DEVELOPMENTAL DISTURBANCES OF ORAL TISSUES Prf. Dr. Gamal Fatah-ALLAH

II- Developmental Disturbances of The Oral mucosa : Titles I- Developmental Disturbances of The tongue. II- Developmental Disturbances of The Oral mucosa : Fordyce’s Granules Leukodema White sponge nevus IV- Developmental Disturbances of The lip. V- Developmental Disturbances of The Jaws. VI- Developmental Disturbances of The Salivary glands. VII- Developmental Disturbances of The face. VIII- Syndromes associated with abnormalities of oral tissues:

II-1- Fordyce’s Granules Ectopic sebaceous glands inside the oral mucosa. Developmental and can be considered a variation of normal.

Clinical Features of Fordyce’s Granules Multiple aggregates of pin-head sized spots (few millimeters in diameter) slightly yellowish in color. Symmetrically distributed Become obvious after puberty. Site: Buccal mucosa Vermilion border of lips. Clinically; Asymptomatic in > 80%.

Fordyce’s Granules in Buccal Mucosa

Fordyce’s Granules in Vermilion Border of Lip

Microscopic examination of Fordyce’s Granules Lobules of sebaceous gland in submucsa or aggregated around excretory ducts, which are blocked with keratin. The individual cells are polygonal in shape with centrally placed nuclei & foamy cytoplasm.

II-2- Leukoedema Asymptomatic developmental condition, more common (&more pronounced) in smokers and in dark-skinned-people It is a diffuse, gray-white, milky opalescent appearance of the mucosa Usually bilaterally on the buccal mucosa. Less often, labial mucosa, palate or floor of mouth. The surface of the area is folded, wrinkled, white streaked lesion. It disappears when the mucosa is stretched.

II-3- White sponge nevus Cannon's disease Autosomal dominant; mutations in keratin coding genes (defect in mucosa keratinization) Usually, present from birth or develop during childhood. Thick, white and velvety on the inside of the cheeks within the mouth. The condition is entirely harmless, and no treatment is required.

leukodema White sponge nevus Increased epithelial thickness, broadening and elongation of the rete ridges, parakeratosis and intracellular edema of the spinous layer. Hyperkeratosis and acanthosis with eosinphilic perinuclear condensation in superficial epithelium Hydropic change

IV- Developmental Disturbances of The lip. Titles I- Developmental Disturbances of The tongue. II- Developmental Disturbances of The Oral mucosa IV- Developmental Disturbances of The lip. Congenital lip pits & fistulas. Cleft lip. Van der Woude’s syndrome Cheilitis glandularis. Cheilitis granulomatosa. V- Developmental Disturbances of The Jaws. VI- Developmental Disturbances of The Salivary glands. VII- Developmental Disturbances of The face.

1- Congenital Lip Pits & Fistulas Rare condition Etiology: Hereditary in nature 70% of the lip pits are associated with cleft lip or palate. Associated with Van der Woude’s syndrome.

Clinical Features of Congenital Lip Pits & Fistulas Site Most cases occur on the lower lip On either side of the midline (vermilion border). Congenital commissural pits; up to 4 mm deep with the tissue around the opening slightly raised. Size: ≥ 3 mm in diameter Up to 2 mm deep. commissural pits upto 4mm deep Shape; may be: Circular or transverse slits. Rarely, It arise at the apex of a nipple-like elevation. It often exude saliva spontaneously or upon pressure.

Clinical Features of Congenital Lip Pits & Fistulas 3 mm or more in diameter and up to 2 mm deep.

Clinical Features of Congenital Lip Pits & Fistulas May be located at the apex of a nipple-like elevation.

Congenital Commissural Pits At the angle of the mouth up to 4 mm deep with elevated tissue around the opening.

2- Cleft Lip It is a gap in the skin of the upper lip that extends variably to the base of the nose. Etiology (Multifactorial); Combination of: Genetic (inherited) Environmental factors: Intrauterine exposure to Drugs, illnesses, alcohol or tobacco Vitamin deficiencies. Associated with syndromes like van der Woude's syndrome.

Development of the upper lip Starts in the 4th week of gestation and is completed by the 7th week. fusion of the maxillary prominences with the lateral and medial nasal prominences. Facial processes are separated by grooves. Merging is gradual filling of the groove until it smooth out.

Pathogenesis of Cleft Lip Failure of mesenchymal migration to unite one or both of the maxillary prominences with the medial nasal prominences results in a unilateral or bilateral cleft of the lip, respectively.

Classification of Cleft Lip Bilaterality: Unilateral cleft lips Bilateral cleft lips (10%) Relation to ala of the nose Incomplete Complete Mixed (if Bilateral) The relation of cleft lip to cleft palate Isolated Cleft lip(20%). Isolated Cleft palate (30%) (more common in females). Combined Cleft lip and cleft palate together (50%).

Unilateral complete: The cleft reach to the ala of the nose. Unilateral cleft lip one cleft starting from the vermilion border of the lip Unilateral complete: The cleft reach to the ala of the nose. Unilateral incomplete: The cleft stop shortly before the ala of the nose.

Bilateral cleft lips 2 clefts (right & left on either side) starting from the vermilion border of the lip Bilateral complete: reach to the ala of the nose. Bilateral incomplete: Does not reach to the ala of the nose.

Bilateral mixed cleft lips formed of one complete on one side and the other incomplete on the side.

Complications of Cleft Lip To overcome the complications surgery is usually performed during The first 3 to 6 months to repair cleft lip between 9 and 14 months to repair the cleft palate. Associated other congenital malformations. Oral: Defects in speech, mastication and deglutition. Ear: Middle ear fluid collections, hearing loss, and speech defects. Dental: missing, extra, malformed, or displaced teeth.

3- Van der Woude’s syndrome Autosomal dominant High penetrance lower lip pits, cleft lip with or without cleft palate.

Extraoral manifestations of Van der Woude’s syndrome Rare & include limb anomalies popliteal webs Brain abnormalities. Accessory nipples congenital heart defects Hirschsprung disease have been reported.

4- Cheilitis Glandularis Inflammation of the lower lip causing it to become enlarged and everted. Etiology: Genetic predisposition. Injury to the lower lip vermilion border and mucosa due to; Chronic lip biting Lip drying Habitual lip licking Combined with sun-induced lip damage.

Pathogenesis of Cheilitis Glandularis Eversion, ulceration, and secondary infection. The mucous glands and excretory ducts of the lip are dilated. The disorder is subclassified into 3 types: Simple (inflammatory changes in ducts of minor salivary glands), Superficial suppurative (swelling of lip & crusting) Deep suppurative (multiple abscesses with fistulas & scaring), is referred to as cheilitis apostematosa.

Clinical Features of Cheilitis Glandularis Usually affects adult males. 4th and 7th decades of life. Enlargement and eversion of lower lip. The minor salivary glands of the inner side of the lip get enlarge with dilatation of their ducts. Eversion of the lip increase susceptibility to actinic damage. Predisposing factor for the development of lip cancer.

Clinical Features of Cheilitis Glandularis

Histopathological Features of Cheilitis Glandularis Inflammatory changes in minor salivary glands (Multiple abscesses with scaring).

5- Cheilitis Granulomatosa Chronic swelling of the lip due to non-caseating granulomas in the lamina propria. The cause is unknown. Melkersson-Rosenthal syndrome Genetic Associated Facial palsy Fissured tongue.

Clinical Features of Cheilitis Granulomatosa The earliest manifestation is sudden diffuse or nodular swellings of the lip or the face Non-tender swelling and enlargement of one or both lips It may feel soft, firm, or nodular on palpation. Sites: The upper lip, the lower lip, and one or both cheeks. The forehead, the eyelids, or one side of the scalp may be involved (less common).

Clinical Features of Cheilitis Granulomatosa

Histopathological Features of Cheilitis Granulomatosa Non-caseating granuloma: macrophages “epitheliod cells”, lymphocytes, fibroblasts & collagen fibers.

Differential Diagnoses of Cheilitis Granulomatosa Dental abscess or infection Trauma Orofacial granulomatosis/Crohn disease Sarcoidosis Foreign body reaction Lymphoma Angiodema Acquired Hereditary Leprosy Lymphoma Tubercuosis Sarcoidosis Fungus

Main Points Discussed in this Lecture Fordyce’s granules: etiology, clinical & microscopic features. Congenital lip pits & fistulas: etiology, clinical features & their relation with Van der Woude’s syndrome. Cleft lip: etiology, pathogenesis, classification, its relation with cleft palate & complications. Cheilitis glandularis: etiology, pathogenesis, clinical & histopathological features. Cheilitis graulomatosa: etiology, pathogenesis, clinical & histopathological features.