Cleft lip and palate syndrome 

Cleft lip and palate syndrome:

1. Introduction :

Clefts affect one in 750 births, this syndrome affects the fetus during the first trimester. Clefts are one of the most common congenital dysmorphoses causing more or less significant aesthetic and psychological damage justifying multidisciplinary care.

The baby is born with a crack like a fracture line in the upper lip (not always affected).

2. Definition :

These anomalies observable at birth correspond to a solution of continuity either at the level of the upper lip or at the level of the soft palate or at the level of the upper jaw or at the level of all these structures at the same time. In more serious cases, they can extend from the nostrils to the pharynx.

Embryological reminder: 

The cleft appears during the formation of the maxillary buds due to the persistence of the membrane which separates them.

3. The frequency of the slots:

It is very variable, 1 case in 650 to 1300 in Caucasians.

It is twice as high in the oriental race.

Depending on gender, it is twice as common in boys as in girls.

Isolated cleft palate (lip not affected) 32%

Isolated cleft lip 1%

Cleft lip and palate 40%

By region (2007)

Oran 1% then Mascara then Ghilizen then Saida 

4. The etiology of clefts:

 There is an interaction between the environmental factor and the genetic factor.

Genetic factors: certain genes are responsible for the appearance of clefts.

Environmental factors: 

Tobacco (active or passive smoker)

Alcohol

Epilepsy and antiepileptic drugs

5. Description of the slots:

• Cleft lip: it only affects the lip, it can be unilateral or bilateral, passing through both nostrils and causing the flattening of the lip tip.
• Labio-alveolar clefts  : affect the lip and the alveolus or the palate, it is a defect in the fusion of the internal nasal bud and the maxillary bud, it leads to the path of the filtral crest. The premaxilla and the postmaxilla are separated by a space, it stops at the level of the anterior palatine canal, it can also be bilateral.
• Cleft lip and palate  : this is a more severe case, it is a fusion defect between the maxillary bud and the internal nasal bud, it passes through the germ of the upper lateral incisor giving two segments, one which is large and which continues its growth normally because it is attached to the nasal function and the different muscles and the small fragment which remains atrophic
• The bilateral form: we have two fracture lines, the premaxilla and 2 lateral fragments, the central fragment will be projected forward because it will benefit from the nasal thrust.
• Isolated cleft palate ( only the soft palate is affected): it can be partial or total (affects the entire soft palate).

6. The consequences of cleft lip and palate:
7. The anatomical consequences:
8. Malformative hypoplasia:

At the level of the lip and nose: absence of the anatomy of the lip and the Cupid’s bow whether it is unilateral or bilateral. When it is bilateral the tip of the nose is flattened.

At the level of the palate: the fracture line passes through the entire palate, giving a large fragment and a small fragment.

At the palate level: hypoplasia is certain and very marked and is remarkable during growth.

Cleft lip and palate syndrome 

2. Muscle imbalance:

• The tongue: the tongue is placed at the level of the opening of the palate, causing the two parts to become vertical.
• Abnormal muscle insertion:
• At the lip level: when the cleft is unilateral, the nostril is pulled towards the side where the cleft line passes. When it is bilateral, the nostrils are each pulled towards its left or right side, causing the tip of the nose to flatten.
• At the level of the palate: in the normal case, the insertion of the muscles is almost horizontal, when there is a cleft in the soft palate the insertion of the muscles becomes vertical. 
• Abnormal contractions:

In the case of a unilateral cleft lip: there will be a stretching of the nostril, the large fragment is pushed outwards and benefits from the growth spurt while the small fragment remains atrophic and inside.

• In the case of a bilateral cleft: only the small fragment is pushed by growth, the other two remain atrophic and set back in relation to the central fragment.

2. Disruption of functions:

If the lagoon does not take its normal position and the palate is open the functions are disturbed.

1. Ventilation or breathing: the patient cannot inhale and exhale through the nose, so there will be a deformation of the nasal passages.

Pulmonary breathing disorders

Delayed skeletal and statural maturation and cardiovascular problems

Underdevelopment of the sinuses = atrophy of the maxillary arch and recession of the anterior nasal spine

Generally, cleft cases present a class III malocclusion (not systematically).

2. Feeding: it will be very difficult for the baby, breastfeeding will be more difficult. If the cleft palate, there will be a rejection of food through the nose and to create the negative pressure of the seal formed by the two lips this is no longer possible since the cleft touches the upper lip.

Various means of feeding: 

Orthostatic breastfeeding: the baby is held in a vertical position to avoid rejection and choking.

The special bottle

Spoon or nasogastric tube feeding

3. Swallowing:

The sealing of the joint achievable with the lips is absent.

4. The phonation: 

There is nasalization, pronunciation is altered by the leakage of air from the oral cavity to the nasal passages.

5. The hearing: 

If the veil is affected, the ear is not ventilated and then there will be hearing problems.

3. Aesthetic consequences:

If the cleft touches the lip, we have flattening of the tip of the nose.

4. Disturbance of teeth:

The line of the cleft passes through the germ of the lateral incisor, we will have a problem in the transverse direction, in the sagittal direction. Either we will have a class II or III depending on the projection of the central fragment.

Cleft lip and palate syndrome 

7. Treatment of cleft lip, maxillo-palate:
8. We have several protocols or therapeutic charters:
9. According to the attitude of Veau and Petit:

No preoperative equipment.

At 6 months, cheiloplasty.

At 18 months, palatal intervention.

As a result, we will not have fistulas but we will have endognathism, retromaxillary and phonetic delay.

2. Schweendiek method:

Preoperative device, as soon as the baby is born a plate is placed on it so that it can take its milk.

At 6 months, surgery on the lip and soft palate

The palate is permanently closed around 7-12 years old

3. Early method in 2 reversed times by Malek and Psaume:

Preoperative orthopedic device

Veil surgery at 3 months

At 6 months, cheiloplasty and at the level of the bony palate

If the cleft is bilateral at 6 months we close one side and at 8 months we close the other side.

4. The pattern to follow according to Thalmant: 

No early orthopedics.

6 months, the soft palate and lip are closed and a nasal conformator is worn for 4 months

18 months, closure of the bony palate in 2 layers

From 3 and a half years: anterior orthopedic expansion

4-5 years: gingivo-periosteoplastic.

5. Whatever it is, the pattern will be as follows:

The first 6 months: neonatal orthopedics, fitting of a plate to close the cleft so that the tongue does not lie at the level of this cleft and so that the baby can feed without rejection.

6 months: cheiloplasty

18 months: uranostaphyloplasty, i.e. palate and soft palate

After 2 years, orthodontic and speech therapy treatment

2. Immediate treatment:
3. Psychological help:

To de-dramatize

Talk to parents and show them photos of people who have been cured 

Connecting parents with other parents of children with clefts

2. Advice for parents:

Regular nipples with good holes

Breastfeeding in an upright position

Spoon feeding very early post-op to avoid trauma to the surgical site

Have the baby lie on the side corresponding to the large fragment

3. Preoperative orthopedics:

Orthosis: any orthopedic device intended to protect, immobilize or support the body or one of its parts to which it is directly attached.

• Obturator palatal plate (passive plate): as soon as the baby is born, an alginate impression is taken, and the model is poured and an orthosis is made that the baby wears gradually until surgery.

This orthosis will consolidate the two fragments, thus facilitating the surgical procedure. It also closes the slit and prevents the tongue from getting between the fragments, thus avoiding their verticalization.

• Active plates: some orthoses contain hooks, compensation loops, jacks, etc. depending on the case. 

Among these plates, we have the nasolabial orthopedic plate, which is both an orthosis and contains a resin part that is placed at the level of the nostril to prevent it from flattening while waiting for surgery.

We also have the Latham fixed appliance, which is an orthosis with metal chains that connect the two fragments to prevent them from moving apart and each time we activate these chains.

We also have the orthopedic traction device used on bilateral clefts with the same principle as the last device.

The orthosis is worn 24/7, it is removed every 3 days for cleaning and replaced every 3 months (depending on the case).

Cleft lip and palate syndrome 

3. Surgical interventions:

1. Primary cheiloplasty: it is called primary because there will be another one in adulthood.
2. Primary Rhinoplasty
3. Primary surgical treatment of cleft palate:

Partial or narrow forms: these are done in a single surgical procedure

Large shapes: are done in 2 stages

4. Veloplasty: closing the soft palate
5. Closure of the bony palate
6. Gingivoplasty
7. Supervision of the child up to 2 years:
8. The dental check-up: 

Adaptation of the obturator plate as the eruption progresses

2. Psychological support (rejection or overprotection)
3. ENT check-up: twice a year (nose, ears, phonation)
4. Support for temporary dentition slots:

1. Recording of expiratory airflow during breathing or phonation
2. Nasonication or oralization of phonemes
3. Evaluation and treatment of velopharyngeal disorders
4. From an ENT point of view (adenoids)
5. From a dental point of view: strict hygiene, prevention and screening of cavities
6. Orthodontic treatment; do not extract supernumerary teeth (bone support)
7. Orthopedic treatment: generally class III report with a problem in the transverse direction then we put a Quad’helix to widen the maxilla with elastics to correct the reverse bite. In the sagittal direction, we use the Delaire mask.

• Should orthodontic or speech therapy treatment be preferred? If there is no language delay, there is no contraindication to undertaking early orthodontic treatment.

In case of language delay: Quad’helix at a distance from the incisors to leave the retro-incisal support area free

Prioritize speech therapy over orthodontics. 

6. Mixed dentition slot support:

In the transverse direction, we will widen the upper jaw and use the Criss-Cross elastics

In case of a reverse articulation, we try to establish it to avoid the installation of a class III

In case of superior retroalveolism, the sagittal direction is corrected

In case of moderate discrepancy, the Delaire mask is used in combination with fixed orthodontic therapy .

In case of significant maxillary hypoplasia, we move on to the Delaire mask and finish with an ante-pubertal (secondary) gingivoperioplasty.

7. Management of adult tooth gaps:

We only have orthodontic treatment

The transverse direction is corrected by the Quad’hélix and the Criss-cross elastics

The sagittal direction is corrected by alveolar compensation (no correction of the base shift)

If the lateral incisor is present, it must be preserved as long as possible, otherwise the space must be closed or kept in order to opt for prosthetic treatment.

If there is a problem in the sagittal direction, we can resort to bone distraction of the palatine suture which will allow the advancement of the maxilla.

8. Treatment of cleft sequelae:

After the end of growth, we make corrections and aesthetic treatments such as secondary cheiloplasty, tertiary gingivoperioplasty, secondary rhinoplasty… 

For dento-skeletal sequelae, we resort to alignment (orthodontic treatment) then surgery

Sometimes after surgery of the palate there is a persistent breach or fistula, its closure is later.

Cleft lip and palate syndrome 

Dental crowns are used to restore the shape and function of a damaged tooth.
Bruxism, or teeth grinding, can cause premature wear and often requires wearing a retainer at night.
Dental abscesses are painful infections that require prompt treatment to avoid complications. Gum grafting is a surgical procedure that can treat gum recession. Dentists use composite materials for fillings because they match the natural color of the teeth.
A diet high in sugar increases the risk of developing tooth decay.
Pediatric dental care is essential to establish good hygiene habits from an early age.
 

Cleft lip and palate syndrome