ANOMALI UKURAN GIGI

1. Makrodontia
14.4.1 Crownsize
There is a degree of subjectivity regarding what constitutes normal tooth size and
shape. Teeth which are obviously larger than normal are referred to as megadont or
macrodont, whereas teeth which are smaller than normal are termed microdont.
Crown size is often related to root size, so teeth with large crowns often have large
(broad) roots, while teeth with small crowns tend to have small (slender) roots. Mi-
crodontia can be associated with hypodontia, as in the example of X-linked
hypohidrotic ectodermal dysplasia where a heterozygous fe- male has missing 31 and
41 and microdont lower permanent lateral incisors (Fig. 14.7).

Megadont/macrodont teeth

Megadont/macrodont teeth can occur as a result of either fusion of ad- jacent tooth-
germs or an attempt at separation of a single tooth-germ to form two separate teeth—
gemination (see Section 14.5.1 for further explanation). The permanent maxillary
central incisors are most often affected (Fig. 14.8), followed by the mandibular
second premolars. Isolated megadontia in the permanent dentition has been estimated
to occur in approximately 1% of patients. The condition may be sym- metrical.
Generalized megadontia has been reported in association with pituitary gigantism,
unilateral facial hyperplasia, and hereditary gingival fibromatosis. The diagnosis of
KBG syndrome (ANKRD11) is based on the recognition of the following symptoms:
macrodont upper central incisors, intellectual deficiency, characteristic facial
appearance (brachy- cephaly, a triangular shaped face, hypertelorism, prominent nasal
bridge, long philtrum, and thin upper lip), short stature, and skeletal anomalies.

Microdontia

Microdont primary teeth are uncommon, with a reported prevalence of 0.2–0.5%. In

the permanent dentition the prevalence is approximately 2.5% for individual teeth,
with generalized microdontia occurring in ap- proximately 0.2% of individuals.
Females are more often affected than males, with the maxillary lateral incisor being
most commonly affected, having a peg-shaped or conical crown (Fig. 14.9). As noted
in Section 14.2, there is an association between microdontia and hypodontia. In
patients with a SMOC2 mutation, microdont primary teeth, oligodon- tia in the
permanent dentition, and very limited root formation are combined.

14.4.2 Rootsize
Root length appears to be subject to some ethnic variation, with shorter roots being
seen in people of Oriental background and larger roots in patients of African origin.
Large root size

Larger than normal roots are most typically seen affecting the perma- nent maxillary
central incisors, with a population prevalence in one Swedish study of 2.3%. Males
were four times more likely to be affected than females.

Small root size

Short-rooted teeth in the primary dentition may be associated with other dental
abnormalities. Short roots may also be seen in a number of conditions affecting the
dentine and/or pulp (see Section 14.6).

Short roots affecting the permanent maxillary central incisors are present in
approximately 2.5% of children, and some 15% of these may have shortened roots on
other teeth, most often premolars and/ or canines. The cause is often unknown,
although it can occur as a result of orthodontic treatment.

In regional odontodysplasia (see Section 14.7.1) there is typically abnormal root

formation as well as abnormalities of the crowns of the teeth.

Irradiation of the jaws or chemotherapy during the period of root for- mation may
lead to truncation of roots which were developing at the time of treatment (Fig.
14.10).

14.4.3 Treatment
As with hypodontia, the active cooperation of a paediatric dentist, an orthodontist, and
a restorative dentist should be encouraged to opti- mize treatment planning for young
people affected by these conditions from an early age.

A megadont maxillary central incisor can be unaesthetic and treat- ment decisions
may need to be considered soon after (or, in some cases, before) eruption of the tooth.
The options include acceptance, reshap- ing or extracting the tooth, with orthodontic
treatment if necessary, and subsequent restoration of the space with a bridge, denture,
or implant (Fig. 14.11).

A microdont tooth, particularly if this affects the maxillary lateral inci- sors, can be
modified by the addition of composite resin to the tooth to reproduce the typical
contours of the crown (Fig. 14.12). In adult life, porcelain veneers or adhesive
porcelain crowns may be considered. However, the continued use of composite has
the benefit of no/mini- mal tooth preparation and delays the start of the ‘restorative
cycle’ with its ultimate end of tooth loss.

There are considerable variations in the timing of eruption of the per- manent
dentition. There may be some ethnic variation, and eruption may also be influenced
by environmental factors such as nutrition and
Treatment

As with enamel defects, severe psychosocial problems may occur as a result of the
appearance of the teeth. Many of the arguments presented in the consideration of
enamel conditions also apply to dentine. In DI, management is focused on the
prevention of tooth wear, the mainte- nance of the vertical dimension, and
improvement of the appearance (Chapter 11). In rickets, the treatment should be
similarly directed. Ag- gressive dental support, including cuspal or full crown
coverage without tooth preparation, may be needed in order to prevent bacterial
access to these extended pulps, whereas cases presenting late may require acute
management of dental abscesses as a result of pulp death.

14.7.4 Cementum defects

The cementum can be affected in a number of genetic disorders. The consequences of
alterations in cementum can have profound effects on the fate of the dentition.

There are a number of rare but significant conditions associated with the early loss of
primary teeth. Any case of early or spontaneous loss of teeth is a cause for further
investigation. In one of these, hypophos- phatasia (both autosomal dominant and
autosomal recessive inherit- ance are known), there may be premature exfoliation of
the primary teeth or loss of the permanent teeth. The serum alkaline phosphatase level
is low and phosphoethanolamine is excreted in the urine. Histo- pathological
examination in hypophosphatasia will show aplasia or marked hypoplasia of the
cementum. There may also be abnormal dentine formation with a wide predentine
zone and the presence of interglobular dentine (similar to vitamin-D-resistant rickets).

Treatment

Local measures such as scrupulous oral hygiene may slow the loss of teeth in cases of
hypophosphatasia, but the prime focus of treatment may need to be the replacement of
teeth of the primary and permanent dentitions as they are lost.

illness. Eruption times of permanent teeth in females tend to be slightly ahead of the
corresponding eruption times in males; this difference be- comes more marked with
the later erupting teeth.

Prematureeruption
Some families report that early tooth eruption is a family feature. Chil- dren with high
birthweight have been reported to have earlier eruption of their primary teeth than
children with normal or low birthweight. Early eruption of the permanent dentition
may occur in children with precocious puberty and children with endocrine
abnormalities, particu- larly those of the growth or thyroid hormones.

Natal and neonatal teeth

Teeth present at birth are known as natal teeth and those that erupt within the first
month of life as neonatal teeth. Approximately one in 2000–3000 live births are
affected. The mandibular central incisor is the most common natal or neonatal tooth.
Occasionally maxillary (central) incisors or the first molars may appear as natal teeth.
The vast major- ity of cases represent premature eruption of a tooth of the normal se-
quence. It has been suggested that this condition is a result of an ectopic position of
the tooth-germ during fetal life.

Natal or neonatal teeth may also be seen in association with some syndromes
including pachyonychia congenita, Ellis–van Creveld syn- drome, and Hallermann–
Streiff syndrome.

Natal or neonatal teeth are often mobile because of limited root de- velopment and
may be a danger to the airway if they are inhaled. The crowns may be abnormal in
form and the enamel may be poorly formed or thinner than normal. The mobility of
the tooth frequently causes in- flammation of the surrounding gingivae. Trauma to the
ventral surface of the tongue may cause ulceration (Fig. 14.44), and there may be dif-
ficulty with breastfeeding.

Treatment

Local measures such as smoothing the sharp edges of the tooth with a rub- ber cone in
a dental handpiece may help resolve the ulceration. In a num- ber of cases, the tooth
should be extracted if it is markedly loose or causing problems with feeding, as it is
unlikely to form a useful part of the dentition

Prematureeruption
Some families report that early tooth eruption is a family feature. Chil- dren with high
birthweight have been reported to have earlier eruption of their primary teeth than
children with normal or low birthweight. Early eruption of the permanent dentition
may occur in children with precocious puberty and children with endocrine
abnormalities, particu- larly those of the growth or thyroid hormones.

Natal and neonatal teeth

Teeth present at birth are known as natal teeth and those that erupt within the first
month of life as neonatal teeth. Approximately one in 2000–3000 live births are
affected. The mandibular central incisor is the most common natal or neonatal tooth.
Occasionally maxillary (central) incisors or the first molars may appear as natal teeth.
The vast major- ity of cases represent premature eruption of a tooth of the normal se-
quence. It has been suggested that this condition is a result of an ectopic position of
the tooth-germ during fetal life.
Natal or neonatal teeth may also be seen in association with some syndromes
including pachyonychia congenita, Ellis–van Creveld syn- drome, and Hallermann–
Streiff syndrome.

Natal or neonatal teeth are often mobile because of limited root de- velopment and
may be a danger to the airway if they are inhaled. The crowns may be abnormal in
form and the enamel may be poorly formed or thinner than normal. The mobility of
the tooth frequently causes in- flammation of the surrounding gingivae. Trauma to the
ventral surface of the tongue may cause ulceration (Fig. 14.44), and there may be dif-
ficulty with breastfeeding.

Treatment

Local measures such as smoothing the sharp edges of the tooth with a rub- ber cone in
a dental handpiece may help resolve the ulceration. In a num- ber of cases, the tooth
should be extracted if it is markedly loose or causing problems with feeding, as it is
unlikely to form a useful part of the dentition