mixture of substances, placed in the mouth or chewed and remaining in contact with the mucosa, usually containing one or both of the two basic ingredients, tobacco and/or areca nut, in raw or any manufactured or processed form."Clear delineations on contents of the quid (areca nut quid, tobacco quid, and tobacco and areca nut quid) are recommended as absolute criteria with fi ner subdivisions to be added, if necessary.The betel quid refers to any quid wrapped in betel leaf and is therefore a specifi c variety of quid. [8]al submucous fi brosis is seen predominantly in Asians who chew betel nut quid or its variants such as gutkha (mitha pan), kiwam, zarda and pan masala.Sweetened versions of betel nut quid are sold to children as sweet supari, gua, mawa or mistee pan.The easy availability of packaged dried product such as gutkha, is increasing the incidence of this condition, as it consists of a greater dry weight of areca nut quid along with other OSMF producing ingredients than the traditional quid rolled in betel leaf (paan).Only betel nut chewing does not cause OSMF, contribution of other ingredients like slaked lime is more important in the causation of OSMF. [9]al submucous fi brosis can be seen at any age, but is rare in young children.The predominant age group affected is 20-40 years.Compared to traditional betel nut quid, gutkha chewing tends to begin at a younger age and has a shorter time to the development of disease, so cases of OSMF have begun to appear in younger age group.The list of treatment modalities is extensive.First and foremost is the behavior therapy to condition the subjects to stop this addiction.A wide variety of treatment modalities has been suggested, which include the use of micronutrients and minerals, CO 2 laser, drugs like pentoxifylline, lycopene, interferon gamma, turmeric, hyalase, chymotrypsin, and placental extracts.As fi brosis cannot be reversed when mouth opening is severely reduced, surgical interventions such as myotomy, coronoidectomy, and excision of the fi brotic bands have also been reported in the literature.Alternatively, procedures such as insertion of stent, physiotherapy, local heat therapy, and mouth-opening exercises with acrylic carrots and ice-cream sticks have been tried with variable success rates. [10,11]ll today, no well-established treatment for OSMF exists and approaches using injections of steroids, chymotrypsin, hyaluronidase, or alcohol, and surgery using mucosal or non-vascularized split thickness grafts have not only been ineffective but have also often exacerbated the condition, with added scar tissue. [12]

CONCLUSION
There is a close relationship between areca-nut chewing and the development of OSMF.The present drug treatments are empirical and symptomatic in nature.So, there is no certain cure for the condition, just training and prevention seem to be the only way to reduce the risk of OSMF.In the near future, younger ones may be affected more and an active preventive approach is required to limit the morbidity associated with the modern pouch culture.

Figure 1 :
Figure 1: Picture showing reduced mouth opening with blanched oral mucosa

Figure 4 :
Figure 4: (a) PAS Stain negative for Glycogen, and (b) Von Gleisson Stain negative for muscle a b