36: Multiple missing and abnormally shaped teeth

36 Multiple missing and abnormally shaped teeth

Case 1

Summary

Ellen is almost 11 years old. She is very concerned by the gaps between her upper and lower front teeth. She is hoping to become an actress. She is a new patient to your surgery and has been brought by her mother. What are the causes of the problem and how may it be treated?

History

Ellen is a regular attender with no caries. Her oral hygiene is excellent. There is no history of tooth extraction in either the primary or permanent dentitions.

Medical history

Ellen has no medical problems. She is an active girl who is involved in orienteering with her family as well as being in the local amateur dramatic society.

image What question do you need to ask Ellen’s mother?

Is there a family history of missing teeth or gaps?

As you are speaking to Ellen’s mother you notice that she has a missing upper right lateral incisor and the canine tooth is prominent. She confirms your question that not only has she got a missing tooth, but her brother and her nephew (son of her brother) have either missing or very small upper lateral incisors.

image How prevalent are missing teeth in the population?

0.1–0.9% in the primary dentition.
3.5–6.5% in the permanent dentition (discounting third molars).
More common in females, than males (1.4–4×). The most common teeth to be absent are the last teeth in each series (i.e. lateral incisor, second premolar, third molar). The presence of a conical (peg) tooth is frequently associated with a missing tooth on the opposite side of the arch. There is frequently a family history.

image There are a significant number of syndromes of the head and neck that manifest with missing teeth. Can you name some?

Ectodermal dysplasia.
Cleft lip and/or palate.
Down syndrome.
Chondro-ectodermal dysplasia (Ellis–van Creveld syndrome).
Reiger syndrome.
Incontinentia pigmenti.
Oro-facial-digital syndrome (types I and II).

Ectodermal dysplasia describes a group of inherited disorders involving ectodermally derived structures, i.e. hair, teeth, nails, skin and sweat glands. The most common form is the hypohidrotic X-linked form. The usual presentation is a male child with:

Multiple congenital absence of teeth.
Fine, sparse hair with shaft abnormalities.
Dry skin.
Frontal bossing.
Maxillary hypoplasia.
Thin lips showing little vermilion margin.
Heterozygous females can be identified dentally with microdontia and hypodontia.

Down syndrome affects 1 in 700 births and is commoner in offspring of older mothers, and is a result of chromosomal translocation. Apart from the morphological features characteristic of the syndrome, there is commonly delayed eruption and generalized microdontia as well as hypodontia of some teeth.

The diagnosis and management of all dental anomalies is extremely important and should not be delayed. Genetic consultation is often desirable to not only confirm the diagnosis but also to help parents understand the risk of future offspring and generations being affected. Conversely, geneticists may require help from paediatric dentists to clarify a diagnosis.

Key point

Hypodontia:

• Prevalence is 3.5–6.5% in permanent dentition (excepting third molar).
• May be associated with a number of syndromes.
• Requires multidisciplinary care.

image What factors would you consider important in the management of dental anomalies?

Reassurance of child and parent.
Elimination of pain.
Genetic counselling.
Restoration of aesthetics.
Provision of adequate function.
Maintenance of vertical dimension of occlusion.
Interdisciplinary formulation of definitive treatment plan.

Examination

Extraoral

Ellen had a normal facial appearance with a Class 1 skeletal pattern.

Intraoral

The following teeth were present:

image image were mobile. The other primary teeth were firm. The primary molars were infraoccluded.

image What special investigations are required?

A dental panoramic tomogram (Fig. 36.1) to check on the present/absence of other permanent teeth.

image

Fig. 36.1 Severe hypodontia

(from Millett and Welbury (2000) with permission).

image What is visible from the radiograph?

Absence of: image

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Related posts:

  1. 27: Facial swelling and dental abscess
  2. 39: Gingival bleeding and enlargement
  3. 3: Absent upper lateral incisors
  4. 6: Palatal canines
  5. 30: The fractured permanent incisor root
  6. 38: Dentinogenesis imperfecta

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Jan 2, 2015 | Posted by in Orthodontics | Comments Off on 36: Multiple missing and abnormally shaped teeth

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