Dr. Salman Rasheed. House Officer Orthodontics Department, SBDC&H.

Downs sydrome was first described by JOHN LANDON-DOWN who orignally called this condition "Mongolism. Approximetly 95% of down syndrome cases are due to (trisomy 21) it is the presence of 3 instead of normal 2 chromosomes.

Prevelence is 1 in 650-800 live births.

Down syndrome is the most common chromosomal abnormality.

Cleft lip and/or palate are reported in 3%-5% of Down syndrome patients [Gorlin et al. 2001]

Exact cause not known.

PROPOSED THEORIES:
1) Increased maternal age 2) Viral infections 3) X-rays 4) Immunologic problems 5) Genetic predisposition 6) Hormonal abnormalities

Trisomy 21: Account for 95% and increase with maternal age have an extra chromosome,making a total of 47 instead of 46 chromosomes. It is produced by MATERNAL MEIOTIC NONDISJUNCTION(failure of chromosome to separate during meiosis or mitosis).

DENTAL C/F:
1)Agenesis/Microdontia/Hypodontia. 2)Conical teeth. 3)Delayed eruption. 4)Impacted teeth. 5)Supernumery teeth. 6)Taurodontism. 7)High vault palate. 8)AOB. 9)Ant + Post Crossbite. 10)Caries & Periodontal disease.

Epicanthal folds. Oblique eye fissures. Depressed nasal bridge. Small maxilla(A/p & Tran). Mandibular prognathism.

Downs syndrome child have a smaller then normal and abnormally shaped head. Flattening of back of the head. Upwards slanting eyes. Depressed nasal bridge. Slightly smaller eyes. Loose ligaments. Small hands and feets.

Macroglossia. Narrow oro-pharynx. Cracks and fissure in tongue. Angular chelitis.

CRANIAL FEATURES:
1)Brachycephaly 2)Diminished Ant.cranial base

Mental retardation. OSA. CVS,Genitourinary disorders. Respiration/Immune disorders. Speech and Mastication difficulties.

RADIOLOGICAL FEATURES:

Obtuse CBA(Platybsia). Cervical abnormality.

Orthodontic Mx of malocclusion in children with downs syndrome is considered problematic.This is due to the fact that compliance is perceived to be low (Beckman et al 2003)

Due to inability to understand the need of wear appliance and inability to adequatly maintain oral hygiene,this leads to high risk of Rx failure,CARIES and PERIODONTAL DISEASES.

Aims of pre-treatment visits.

To raise the pt level of confidence in dental enviroment.

To assess the pts & parent compliance in dental home care. To evaluate the expected degree of cooperation that will be finally be forthcoming.

Clearly,SEDATION & G.A cannot be performed for each visit for orthodontic appliance adjustment,,,,it is important to determine,if the pts can reach a level at which simple tasks may be performed with behaviour management technique only.

For those pts who have difficulties in communication & relatively inability to cooperate;we can offer 1)Conscious sedation. 2)I/V sedation. 3)Use of G.A.

. It is essential to establish that for most routine visits for appliance adustment;the use of BEHAVIOUR MANAGMENT techniques such as(Tell , show , do) behaviour modification, positive & negative reinforcement is adequate to achieve the goals of respective visits.

1)REALISTIC Rx GOALS:
Each child has his or her own acheivible optimum,which needs to be assessed by clinically,who must then apply Rx procedure appropriate for the child.

We must assume that an adequate clinical examination is possible by using behaviour modification tech alone,problems will often arise when attempting to take impression and radiographs. In pts with lack of cooperation,neuromuscular disorders or mental retardation;therefore multiple PERIAPICAL or LATERAL EXTRAORAL jaw view are recommended.

It is wise to establish reasonable goals on a modular basis,and to reassess them after each stage,being prepared to make the necessary changes if needed based on Rx experience with the previous stage for a particular disabled pts.

From orthodontic point of view,FIXED APPLIANCE are more difficult to place;especialy in these children because they require specific conditions,such as the need for pt to sit still for long periods of time to enable to precise positioning of brackets and with complete dryness of mouth. .Thus sedation or G.A is sometimes needed to facilitate their placement which is not in case Removable appliance adjustment of removable appliance is made extraorally and does not disturb the child. .In contrast,adustment of F.A can involve unpleasant sensations of pressure caused by introduction and maipulation of instrument within mouth.

5)Adapting Rx to sedation/G.a:

Aspiration is one of the most dangerous sequellae of any procedure that involve a partial or total loss of pts protective reflexes..due care and application of specific safety measures are essential to prevent debris,,water,,,saliva,,blood or orthodontic material entering the airway and producing larngospasm or possible infection of trachea or bronchi.
CHAUSHU & Colleagues have recommended the use of rubber dam as a useful aid and an effective safeguard in bracket bonding during General anesthesia. BECKER & Shapira find excellent acceptance and rapid results with full time wearing of removable appliance.

.Use of TIP EDGE APPLIANCE versus other types of STRAIGHT WIRE BRACKETS is advantageous because,it permits the insertion of heavier arch wire that are less likely to deform in early stages. .High quality and accurate bonding must be assured to avoid the need for subsequent rebonding without sedation..the most reliable and proven bonding materials should be employed. .Recently developed primers that enhance the strenght of bonding even in wet envirnment are particularly useful in pts with excessive salivation these together with the use of ANTI-SIALOGOGUE DRUGS

6)RELAPSE & RETENTION:

Children with skeletal discrepencies particularly the vertical discrepencies or with large tongue may never achieve stability.

.This should be predictable before treatment is undertaken and once Rx is completed,retention must be for an extended period of time. .Removable retainer will hold the alingment of teeth within the maxillary and mandibular arch,but cooperation must be assured. .Where this may be in doubt Bonded lingual splints are prefered,even though this may involve a further sedation session for its reliable placement.