What Is Moebius Syndrome?see photo In 1892, a German physician named Moebius described a syndrome whose findings included muscle weakness on both sides of the face, an inability to move the eyes from side to side, and hand deformity.
This collection of findings was latter called Moebius syndrome.
GENERAL FEATURES OF MOEBIUS SYNDROME:
1. Muscular weakness on both sides of the face, due to facial nerve paralysis.
Children with Moebius syndrome have difficulty showing facial expression.
2. Inability to move the eyes from side to side.
This paralysis of the lateral rectus eye muscle is caused by palsy of the abducens nerve.
3. Congenital deformities of the limbs, such as club foot, webbed fingers (syndactyly), or other deformities of the hand or foot.
4. Mild mental retardation in 10 percent of affected children.
5. Occasional anomalies of the ears.
ORAL FEATURES OF MOEBIUS SYNDROME:
1. Muscular weakness on both sides of the face, due to facial nerve paralysis.
This leads to difficulty in eating, swallowing, and clearing food and liquids from the oral cavity.
2. Poor muscle tone of the tongue, or quivering of the tongue, due to paralysis of the tongue's (hypoglossal) nerve.
3. A small lower jaw (micrognathia).
4. A high arched palate, due to poor tongue function.
5. Difficulty with feeding.
A tendency to choke on food or liquids.
6. Speech impediments.
7. Poorly formed enamel on the teeth (enamel hypoplasia).
POSSIBLE CAUSES OF MOEBIUS SYNDROME:
1. Insufficient blood supply to the oral and facial structures, which are nourished by the developing primitive subclavian artery, in the fetus.
2. Unfavorable intrauterine posture of the fetus, in which pressure from the shoulder of the fetus results in significant pressure on the facial nerve - resulting in facial muscle weakness.
3. Intrapartum (during delivery) pressure in which the head of the fetus is pushed against a bony prominence of the mother, such as the pubic rami.
This can lead to damage of the child's facial nerve.
4. Any interference with normal fetal circulation or circulation in the uterus (any transient ischemic insult).
5. Poor development (hypoplasia), lack of development, or degeneration - of the central brain nuclei.
MEDICAL TREATMENT FOR CHILDREN WITH MOEBIUS SYNDROME:
1. Certain surgical procedures aim to relieve the facial paralysis in affected children.
The surgeon transfers functioning muscle tissue from an unaffected part of the body to the affected area of the face.
This surgical procedure helps to animate the face.
ORAL CARE FOR CHILDREN WITH MOEBIUS SYNDROME
1. Parents can modify oral hygiene techniques, depending on the child's age. For small infants, the gums need to be cleaned once or twice a day with a piece of clean gauze. This will help to establish a healthy oral environment for the baby teeth. Infants should be introduced to the toothbrush around the age of one.
2. Parents should not put children to sleep with a bottle containing any liquid other than water. Parents should encourage their infants to begin drinking from a cup around their first birthday.
3. Parents should help brush their children's teeth every day, after every meal.
4. Parents should not let their children drink fruit juice or sweetened drinks from a bottle or "tippy" cup, since this prolongs the exposure of teeth to harmful sugar.
5. Parents should provide healthy, balanced meals for children. They should limit the amount of sugar-laden foods and snacks in their diet. Plenty of healthy snacks should be available for children. Cheese products actually fight dental caries.
6. Parents can help make children's teeth more decay-resistant by using an ADA-approved children's toothpaste. Place only a pea-sized drop of toothpaste on the toothbrush. Until a child is 3 years old, parents should only use baby tooth cleanser - to avoid causing fluorosis discoloration of the adult teeth.
7. Children taking oral medications should have their teeth cleansed after each dose of medication. Nearly 100% of children's medications contain sucrose, which can increase the risk of developing dental caries.
8. Children should have their first oral/dental health evaluation by the age of 12 months, or within 6 months of the eruption of the first tooth.
A recent study published in Archives of Disease in Childhood investigated "the role of neuroanatomical abnormality as a cause of unilateral congenital facial palsy."
Congenital facial palsy is one of the major symptoms of Moebius syndrome.
The study concluded that developmental abnormalities of the facial nucleus (located in the pons region of the brain) "constitute a important cause of monosymptomatic, unilateral, congenital, facial palsy."
Jemec B, Grobbelaar A, Harrison DH: The abnormal nucleus as a cause of congenital facial palsy.
Archives of Disease in Childhood. Sept 2000. 83(3) 256-258.
The Mouth Is A Window To Healthsee photo Oral health in children cannot be separated from overall health.
A number of children's medical conditions have oral sign and symptoms.
In some cases, the first indication of a health problem will appear in the mouth.
SIGNS OF BEHAVIORAL DISORDERS:
1. Bulimia is accompanied by: the loss of dental enamel due to acidic stomach contents. The swelling of the salivary (parotid) glands is due to frequent vomiting.
2. Psychiatric medications can cause: a decreased flow of saliva, which increases the risk for dental caries.
SIGNS OF BLOOD DISORDERS:
1. Neutropenia is accompanied by: severe gingivitis and occasional tooth loss.
One of the first lines of defense against bacterial infections are the neutrophil cells.
In neutropenia, there is a decrease - or total absence - of these important cells.
As a result, children affected by neutropenia have periodontal infections, with early loss of teeth.
2. Platelet disorders are accompanied by: bleeding of the gingiva, as well as bleeding of the soft tissue in the mouth (the oral mucosa).
3. Hemophilia is accompanied by: an increased incidence of bleeding of the mouth and gingiva, especially after oral trauma.
4. Sickle cell anemia is accompanied by: an increased risk of bodily (systemic) infection after dental treatment.
Sickle cell disease is chronic illness, punctuated by multiple crises, which can become life-threatening at any time.
A sickling crisis in children can be caused by lack of oxygen in any part of the body, dehydration, infection - which can lead to acidosis, fatigue, or stress.
Death in children who have sickle cell disease can be caused by infection, acute chest syndrome, splenic sequestration, or other causes.
SIGNS OF CANCER:
1. Cancer and chemotherapy are accompanied by: frequent oral infections, spontaneous oral bleeding, and poor healing of oral infections.
Chemotherapy causes ulceration of the mouth and facilitates opportunistic infections.
2. In acute leukemia, malignant white blood cells invade the gingival tissue, causing extensive bleeding and loosening of teeth.
SIGNS OF DIGESTIVE TRACT PROBLEMS:
1. Crohn's disease is accompanied by: a cobblestone appearance of the oral mucosa, persistent lip swelling, vertical fissures on the lips, redness around the mouth (perioral erythema), enlargement of the lymph nodes (cervicofacial lymphadenopathy), recurrent oral stomatitis (pyostomatitis vegetans, pseudo-pyostomatitis vegetans, stomatitis gangrenosum, chronic stomatitis, oral staphylococcal mucositis), epithelial folds, enlargement of the gums (gingival enlargement), linear aphthous ulceration, and redness of the gums (gingival erythema).
2. GERD is accompanied by: chemically eroded primary and permanent teeth.
Dental erosion is the demineralization of enamel and dentin caused by chemicals, such as gastric acid.
In GERD the acidic gastric (stomach) contents travel up into the oral cavity.
SIGNS OF HORMONE DISORDERS:
1. Addison disease is accompanied by: the appearance of bluish-black patches in the mouth.
2. Diabetes mellitus is accompanied by: an increased risk of gingival infections, periodontal infections, dental infections, and yeast (candia) infections of the mouth.
Children with poorly controlled diabetes are at increased risk for periodontal disease due to a number of factors.
These factors include: impaired neutrophil function, microvascular abnormalities, and altered collagen metabolism.
3. Hypophosphatasia is accompanied by premature exfoliation (loss) of all primary teeth in 75% of affected children.
In this genetic disease, the low levels of serum alkaline phosphatase impairs the health and function of the suspensory ligaments, which hold the teeth in place.
4. Papillon-Lefèvre syndrome is accompanied by: by palmoplantar hyperkeratosis and severe early onset periodontitis that results in the premature loss of the baby and adult teeth.
The absence of an important enzyme, cathepsin C, is responsible for the premature loss of all teeth in children afflicted with the rare Papillon-Lefèvre disease.
The type of gum disease associated with this syndrome begins very early in life, usually before age 3, and is very severe.
SIGNS OF INFECTIOUS DISEASES:
1. Scarlet fever is accompanied by: sore throat, a pale color around the mouth (circumoral pallor), red palate, and a "strawberry tongue."
2. German measles (rubella) is accompanied by: small red spots on the palate (Forscheimer spots) which appear before the body rash.
3. Mumps are accompanied by: enlarged cheeks due to swelling of the parotid glands on both sides of the face.
4. Herpes Simplex Virus is accompanied by: intraoral blisters during the first infection.
Subsequent attacks by HSV affect areas outside of the mouth, such as the lips and face.
5. Chicken pox (due to the varicella zoster virus) is accompanied by: vesicles and blisters inside of the mouth.
6. Herpangina is accompanied by: painful vesicles on the throat and tongue.
7. AIDS is accompanied by: candida (yeast) infections, gingival inflammation, and mucositis.
SIGNS OF NUTRITIONAL PROBLEMS:
1. Niacin deficiency (pellagra) is accompanied by: an inflammation of the tongue surface (glossitis).
The tongue appears enlarged and red, and is painful.
2. Vitamin B6 and vitamin B12 deficiency is accompanied by: glossitis, aphthous ulcers, and an inflammation inside of the mouth (stomatitis).
SIGNS OF RESPIRATORY PROBLEMS:
1. Asthma is accompanied by: dry mouth (xerostomia), which poses an increased risk for caries development.
The decreased salivary flow is caused by the action of B-2 agonists (Proventil) on salivary gland function.
The gums (gingiva) are usually red (erythematous).
2. Halitosis in children may be caused by: enlarged tonsils and respiratory infections.
SIGNS OF SEIZURE DISORDERS:
Seizure disorders are accompanied by: enlarged gums (gingival overgrowth) due to the effects of certain seizure medications.
SIGNS OF DERMATOLOGIC DISORDERS:
Erythema multiforme is accompanied by: intraoral blisters and extraoral target lesions.
The target lesions of the skin resemble a bulls-eye, with 3 colors.
Erythema multiforme can be caused by an antigenic stimulus, such as penicillin, or a sulfa-containing drug.
A recent article in JAMA reviewed the effects of oral disease on overall health.
It discussed dental disease, the risk of heart disease due to dental infections, and the links between oral and general health.
Slavkin HC, Baum BJ: Relationship of dental and oral pathology to systemic illness.
JAMA. Sept 13, 2000. 284(10)1215-1217.
The Protective Nature Of Salivasee photo What good is saliva?
It's messy, and most people don't like it.
Actually, saliva cleans and protects our teeth,
helps fight oral infections, and helps us to chew and swallow our food.
Many salivary protective factors work together to protect our
oral health.
Saliva is produced by the major and minor salivary glands.
The major glands include the parotid, submandibular, and sublingual
glands.
The sublingual gland produces mostly thick mucus.
The minor salivary glands are found just below the surface of the
soft (mucosal) tissue of the mouth and lips.
WHAT CAN CAUSE DRY MOUTH IN CHILDREN?
1. Children affected by severe dry mouth (xerostomia) experience
a great increase in dental caries, difficulty with swallowing, and
inflammation of the oral tissues (mucositis).
2. Head and neck radiation treatment can destroy the saliva-producing acinar
cells, greatly increasing the risk of future dental caries.
3. Psychiatric medications, such as tricyclic antidepressant drugs, can
cause a decreased flow of saliva, which then increases the risk of
dental caries.
4. Asthma is accompanied by dry mouth (xerostomia), which poses an
increased risk for caries development.
This decreased salivary flow in asthmatic children is caused by the
action of B-2 agonists, such as Proventil, on salivary gland function.
The gums (gingiva) are usually red (erythematous).
Let's look now at some of the main functions of saliva.
PROTECTING THE TEETH FROM ACID ATTACK:
1. Bicarbonate in saliva acts to buffer teeth from the
destructive effects of the acid which is produced by the
cavity-causing (cariogenic) bacteria.
2. Any medical problem which causes a reduction in the flow of saliva
will therefore cause an increased risk of dental caries.
3. Proline-rich proteins are produced by the parotid gland, and
they protect dental enamel by binding to it and protecting it.
Since proline-rich proteins also bind to bacteria, cariogenic bacteria
can't attach to the enamel and cause damage.
4. Mucins are large molecules (glycoproteins) which also protect
the teeth from the destructive acids produced by cariogenic bacteria.
PROTECTION AGAINST BACTERIA:
1. Lysozymes are enzymatic proteins in the saliva which can kill
the bacteria that cause dental caries (mutans streptococci).
2. Salivary IgA is also important, and it is the main antibody found
in saliva.
It is produced by special cells (plasma cells) located in both the
major and minor salivary glands.
The secretory form of IgA prevents certain strains of bacteria from
attaching to enamel, which thereby protects the teeth from bacterial
attack.
PROTECTION AGAINST FUNGAL INFECTIONS:
1. Histatins are some of the smallest proteins found in saliva, and
protect against fungal infections.
The histatins inhibit the growth of candia, an oral fungal infection.
HARDENING OF TOOTH ENAMEL:
1. Statherin is a small protein found in saliva which helps harden
and remineralize the teeth.
It is also a lubricant for teeth.
2. Histatins act to maintain the strength of the enamel (hydroxyapatite)
crystals on the surface of the enamel.
This helps to protect against dental caries.
A previous study in Pediatric Dentistry
investigated whether hyperglycemia in children with diabetes mellitus
could interfere with salivary secretion rates, salivary glucose levels,
and salivary microbial counts.
The study demonstrated that hyperglycemia was associated with decreased
salivary flow rates.
As a result, there was an increase in salivary lactobacilli and yeast
counts
Karjalainen KM, Knuuttila MLE, Käär M: Salivary factors in
children and adolescents with insulin-dependent diabetes mellitus.
Pediatric Dentistry 1996 (18)4 306-311.
