Oral pathology in paediatric patients-mini-systematic review

Gingival/Dental lamina cyst of neonates

These type of cysts arise from remnants of dental lamina. They don’t show any symptoms, may be solitary or multiple, 1-3 mm in diameter ,may be nodular, creamy white in colour mostly seen bilaterally on the rigde of alveolar bone. On histopathological examination it shows keratin-filled true epithelial cysts. These lesions are present at birth and are self-resolving hence they do not require any intervention.8, 9 Prevalence ranges from 25-53%. 9, 10

Bohn’s nodules

Bohn’s nodules are remnants of minor salivary gland epithelium. These lesions also do not show any symptoms, they are mostly seen as whitish in colour, smooth, keratin filled nodules or papules ranging from 1-3 mm in diameter mostly arise on the buccal and lingual aspects of the ridge away from the midline. They resolve within first 2 to 3 months of life and have an Incidence of 47.4%. 3, 6, 11, 12

Congenital epulis of the newborn

It is also named as Granular cell tumor/Neumann’s tumor is a rare benign tumor of uncertain histological features in the newborn infants. It arises as a bulging gingival mucosal mass on the anterior maxillary ridge. The etiology is unknown many studies have reported the lesion is hormone related, degenerative or reactive. Cinically it is seen as solitary , firm in consistency with a regular surface. It may be multilobed, sessile or pedenculated, pink or red lesion non tender on palpation. The diameter varies from a few millimeters to centimeters.13 Incidence is 0.0006 with a female predilection of 8:1 to 10:1. Larger lesions may lead to mechanical obstruction in respiration and feeding. Diagnosis is confirmed by site of origin, prenatal/ natal, Ultrasonography (USG), Computed Tomography (CT)/Magnetic Resonance Imaging (MRI) and on histopathological examination it shows scattered odontogenic epithelium, absence of interstitial cells, angulate bodies and vessels. Treatment of choice is surgical excision. 14, 15

Eruption cyst

The eruption cyst is a form of soft benign cyst and it arises around an erupting tooth when the dental follicle separates from the crown of tooth and results in fluid collection within this space.15 Shape of this cyst resembles a dome and its colour varies from normal to blue-black, purple or brown.it is also known as eruption hematoma. It is sometimes is seen as transparent. Since the tooth erupts through the lesion it resolves. If it becomes infected or does not rupture surgical opening of the roof of the cyst is indicated.15, 16 The prevalence of eruption cysts is 22%.

Epidermoid and dermoid cysts

This slow growing, asymptomatic cyst arises on the floor of the mouth and the sub mental region these soft, cystic lesions are nodular with a sessile base and lined with squamous epithelium. An epidermoid cyst is lined by epidermis and a dermoid cyst is lined with adnexa glands Clinical diagnosis is through enlargement which leads to respiratory disorders and feeding difficulties. Diagnostic tests include, Magnetic Resonance Imaging (MRI)/ Computed Tomography (CT), prenatal/natal Ultrasonography (USG), Fine Needle Aspiration Biopsy (FNAB) and histopathology. Surgical enucleation is the treatment of choice and its recurrence is very rare.2, 3, 16 The prevalence in head and neck patients is 7% and in the oral cavity is 1.6%.16

Mucocele

This is a bluish, well-circumscribed, translucent, fluctuant swelling. Its most common site is the lower lip lateral to the midline. When the excretory duct of a minor salivary gland ruptures due to mechanical trauma andmucin leaks into the surrounding connective tissues with in a fibrous capsule it arises. It may be normal or whitish and keratinized. Sometimes may also appear on retromolar region, buccal mucosa, ventral tongue surface and floor of the mouth as a ranula. Superfcial mucocele resolves on bursting spontaneously with a shallow ulcer. Treatment minimizes the risk of recurrence.17 The prevalence is 2.4 cases per 1000 people.

Riga-Fede disease

The rubbing of natal or neonatal tooth during feeding on the ventral tongue surface, lip, gingival, vestibular mucosa, palate and floor of the mouth leads to ulcers. If it is undiagnosed and not treated this lesion may result in dehydration and inadequate nutritional intake. Diagnosis is confirmed by clinical examination and histopathology reveles bacterial, fungal infections, immunologic diseases, and neoplasia as causative factors for ulcers Conservative treatment is the best and focus on creating round, smooth incisal edges. Alternatively extraction is the treatment of choice. Prevalence of natal-neonatal teeth ranges from 1:6000 to 1:800.16, 17, 18

Neonatal osteomyelitis of maxilla

This isa rare infection attributed to risk factors like catheterization, parenteral nutrition status, prolonged hospitalization, ventilator support and nosocomial infection or iatrogenic. It caused by organismsincluding Staphylococcus auras, group B Streptococcus (Streptococcus agalactiae) and Gram-negative organisms (Escherichia coli and Klebsiella pneumonia). Main feature of this condition is gross swelling on the affected side. Maxilla affects both eyelids with proptosis and chemosis; conjunctivitis; swelling and in duration of cheek and includes a unilateral nasal discharge. Chronic osteomyelitis is uncommon in children. Diagnosis is based on positive blood culture and laboratory tests (erythrocyte sedimentation rate, C-reactive protein, leukocyte count). Antimicrobial drugs are drug of choice Swith or without surgery are required. The morbidity of this condition is high hence the prognosis is poor.19

Neonatal candidiasis

This is trasnsmitted via external contamination in preterm newborns. The candida species include Candida albicans (75%), krusei, glabrata, tropicalis and parapsilosis. Risk factors include prolonged hospital stay, immature immune system and prolonged catheterization. It manifests as white plaques ofhyphae, epithelial cells and necrotic tissues on the oral mucosa. Systemic features include endolphthalmitis, meningitis, urinary tract and cardiovascular infections. Diagnosis is confrmed by blood culture, urine and CSF. Treatment mainly is preventive measures. The Incidence of disseminated candidiasis in preterm infants is 7%-20%.20

Neonatal herpes simplex virus infection

Herpes simplex virus 1 causes orolabial lesions whereas HSV1 and HSV2 cause genital lesions. They are transmitted during parturition and depend on maternal infection (primary or recurrent), maternal antibody, intact mucocutaneous barriers, duration of membrane rupture and delivery.18, 20 The incubation period ranges from 4-21 days post delivery and the symptoms arise between 6-21 days. The vesicles arise in the mouth, face, scalp, palms and feet. They may be single or clustered; 1-3 mm in diameter and eventually ulcerate. Other symptoms include hepatitis, pneumonitis and seizure and disseminated intravascular coagulation. Diagnosis is based upon viral culture, serology and a polymerase chain reaction amplifcation analysis of Cerebrospinal Fluid (CSF). Antiviral therapeutic agent is Acyclovir. Incidence is 31 per 100,000 births.

Neonatal pemphigus vulgaris

This rare autoimmune, vesiculobullous disease is subsequent to trans placental transmission of maternal immunoglobulin G auto antibodies which counter transmembrane glycoprotein desmoglein three.It manifests as multiple mucosal, cutaneous or mucocutaneous ulcerations after birth. These blisters may arise on soft palate, ventral surface of the tongue, gingiva, buccal mucosa, and lower lip. In more advanced stages desquamative or erosive gingivitis may be present. The other oral manifestations include halitosis, sialorrhea and brown or blackish crusts at the vermillion border. Symptoms resolve in 2 to 3 weeks and the diagnosis is confirmed by histopathology and immunofluorescence.21 The incidence is 0.68 cases per 100,000 persons per year but varies in different regions.

Hemangioma

Hemangioma is a benign vascular neoplasm emerging as a macule at birth but after few weeks it regresses and appears as spotted pigments. The course of disease follows a rapid proliferating phase (0-1yr),involuting phase (1-5 yr) and involuted phase (5-10 yr). It appears on neck and head, trunk, extremities, lips, tongue, buccal mucosa, palate and uvula.3 Infantile age, infant birth weight, childbearing age, gestational hypertension, Kasabach-Merritt syndrome are predisposing factors.20 Diagnosis is made by clinical history, Fine-Needle Aspiration Cytology (FNAC), MRI, and/color doppler USG, histopathology, and immunohistochemistry ruling out other vascular malformations.22 Stage specifc treatment drugs (α-interferon, propranolol, corticosteroids), surgery and lasers (CO2, flash lamp pulsed dye, diode) are the treatment modalities. Some cases resolve completely but some show permanent skin such as hypopigmentation, telangiectases, anetoderma stippled scarring and fibro-fatty residues. Incidence is 4 to 5%

Melanotic neuroectodermal tumor of infancy

This rare pigmented benign neoplasm appears in the first 6 months with a male predilection. It arises from the neural crest cells and may be located on the tongue, buccal mucosa, palate or floor of the mouth.It may be present in the craniofacial region, brain, skull, maxilla, mandible and the genitals. It is a painless, expansile, non ulcerative rapidly growing, pigmented, lesion with a locally aggressive behavior. Diagnosis is based on clinical assessment, histopathology and CT/MRI. This biphasic tumor comprises of melanocytic and neuroblast-like round cells. Differential diagnosis includes desmoplastic small round cell tumor, Ewing’s sarcoma, neuroblastoma rhabdomyosarcoma, peripheral neuroepithelioma, peripheral primitive neuroectodermal tumor, malignant melanoma and neuroblastoma. Treatment includes chemotherapy and radiotherapy either alone or in combination with surgical excision. Recurrence is rate is high at the same time metastasis and malignant transformation rate is also high. The overall incidence of local recurrence is 10-15%. 23

Restrictive mandibular lingual frenum/Ankyloglossia

This is a developmental anomaly it is a thick, tight, short fibrous vertical band of tissue called lingual frenum formed by a small band or fold of mucosal membrane. It restricts the movement of tongue (partial ankyloglossia/tongue tie) or fuses the tongue and floor of the mouth (total ankyloglossia). Difficulties associated with neonates include discomfort on breastfeeding, limited mobility of tongue, speech pathology, malocclusion, gingival recession, deglutition problems and localized gingival recession on the lingual aspect of the mandibular incisors. It may be associated with craniofacial anomalies or appear independently. It may be mild, moderate, severe or complete. Treatment may include surgical correction via frenuloplasty. The incidence varies from 0.1% to 4.8%. 24

Mandibular labial frenum

A high frenum present in the shallow vestibular region of the mandibular permanent central incisor inserts into the free or marginal gingival tissue. Lower lip movements pull the fibers and lead to food and plaque entrapment. Inflammation, pocket formation, recession, alveolar bone loss and/or tooth can be prevented by early treatment. It is treated by a surgical incision through frenuloplasty/ frenotomy thereby releasing the frenum and correcting anatomy or frenectomy via cutting frenum, hemostasis and wound management. Post procedure care includes soft diet, oral hygiene instructions and analgesic. Other techniques such as electro surgery or lasers have a short working time, better hemostasis, few postoperative complications (swelling, infection), reduced intra-and post-operative pain, better patient acceptance and no suture removal. Laceration of frenum may lead to profuse bleeding the prevalence of interdental space in the primary dentition is 70% for the maxilla and 63% for mandible. 25

Natal and neonatal teeth

Natal teeth are present at birth and neonatal teeth erupt within the first 30 days of life. The incidence varies from 1:1,000 to 1:30,000. Mandibular primary incisors are affected.2 Molars may be associated with systemic conditions or syndromes such as histiocytosis X, Pfeffer syndrome. Etiology maybe a superficial tooth germ associated with a hereditary factor. Tooth should be preserved if excessive tooth mobility or feeding problems are absent. Riga-Fede disease is an ulcerated disorder which is caused by rubbing of the natal or neonatal tooth against the ventral tongue surface at the time of feeding. Failure to diagnose and treat this lesion may lead to dehydration and inadequate nutritional intake for the infant. If the treatment is conservative then the incisal edge is smoothened. The alternate option is extraction. Potential for haemorrhage is important to consider prior to extracting a natal or neonatal tooth and for a child less than 10 days of age a pediatrician should be consulted regarding adequate hemostasis. 26

Odontogenic infections

These are typically polymicrobial and comprise of aerobic, facultative anaerobes and strict anaerobes. Intraoral infections and abscesses develop through basic inflammatory processes, relevant pathogens,biochemical processes mediated by pro-inflammatory molecules, host response and manifest clinically as intraoral septic processes. They are secondary to caries, trauma or periodontal problems and may involve more than one tooth. Soft tissue infections of odontogenic origin spread along planes of least resistance from the supporting structures of the affected tooth to various potential spaces in the vicinity. Accumulated pus must perforate bone, generally at the site where it is thinnest and weakest, before extending into the periapical areas or deeper facial spaces. If the pus perforates through either the maxillary or mandibular buccal plate inside the attachment of the buccinator muscle, infection is intraoral. If the perforation is outside this muscle attachment, infection will be extraoral. 27

If these infections are left untreated they may lead to abscess, pain, cellulitis and discomfort on eating or drinking and dehydration. Infection in the upper part of the face includes facial pain, fever, and inability to eat or drink and diffcult to localize. Sinusitis may mimic the symptoms of an odontogenic infection hence should be ruled out. Infections in the lower part of the face include pain, swelling, and trismus.26 They may be associated with the skin, teeth, lymph nodes,and salivary glands.26 and a swelling of the lower face is most likely associated with a dental infection. Management of odontogenic infections includes pulp therapy, incision and drainage or an extraction. Antibiotics are indicated in systemic manifestations such as facial cellulitis, diffculty in swallowing or breathing, fatigue, high temperature (102 to 104 degrees Fahrenheit) and nausea. Rare but severe odontogenic infection complications such as Ludwig’s angina and cavernous sinus thrombosis4, 5, 6, 7, 8, 9, 10 may be life threatening and may require instant hospitalization, intravenous antibiotics, incision and drainage and referral/consultation for an oral and maxillofacial surgeon (false).

Pediatric Oral Pathology Management

Diagnosis for paediatric oral lesions is based on a thorough history, assessment of risk factors, detailing the signs and symptoms of the lesion. The lesion most likely to be the working diagnosis is determinant of the initial management. A definitive diagnosis is made after a biopsy of the unhealthy tissue. Biopsy is the gold standard for diagnostic tests. It is the exclusion of a portion of live tissue for diagnostic study.19 Excisional biopsies are the total removal of small sized lesions smaller than one centimeter. An incisional biopsy is performed on a suspected malignancy in which a lesion maybe large, diffuse or multifocal. Multiple incisional biopsies may be indicated for lesions which are diffuse. Diagnosis maybe assisted by adjunctive tests such as a fine needle aspiration, exfoliative cytology and the cytobrush technique. 28

The biopsy specimen from the oral and maxillofacial region is submitted for histopathological examination. Tissues excluded include carious teeth without soft tissue attachment, extirpated pulp and normal tissue from gingival recontouring.28 The description of grossing should always be entered into the patient s record book. A lesion which persists for more than two weeks despite removing the causative agent or drug therapy warrants a soft tissue biopsy. If the differential diagnosis indicates more than one disease or neoplasm the hard or soft tissue must be evaluated by a pathologist. Histopathology informs about the clinical behaviour, a defnitive diagnosis, prognosis, the need for additional treatment or follow-up, allows evidence-based care provision with an increased likelihood for a positive result.29 However Oral biopsies can be performed by a pediatric dentist as well. The lesions which should have a biopsy include gingival hyperplasia unresponsive to oral hygiene regimen, mucocoele, pyogenic granuloma and other reactive lesions of gingiva, squamous papilloma or oral wart, irritation fibroma, periapical cyst or granuloma which may or may not be attached to an extracted tooth, inflamed operculum, hyperkeratosis of uncertain cause, persistent oral ulcers, benign migratory glossitis with an atypical or stationary pattern, smokeless tobacco keratosis, mucocutaneous diseases, odontoma may be complex and compound and dentigerous or dental follicle cyst. 29, 30