A Message from a Dentist and a Moebius Dad
by Greg Osborne, DDS
There are many considerations to contend with in maintaining and correcting the dentition of patients having bilateral facial diplegia or Moebius syndrome. My information on this subject comes from reviewing the sparse dental literature on Moebius syndrome and listening to Barry Grayson, D.D.S., Assoc. Professor of Clinical Surgery (orthodontics) at New York Univ. Medical Center at the Second National Conference of the Moebius Syndrome Foundation. Since I have observed and treated our daughter who has Moebius syndrome, I felt compelled to summarize what I have learned and will continue to learn so that those born with or others involved with family members with Moebius Syndrome might benefit. I am a general dentist in a private family practice in San Antonio, Texas. I have spent most of my post graduate continuing education classes in studying orthodontics and orthopedics, having graduated from the University of Texas Health Science Center Dental School in San Antonio in 1981.
The following information is intended to help someone understand more about the general dental concerns of a person with Moebius syndrome. Each person is different and, of course, more specific information is best found in consultation with a dentist who has evaluated the patient. As Dr. Grayson mentioned, most of the dental concerns for the person with Mobius syndrome are basically the same as all people. In summarizing these concerns, he divided the information into four categories related to the normal growth patterns seen in the human dentition: 1) the Neonatal Period: from birth to the eruption of the first tooth, 2) the Primary Dentition: from eruption of the first tooth until the eruption of the first permanent tooth, 3) the Mixed Dentition: from the eruption or the first permanent tooth to the loss of the last primary tooth, 4) the Permanent Dentition: from the loss of the last primary tooth until the eruption of the third molars (wisdom teeth) and thereafter.
1) Neonatal Period: When a child is born with Moebius syndrome, there may be difficulty in closing the mouth or swallowing. The tongue may be hypotonic (low muscle tone) or faciculate (quiver). The tongue may be larger or smaller than average. There may be low tone of the muscles of the soft palate, pharynges, and the masticatory system. The palate may be arched excessively (a high palate) since the tongue does not form a suction that would normally shape the palate down further. The palate may have a groove (this may be partially due to intubation early on if it is for an extended period of time) or may be incompletely formed (cleft palate). The opening to the mouth may be small. Feeding problems may become a critical issue early on if adequate nutrition is difficult. If the infant can close and suck but needs assistance, the Mead-Johnson Soft Bottle can help. If the infant can suck just minimally, the SpecialNeeds® Feeder from Medela can be used to express milk for the baby. If the infant tends to aspirate, a gastrostomy feeding tube may be required. Often this is in combination with the tracheostomy breathing tube if the infant cannot control salivary secretions.
2) Primary Dentition: The primary (baby) teeth generally have started coming in by the first birthday and all 20 teeth may be in by the second birthday. The eruption timing varies a lot. There may be an incomplete formation of the enamel on the teeth called enamel hypoplasia that makes the teeth more vulnerable to caries (cavities). There may be missing teeth. The lower jaws become more noticeably deficient (micrognathia or retrognathia) if the infant is not closing down properly. The front teeth may not touch when the child closes down because the back teeth have overerrupted. This condition is called an anterior open bite and has facial/skeletal implications. The saliva may be thick or the infant may have a dry mouth. As soon as the first tooth comes in the parent can start to gently brush the teeth each day. Minimal toothpaste is needed or just a wet toothbrush is acceptable if the child tends to aspirate. As more teeth come in, it becomes important to start flossing between teeth. Babies should not be “put to bed” with a bottle of milk or juice as the residual nutrients in the mouth allow bacteria to proliferate and cause a condition called Bottle Caries. The first appointment with a dentist should come by age one and a half or two. This should probably be with a pediatric dentist, though there may be a need for a dental team to be involved as the child grows. The team may include the general dentist, orthodontist, and oral and maxillo-facial surgeon. Examinations and professional cleaning should be performed at least every six months. Fluoride treatments can be applied in the office or it may be prescribed as a gel to be applied at home. In addition, fluoride tablets or drops may be prescribed if the patient lives in an area that does not have an optimal amount in the water supply to help prevent dental caries. Sealants can be applied to the deep grooves of the back teeth as another prevention method that is very effective.
3) Transitional Dentition: Between age 5 and 7 most children start loosing their primary teeth. Occasionally some primary teeth are slow to exfoliate (fall out) and the dentist will have to remove that tooth. There are other reasons that a dentist may want to remove a primary tooth early to prevent orthodontic problems. Likewise, premature loss of primary teeth may create orthodontic problems later on. Removable or fixed spacers may be needed to prevent the shifting of teeth when a tooth is lost prematurely. Interceptive orthodontic treatment may be initiated at this stage of development to help with crowding or to help relate the upper and lower jaws. Consistent with a high palate is a narrow arch shape of the upper teeth as they line up in the mouth. This may cause the upper front teeth to flare out and become more prone to fracture if accidentally hit. Interceptive orthodontics has an important role in this situation. Appliances that expand the upper arch tend to bring the front teeth back into a more normal position. Some appliances can even help allow the front teeth to close to normal in an open bite situation. The mouth and lips may tend to get dry with the Moebius patient. Lack of a good oral seal (lips together) allows the gingiva (gums) to get dry and may get inflamed and irritated. Oral products such as Oral Balance, Xerolube, or Salvert Spray may be helpful.
4) Permanent Dentition: After the last primary tooth is lost, usually around age twelve, final orthodontic treatment can be initiated. A patient that has not been able to close or swallow well, probably will have an open bite, deficient lower jaw growth, a narrow archform with crowded teeth and upper anterior flaring of teeth. Orthognathic (jaw) surgery may be indicated. This should be completed in most situations before someone like Dr. Zuker would do the smile surgery where the Gracilis Muscle is grafted to the face. Good home care of the mouth and teeth includes brushing with a fluoride toothpaste and flossing (they make floss holders that are easier to hold). In addition water irrigation devises can be very helpful. Regular check-ups at the dental office also are important to help maintain a healthy mouth for a lifetime.
