Like most other mammals, humans have two sets of teeth during life. The first set of teeth are called primary (baby or milk or deciduous) and the second set permanent (these are the adult teeth).

Typical Dental Development

Humans typically have 20 primary and 32 permanent teeth. There are 20 teeth in the primary dentition – 10 in the upper jaw (maxilla) and 10 in the lower jaw (mandible). Each jaw holds four incisors, two canines and four molars.

The primary teeth are pushed out by a permanent tooth. The process begins around the age of 5 or 6 and continues until early teens.

Lower and upper teeth diagram

The primary molars are replaced by permanent premolars (bicuspids). The 8-12 adult permanent molars erupt (emerge from the gums) towards the back of the jaws; these adult molars do not replace any of the baby teeth, giving a total of 32 teeth in the permanent dentition. The permanent dentition is thus made up of four incisors, two canines, four premolars and six molars in each jaw. Some adults are missing the third molars (known as wisdom teeth) giving a total of 28 teeth.

Teeth and Dental Care in Ectodermal Dysplasia

One of the main characteristics of Ectodermal Dysplasia, and the one on which a diagnosis is frequently made or proven, is the absence of one or more teeth (hypodontia) and/or tooth malformation. Teeth that are present may be widely spaced, pointed, and in some cases the enamel is defective and discoloured. The teeth in people affected by Ectodermal Dysplasia often erupt later than usual and children with this condition may not have any teeth emerge until they are at least two years old.

It is not unusual for a general dental practitioner to have had very little experience of patients with Ectodermal Dysplasia. Fortunately, specialists in paediatric dentistry and in restorative dentistry are trained in the management of the condition, and those in other specialties or with specific skill sets may have acquired such expertise.  

Some dental hospitals have specialist teams to help patients affected by hypodontia, who can provide a coordinated treatment plan involving all the necessary experts. Ask your dentist about being referred to one if this facility is available in your area. If it is not then some hospitals can, on occasion, treat Ectodermal Dysplasia patients from other regions. Ask to be referred to your nearest clinic for an assessment. The ED Society maintains a list of specialist clinics on their web site www.edsociety.co.uk

Teeth are important for nutritional, cosmetic, psychological and social reasons. Should a child have pointed upper front teeth, and wish this to be changed, then it is usually relatively easy for the dentist to build them up with an adhesive tooth-coloured material (dental composite). This can help to produce a more typical appearance.

Missing teeth may be replaced using removable appliances (dentures) from childhood onwards, while bridgework and/or implants may be considered in older individuals. Not all of these may be suitable in a given situation or at a particular time, some may be used sequentially (i.e. dentures until they are old enough for implants), and it is important to have specialist advice before embarking on a lengthy treatment plan.

The dentist’s chair may become a place where your child will spend a lot of time. It is important for the dentist to get to know your child and vice versa before any treatment is commenced. Make sure your dentist is prepared to spend time with your child to build up a rapport, to explain about the room and the tools, to show how the chair works, what he/she is going to do and most importantly, to have some fun.  Of special importance is the ability of the child to feel in control of his/her care.

Helping Your Child Enjoy Healthy Teeth for Life

It is important to remember that whatever primary or permanent teeth erupt become precious. A tooth, no matter what shape or size, can be used by your dentist to help with replacement of missing teeth. Primary teeth, especially canine (eye) teeth, may last for several decades if there are no adult teeth to replace them (no tooth to dissolve the root and push them out). Hang on to whatever teeth grow and take extra special care of them. If a non-specialist dentist recommends tooth extraction, for whatever reason, seek a second opinion.

The following list of “Top Tips for Teeth” may be helpful for parents:

  • Commence toothbrushing upon eruption of the first tooth
  • Due to the high caries risk it is recommended to use a rice grain-sized amount of toothpaste containing a fluoride (ideally minimum concentration of 1450ppm, this is shown of the back of toothpaste packaging) as soon as teeth erupt. Please visit https://www.nhs.uk/conditions/fluoride/ for more information regarding fluoride.
  • Brush at night and at one other time during the day
  • Don’t rinse the toothpaste away
  • Only give milk or water to drink
  • Do not allow baby to sleep with a bottle of milk in the mouth
  • Discontinue bottle use, moving to open cup around age 1
  • Give three main meals and two snacks per day once established on solids
  • Access primary dental care regularly
Missing teeth (hypodontia), pointed teeth, small teeth (microdontia).

Some of the possible effects of ED on teeth; Missing teeth (hypodontia), pointed teeth, small teeth (microdontia), retained primary teeth and tooth wear. The number of missing teeth varies with larger numbers being less common.