Читать книгу Color Atlas of Oral Diseases in Children and Adolescents - George Laskaris - Страница 11
Оглавление2 Developmental Anomalies
Orofacial Clefts
Definition
• Orofacial clefts are developmental malformations of multiple tissue maturation processes of the oral cavity and face.
Etiology
• The etiology remains obscure. Genetic and environmental factors may be related to isolated defects.
• Several developmental syndromes may be associated with orofacial clefts.
• In 15–30% of patients, there is a family history of similar congenital defects.
Occurrence
• The prevalence of cleft palate varies from 0.29 to 0.56 per 1000 births in children, while the prevalence of cleft lips is about one per 9000 births.
• A considerable ethnic association with the prevalence of orofacial clefts has been reported.
• Cleft lip and cleft palate occur in 0.52–1.34 per 1000 births.
Localization
• Lips, hard and soft palate, uvula, maxilla, mandible.
Clinical features
• Cleft lip is characterized by a defect that usually involves the upper lip (Figs. 2.1, 2.2). About 75–80% of cleft lips are unilateral, and the left side is more often affected than the right. Isolated cleft lip occurs less often than a combination with cleft palate and cleft jaw defects. Missing and, rarely, supernumerary teeth may be observed.
• Cleft palate is characterized by a defect in the midline of the palate that varies in severity and may involve the hard palate, soft palate, or both (Fig. 2.3).
• Maxillary anterior alveolar clefts are characterized by a bony defect in the maxilla, usually between the central and lateral incisors (Fig. 2.4).
• Bifid uvula is a minor expression of cleft palate, and may occur alone or in combination with more severe defects (Fig. 2.5).
Treatment
• Plastic surgical repair. Prosthetic and orthodontic appliances are also necessary.
Fig. 2.1 Cleft lip, bilateral
Fig. 2.2 Cleft lip, bilateral
Fig. 2.3 Cleft lip and palate
Fig. 2.4 Maxillary anterior alveolar cleft
Bifid Tongue
Definition
• Bifid tongue is a developmental malformation of the tongue caused by lack of fusion of the lateral halves and resulting in cleavage of the tongue.
Occurrence
• Rare.
Localization
• Tip of the tongue, midline of the dorsum of the tongue.
Clinical features
• The complete form is very rare, and may result in the formation of two complete tongues.
• The incomplete form appears either as an asymptomatic deep groove in the midline of the dorsum of the tongue, or as a double ending of the tip of the tongue (Fig. 2.6). Neither form has any clinical significance.
• Bifid tongue may coexist with the orofaciodigital syndrome.
Treatment
• The incomplete form requires no treatment.
• The complete form needs surgical reconstruction.
Ankyloglossia
Definition
• Ankyloglossia or tongue-tie is a developmental malformation in which the tongue is abnormally fixed to floor of the mouth or lingual aspect of the gingiva, due to a short and malpositioned lingual frenulum.
Occurrence
• Rare. Approximately one per 1000 births.
Clinical features
• The lingual frenulum is short, thick or thin, and fibrous (Figs. 2.7, 2.8).
• The malformation may cause partial or complete immobility of the tongue.
• Mild cases are asymptomatic, and may go unnoticed for a long time. Severe cases may cause problems with speaking, eating and breast-feeding.
Treatment
• Surgical clipping of the frenulum in severe cases.
Fig. 2.5 Bifid uvula
Fig. 2.6 Bifid tongue
Fig. 2.7 Ankyloglossia
Fig. 2.8 Ankyloglossia
Lip Frenulum Anomalies
Definition
• Lip frenulum is a normal connective tissue structure extending from the lips to the alveolar process of the teeth.
Clinical features
• Rarely, the central frenulum of the upper and lower lips may be thick, broad and long, with an adhesion to the marginal gingiva.
• In severe cases, a large gap between the central incisors, or gingival regression or tooth movement may occur (Figs. 2.9, 2.10).
Treatment
• Surgical correction.
Congenital Lip Pits
Definition
• Congenital lip pits or paramedian lip pits are developmental invaginations, which may occur alone or in combination with commissural pits, cleft lip, or cleft palate.
Etiology
• They may be inherited as an autosomal dominant trait.
• They develop through incomplete regression of the lateral sulci of the lower lip during embryonic development.
Occurrence
• Rare.
Localization
• A few millimeters from the midline of the vermilion border of the lower lip, usually bilateral.
• Labial commissures.
Clinical features
• Clinically, they present as bilateral or unilateral depressions (Fig. 2.11).
• The size varies from 1 mm to 10 mm, and the depth of blind sinuses may extend to 1 cm or more.
• A small amount of mucous secretion may accumulate at the depth of the pits, and the lip may be enlarged and swollen.
• Occasionally, a fistula may coexist.
• The diagnosis is based on clinical criteria.
Treatment
• Surgical correction for cosmetic purposes.
Double Lip
Definition
• Double lip is a malformation that may be present at birth or later in life.
Etiology
• It may be congenital due to the failure of the pars glabrosa and the pars villosa to fuse along the horizontal sulcus during lip formation.
• Acquired double lip is one of the main components of Ascher’s syndrome (double lip, blepharochalasis and goiter).
Occurrence
• Rare.
Localization
• Often the upper lip, and less frequently the lower lip.
Clinical features
• Clinically, it is characterized by an asymptomatic, protruding horizontal fold on the mucosal surface of the lip.
• Typically, the double lip is visible during speech or smiling, or when the lips are tensed (Figs. 2.12, 2.13).
• The diagnosis is based on clinical criteria.
Treatment
• Mild cases require no treatment.
• Severe cases require surgical correction for cosmetic purposes.
Fig. 2.9 Thick and long central frenulum of the upper lip
Fig. 2.10 Thin and long central frenulum of the lower lip
Fig. 2.11 Bilateral congenital lip pits
Fordyce’s Granules
Definition
• Fordyce’s granules are a normal anatomical variation, characterized by the presence of ectopic sebaceous glands in the oral mucosa.
Etiology
• Developmental.
Occurrence
• They present in about 20–30% of children and adolescents.
• About 80% of adults have Fordyce’s granules.
Localization
• Upper lip, buccal mucosa, retromolar area, anterior tonsillar pillar.
Clinical features
• Clinically, Fordyce’s granules present as multiple yellow or whitish-yellow, slightly raised, tiny pinhead-sized spots that are well circumscribed (Fig. 2.14).
• Solitary enlarged Fordyce’s granules may also be seen (Fig. 2.15).
• The granules are asymptomatic, and come to the patient’s attention by chance.
• The diagnosis is usually based on clinical criteria.
Laboratory tests
• Histopathological examination reveals normal collections of sebaceous glands without hair follicles.
Differential diagnosis
• Candidiasis
• Lichen planus
• Leukoplakia
Treatment
• No treatment is required.
Exostoses
Definition
• Exostoses are developmental harmless bony overgrowths that may affect the jaws.
Etiology
• Unknown. Genetic and environmental factors may be involved in their development.
Occurrence in children
• Rare. The lesions are usually first noticed after 20 years of age.
Localization
• Midline of the hard palate (torus palatinus).
• Lingual surfaces of the mandible, usually in the premolar region (torus mandibularis).
• Buccal surface of the maxillary and/or mandibular alveolar ridge (buccal exostoses).
Clinical features
• Clinically, exostoses present as bony, hard nodules covered by normal mucosa (Fig. 2.16).
• The number and size of the lesions vary considerably.
• Rarely, the overlying mucosa may be ulcerated due to mechanical trauma.
• The lesions grow slowly, and are asymptomatic.
• The diagnosis is based on clinical criteria.
Treatment
• No treatment is required. Surgical excision is indicated only when it is necessary to fit a prosthesis.
Fig. 2.12 Double upper lip
Fig. 2.13 Double lower lip
Fig. 2.14 Multiple Fordyce’s granules on the buccal mucosa and retromolar area
Fig. 2.15 Solitary hypertrophic Fordyce’s granules on the buccal mucosa
Facial Hemiatrophy
Definition
• Facial hemiatrophy, or Parry–Romberg syndrome, is a degenerative disorder characterized by atrophic changes of the deeper structures (e.g. fat, muscle, cartilage, and bone) involving one side of the face.
Etiology
• Unknown.
Occurrence
• Rare. The disease usually starts in childhood, and girls are affected more frequently than boys (ratio 3:2).
Localization
• One side of the face, lips, tongue, maxilla, mandible, teeth.
Clinical features
• The affected side appears atrophic, and the skin is wrinkled, shriveled and often shows hyperpigmentation or hypopigmentation (Fig. 2.17). Rarely, bilateral facial atrophy may occur.
• Unilateral atrophy of the lips and tongue is the most common oral manifestation. Hypoplasia of the maxilla and mandible, delayed eruption of the teeth, and malocclusion may also occur.
• Enophthalmos, alopecia, sweat gland disorders, trigeminal neuralgia, facial paresthesia, and epilepsy may develop.
• The atrophic process progresses slowly for several years and then becomes stable.
• Diagnosis is based on clinical criteria.
Differential diagnosis
• Scleroderma
• Facial hemihypertrophy
• Lipodystrophy
Treatment
• Plastic reconstruction
• Orthodontic treatment, if there is malocclusion.
Fig. 2.16 Buccal exostoses of the maxilla
Fig. 2.17 Facial hemiatrophy. Atrophy of the right side of the face
