Congenital abnormalities of the tongue , mouth and oral cavity

Congenital facial anomalies of

Mouth

Jaw

Tongue.

Outline :

• Introduction
• Jaw anomalies
• Lip anomalies
• Tongue anomalies
• Oral Cavity anomalies

• Congenital anomalies :
• Errors of embryogenesis

( malformation )

• Events affecting embryonic or fetal growth

( deformities , disruptions )

• The more complex the formation of a structre the higher risk of malformation

Jaw anomalies

Mandibular clefting

Microganthia

• Robin sequence

Microganthia

• Retarded growth of the mandible

Results in a tongue tha is disproportionatly large

in relation to oral cavity

• Effects

Neonates ---> upper respiratory tract obstruction

Older children --> cosmetic

Robin sequence

Triad of :

• Microganthia
• Glossoptosis
• U shaped palate

Can occure in isolation or with other complex syndromes

Associated with :

• GERD
• Conductive hearing loss

Treatment :

• Conservative :
• Prone position + gavage feeding
• +/- Nasal trumpet
• Surgical :
• Indications :
• Failure to thrive Or Hypoxemia
• Precedures :
• Tracheotomy
• Mandibular distraction osteogenasis :
• Promising results in experienced hands

• Clefting
• Microstomia
• Macrostomia
• Lip pits
• Labial frenula
• Synaechiae

Classification :

• Paul Tessier

Microstomia

MAcrostomia

Lip bites

Lip fernula

Lip Synaechiae

• Cysts
• Mucoceles
• Macroglossia
• Ankylogolssia
• Lingual thyroid
• Medial rhomoid glossitis

Ankyloglossia

Cysts and pseudocysts :

• Mucoceles
• Ranulas
• Bohn’s nodule

Congenital tumors

• Choristoma
• Eplulies and other granular cell tumors

Treatement :

• Complete excision( preffered )
• Marsupialization and suturing of the pseudocyst wall to the oral mucosa may be effective
• if complete excision cannot be performed.
• Sclerotherapy with OK-432 (picibanil) has also been successful

B- Congenital tumors.

• Teratomas and epithelial choristomas are the most common.
• In addition, granular cell tumors (eg, epulis, myoblastoma)

We are almost done

Summary

Congenital anomalies of the jaw include clefting and hypoplasia of the mandible (micrognathia).

In the neonate, micrognathia may cause upper respiratory obstruction.

Facial clefts occur when normal development of the fetal face and cranium is interrupted

.

The precise mechanism of facial clefting is uncertain. Multiple environmental and genetic factors may contribute.

According to the Tessier classification, facial clefts are numbered from 0 to 14

Congenital anomalies of the lips include clefts, microstomia, macrostomia (transverse facial cleft), lip pits, abnormalities of the labial frenula, and synechiae.

Congenital anomalies of the tongue include cysts, mucoceles ,macroglossia, ankyloglossia , and lingual Thyroid .

Congenital anomalies of the oral cavity include cysts and pseudocysts (mucoceles, ranulas, Bohn nodules and congenital tumors (eg, choristoma and epulis)

Thank you for your attention!

Introduction :

Oral cavity anomalies

Mucoceles

Foramen cecum mucocele

a-Cysts and pseudocysts

Pseudocysts of minor salivary gland origin .

Present as smooth swelling in the buccal mucosa .

Treatement :

• Unroofed or Marsupialized
• Preferably, excised
• with adjacent minor salivary glands

• Originate from salivary glands

• Most common anomalies in the oral cavity

• Thyroglossal duct cysts are found in the floor of the mouth and may be mistaken for ranulas

Failed mandibular fusion

Ranulas

Pseudocysts associated with the sublingual glands and submandibular ducts.

Presentation :

• Blue, fluctuant swellings lateral to the midline in the lower mouth
• IF large  Neck masses
• if they extend through the mylohyoid musculature of the floor of the mouth

Bohn nodules

Inclusion cysts

Single or multiple white or translucent round papules.

symptomatic but may cause concern when first noticed by the parents

No Treatement is necessary since they resolve spontaneously over weeks to months

Christoma

Epulis and other granular cell tumors

Arise from the mucosa of the gingiva,

Present as

• a mass protruding from the mouth
• may interfere with feeding or breathing.

They are uniformly benign and treated with simple excision.

Epulis occurs exclusively in newborns

• Girls are affected 8 to 10 times more often than boys.
• The lesions range in size from a few mm to 8 cm

Mass of normal cells in an abnormal location (ie, heterotopia )

Presents as :

• Heterotopic gastric mucosa
• Enteric duplication cysts
• Heterotopic neural tissue
• Ectopic cartilage and bone

May cause feeding difficulty and airway obstruction

Surgical excision is required

CT and MRI are helpful in defining the extent of the lesion and preoperative surgical planning

Supervised by :

• Dr Mohammad Al-Essa

Micro = Small …. Stomia = mouth

Complete union of the upper and lower lips astomia

Microstomia is Seen in :

• Severe forms of holoprosencephaly
• Valporate syndrome
• Trisomy 18
• Hallermann-streiff syndrome

Lip anomalies

?

Also called transverse facial cleft

Seen in

• Angelman , Morqio , Noonan, Backwith-Wiedemann , Recher Collini and Williums Syndromes.

Surgical correction may require the creation of new oral commissure .

?

Clefting

More common in lower lip

Autosomal dominant in Van Der Woude and other syndromes

Presents as :

• Lower lip bilateral paramedian depression of the vermillion boarder .
• represent small accessory salivary glands and often secretes mucous .

?

Epidemiology :

• Upper lip more common than lower
• 1.4-4.9 / 100,000 live births

Pathogenasis

• Many theories  classical
• Distruption of fusion process

Aetiology :

• Environmental and genetic :
• Eg : Antenatal : radiation , teratogenicity , viral infections , metabolic

Occures in upper and lower lips

If presented during dental eruptions will result in :

• Widely spaced central incisors
• Periodental disease and bone loss

?

Congenital synachiae :

• Found between hard palate and the floor of the mouth , the tongue or oropharynx

Treatement :

• Surgical division .

Treatement :

Complex and must be individualized to the clinical case.

Goal :

• Restore compromised skin , soft tissue and bone
• Reestablish functional and aesthetic qualities to face and cranium

Tongue Anomalies

Median rhomboid glossitis

Lingual Thyroid

Macroglossia

Mucous membrane under the tongue is too short limiting the mobility of the tongue.

Clinical Features :

• Feeding problems
• Speech problems
• Mechnical problems
• Eg .( Lick lips , or remove food depress from teeth )
• Abnormalities in lifting , sidemovment and protrusion of the tongue .
• Heart shaped tounge on protrusion

Well demarcated , depapillated ,pink to plum-colored patch on the surface of the tongue .

Rhomboid in shape ( 0.5-2 cm )

Most found anterior to the foramen cecum

Presentation :

• Pain, Irritation or pruritus
• Also A/W candida infection and DM

May be present at foramen cecum

Diagnosis :

• Radionuclide scanning :  thyroid tissue +/- other functional thyroid tissue

Treatement :

Only indicated incase of airway obstruction

Why ??

• May represent the only functional thyroid tissue
• Mass can be reduced using thyroid hormon

Big tongue

• Glossoptosis big tongue in relation to oral cavity

Two types :

• Focal :
• Congenital tumours
• (Lymphangioma / haemangioma )
• MEN 2B
• Generalized :
• Smooth ( Backwith-Wiedernabb Syndrome/ Hypothyroidism)
• Multi-nodular appearance other causes