1. General Information
1.1. What is a cleft
1.2. Origin and frequency of the cleft
2. Care for a newborn or a child with a cleft
2.1. Prenatal ultrasound diagnosis
2.2. Breastfeeding, weaning and orthopedic plate
2.3. Orthopedic plates
2.4. Who does this
3. Treatment protocol
3.1. Surgical correction of the cleft at the Meyer Paediatric Hospital
3.2. Treatment of complete cleft lip and palate, mono-and bi-lateral
3.3. Treatment of cleft lip and cleft palate and isolated cleft
3.4. Ear problems
3.5. Development of language
3.6. Dental care and growth of the maxilla
Sugars and substances that cause dental caries
First dental visit prevention
Dental abnormalities in children with cleft
Dental malocclusion and altered growth of the maxilla
3.7. Psychological aspects
3.8. School placement
4. Bureaucratic – administrative procedures
4.1. How to make an appointment for a visit
4.2. Visit day
4.3. pre-admission Day hospital
4.4. Hospitalization for surgery
4.6. Health charges exemptions
4.7. How to contact us by phone
1.1. What is a cleft/strong>
It is probably the first time you hear about cleft lip, cleft palate, so some explanations may be helpful. In simple terms, cleft lip (cheiloschisis) and cleft palate (palatoschisis) are, respectively, failed welds of the lip or palate, which are normally merged during the first weeks of pregnancy. A cleft lip may vary from a slight incision on the vermilion (red portion of the lips) in a complete separation of the lip. The cleft lip can affect one or both sides of the upper lip, with varying degrees of severity. If it affects only one side, is called one-sided or unilateral cleft lip (Fig. 1), if affects both sides, is called bilateral cleft lip.
The cleft lip can often bind to the cleft palate, in its various portions: the front portion, bone, is defined hard palate, and the rear portion, free of bone, is defined soft palate and moves during phonation by specific muscles action.
A cleft palate is not a lack of the palate, although at times it may seem so, but a lack of fusion of the two component parts, failed when the baby was developing in the womb.
Because the palate is the roof of the mouth and the floor of the nasal cavity, a cleft palate creates a communication between mouth and nose. The cleft palate can vary greatly in extent, involving only the soft palate or comprising also the hard palate until the alveolar ridge that is located at the level of the gingiva, where teeth come out. The lip and palate develop separately, and it is possible that a child has only a cleft lip, a cleft palate only, or both simultaneously (labiopalatoschisis).
The cleft may involve one side (unilateral) or both (bilateral). When the cleft palate is associated with a very small jaw and breathing difficulties are present at birth, you have the Pierre Robin syndrome.
The clefts are the most common malformations that are present at birth. In Italy, approximately one in 700/800 infants born alive has a cleft lip and/or cleft palate. Although many possible causes have been analyzed, not yet one has been specifically identified. Even though 20% of the cases it is evident a hereditary transmission (presence of similar cases in the family), while, for the remaining 80% of cases, the cleft is due to the combination of not obvious hereditary factors that interact with environmental factors (smoking, alcohol , drugs, viral infections and non) that act negatively in the first two months of pregnancy, period where welding process takes place.
For cleft prevention therapy is suggested with folic acid, the gynecologist will decide whether to prescribe this. In any case, such treatment should be carried out within the second month of pregnancy because after this period, the therapy is not as effective, as the malformation has already occurred.
Most of the families who had a child with a cleft want to know how likely is it to give birth to another child with the same problem. This question can adequately be answered by a specialist in genetics.
The diagnosis of cleft involves an immediate awareness by the parents over this malformation. Parents will be able to deepen their knowledge by coming into contact with the medical team who then will take care of the child. Obviously, when indicated, psychological support will integrate this stage.
During ultrasound examinations in pregnancy, starting from 12 weeks prenatal diagnosis of cleft lip and palate may be made.
Newborns with isolated cleft lip do not have feeding problems and can be nursed like any other newborn.
Babies with a cleft involving the palate may have difficulty however in sucking the nipple due to the opening of the palate that will prevent the creation of vacuum necessary for effective sucking.
Breast-feeding infants with cleft requires considerable patience and a special technique. In the case of cleft lip it is almost always possible, while it is often difficult in cases of cleft lip and palate or cleft palate: for these cases, it is suggested the use of a breast pump and a bottle.
Breastfeeding a baby with the cleft may initially take a while but, afterwards, with practice, everything happens faster. If your child takes 45 minutes or more to feed, it may take too much effort and burn calories needed for its growth. In this case you should talk to your pediatrician. The use of plates (see below) can help children create a vacuum, compressing the nipple and facilitating suction (fig. 3).
A child with cleft does not risk choking on milk. The swallowing mechanism is perfectly regular even if the milk comes out the nose. The only drawback is flow of milk from the nasal cavity which are in direct communication with the oral cavity. Milk leaking from the nose does not determine any consequences.
In some cases of cleft palate since birth a plate is inserted, which facilitates feeding and helps control the development of the maxilla. In children with bilateral cleft lip a particular plate can help change the shape of the nose. A specialist in orthodontics generally places these devices together with a surgeon who will perform the surgery.
The plate will be monitored regularly until the first intervention. Since the plate is fixed with paste adhesives such as those used for dentures we recommend you bring a pack, on controlling visits occasions.
One of the most important decisions to be made about the care of your child, is the choice of the surgeon who will perform the initial surgery. In fact this is the first intervention that will depend largely on the treatments success, the artistic evolution of jaw development and phonation.
Each team is composed of groups of specialists in cleft and other craniofacial anomaly treatments. The team must include at least one normally maxillofacial surgeon, an orthodontist, a speech therapist and/or a speech pathologist and an otolaryngologist, as well as, of course, the pediatrician. As our department within a University Hospital for children, you can consult doctors of all disciplines who can directly solve all child problems or, if appropriate, to address in the most correct and efficient manner.
The advantage of a team is that the treatment and health of the child can be systematically and comprehensively planned. After visiting the child, the team members come together and establish a program for your specific treatment. All of these specialists, together with pediatrician, will follow the birth of the child until his adulthood for all your needs related to this therapeutic program.
The aim of surgery is to repair the lip and nose area of the cleft in the most aesthetic and functional way. The child will have a harmonious good look. The aim of surgery is to close the palate so that the area of the cleft palate can function normally during suction and language: all hindering in the least possible growth of the facial skeleton.
The repair is carried out in two successive times. Depending on the width of the cleft and are applied to two different procedures. In more extensive or bilateral forms, around the 6 months, the child who has reached a stable weight, also subject to screening for other possible health problems and proved to be not subject to any anesthesia risks, will undergo surgery for correction of the lip and nose and closing of the soft palate.
In cases in which the hard palate from this first intervention already can also be shut, this will be repaired together with the soft palate, the lip and the nose during the same surgical procedure. In these cases the alveolus, namely the area where the teeth will grow, will be repaired around 6-8 years with a bone graft.
For cases in which the hard palate has been left open, with a second intervention, around 18/36 months, the hard palate and the alveolus (gingival and alveolar surgery) will be repaired, in this case without the need of a subsequent bone graft intervention. It is important to remember that in terms of both the closure of the lip and palate exclusively and only the child’s tissue will be used: “what it takes to repair it’s all there.” Postoperative care will be explained by the surgeon and/or medical personnel. The scar on the lip will start to look clearer and more flexible after a few months, although always slightly visible.
Sometimes interventions can be carried out which differ slightly from those described in relation to the specific situation of each child. The surgeon, in science and faith, decides, sharing it with the parents, the protocol that guarantees the best results in each case. In a small percentage of children, around 4-5 years as indicated by the speech therapist, may need further surgery to improve phonation. Following corrections of lip, nose, nasal septum and a possible underdevelopment of the maxilla may be necessary or in preschool period (if severe) or even when the child has grown puberty.
In these cases it is usually only a single intervention. For the lip intervention happens around 6 months. Response times are estimates because they can be changed also in relation to the child.
ANY DELAYS ON INTERVENTION TIMES DO NOT AFFECT THE END RESULTS, NOT FROM THE FUNCTIONAL OR FROM THE AESTHETIC POINT OF VIEW!
Children with isolated cleft palate or cleft lip in combination with an increased risk of contracting otitis, in consequence of the inadequacy of some functional muscles of the palate that preside over the opening of the Eustachian tube, small ducts which connect the throat to the middle ear . In fact, when the Eustachian tube will not open in an appropriate way, the air can not penetrate inside the middle ear: the failure for ventilation may cause the formation and accumulation of secretion in the middle ear, a condition known as media diffusive otitis.
Secretion formed can then become infected, causing the baby a fever and a painful earaches (acute media otitis).
If otitis persists, with loss of hearing or if the child has recurrent infections that require the use of antibiotics, you can use a small surgical procedure (myringotomy), which is to create a small opening in the eardrum to drain the mucus, if possible, myringotomy is performed during the course of the lip or palate. After drainage, tiny tubes can be inserted into the slot created surgically, to allow air to enter the middle ear and prevent the formation of mucus.
After tube removal, these small fissures heal quickly and usually do not cause any permanent damage to the eardrum.
Another otolaryngological issue is of an overdevelopment of the adenoids, which can obstruct the Eustachian tube. Adenoids removed in toto, however, can have a negative impact on talk, so it is more appropriate to limit yourself to the removal of the blocking part (selective adenoidectomy).
Children with cleft palate have normal hearing at birth but, for the reasons set out above, it is important to regularly check your child’s hearing in the first years of life. It is appropriate that these checks are performed by an audiologist who uses audiometric equipment suited for examination of very young children.
Parents often wonder how it is possible to control the functionality of hearing in infants or very young children. Audiologists have many techniques to control hearing in such early stages, replaced by other tests for older children.
Children with cleft lip and palate, following the surgical treatment can recover fully their articulatory and phonatory skills developing a language totally intelligible. This is possible due to surgical reconstruction that allows a correct functionality of the organs responsible for phonation. In this process, the anatomical correction of the hard palate and the proper operation of the soft palate allow adequate velo-pharyngeal functionality, preventing air escape in the nasal cavity and thus avoiding the effects of nasalization during phonation. Generally, the emergence of such problems is part of a pattern that is characteristic for patients with cleft lip and no cause for concern. In other cases, such abnormalities are independent of the issues determined by the cleft, while, in a more limited number of cases, speech characterized by nasalization may be compromised. For these subjects speech therapy os indicated, re-education through specific exercises, helps the child to overcome the difficulties.
If the disorder has an anatomical-functional anomaly as primary cause, it can be necessary to add to the speech therapy further surgery to improve the soft palate functioning.
Within the multidisciplinary team that follows individuals with cleft lip and palate, the figure of speech therapist is required to assess, treat and monitor the production of speech of each child.
Through the analysis of intentional communication of young patients in the first months of life and subsequent observations of more specifically verbal language production, the speech therapist follows the parents in the path that will lead the child to communicate properly.
Particular attention is given through observation of the first sound productions of the child, to the mother and child communicative interchange, to the solicitation of some articulators schemes important to promote proper development of sounds that make up language. Children with cleft lip, following the intervention of reconstruction of the lip are able to develop language as all children. After you restore the correct anatomy, the child will produce sounds without any difficulty.
A few weeks after surgery, however, it will be useful to perform a massage over the upper lip to avoid a possible retraction of the scar and help maintain elasticity of the upper lip, and both to facilitate the implementation of the practices used in social life and the realization of lip movements that the child takes place in the production of different speech sounds. Parents are instructed to perform this massage.
In children with cleft lip and palate, language development may be accompanied by a slight delay in the developmental milestones that still does not give rise to any concern if properly monitored in the early years.
This delay is caused by several factors, the main one of which is represented by the particular anatomical structure that the child has at its disposal from birth that, while letting rich vowel and consonant play in the first months of life, it is not conducive to a rich babbling in the following months .
Up until the time of the second surgery, in the case of complete cleft lip and palate, sounds produced by the child may appear limited or made altered, as well as it may sound altered the tone of the voice.
This situation is considered typical in patients with cleft lip and so parents should not worry, anyway must be kept under observation by the clinician who will, from time to time, give information to the family about the appropriate stimulation to offer the young patient.
Immediately after surgery and then at 05-10-18 years of age formal assessments of language are carried out, with records of a sample of language and possibly nose-endoscopic and radiographic examinations (velography).
In some cases development time of language is observed, slightly slower as a result of difficulty of articulation or alterations in the quality of phonation, determined by velo-pharyngeal insufficiency. In these cases it is indicated to make use of a type of speech therapy.
Only in the rare cases where it is noticed a marked improvement in the quality of speech further surgery intervention can be carried out to correct the anatomical structure and put the subject in the best condition to produce correct speech sounds.
Children with cleft lip and palate do not require special dental treatment, but may be subject to more frequent malocclusion problems that involve the use of orthodontic appliances.
Parents should clean the first popped out tooth with a wet gauze after each meal and begin brushing teeth every day, even without toothpaste, after ingestion of solid foods, usually from weaning.
Generally, the pediatrician prescribes fluoride to be assumed in conventional doses. If you use a toothpaste, buy one for children that contains fluoride.
We suggest that you limit the administration of candy, gum and sweets. If you do, give the baby a bottle outside of meals, we remind you that plenty of fluids are highly cariogenic. We strongly advise against giving the bottle with any substance other than water when your child goes to bed. Milk is not cariogenic sugar. Most dangerous substances for the health of the teeth are fruit juices, sweetened teas, honey.
A preventative dental check should generally be around three years of age at the family dentist or at the Meyer dentist.
The cleft may be associated with dental problems: it can affect the alveolar ridge (upper gum containing the teeth) and alter the shape, position, and the enamel of the teeth, prevent development or on the contrary determine an oversupply. The most commonly affected tooth is the lateral incisor on the side of the cleft.
In order to follow teeth and jaw bones growth and define an orthodontic treatment plan, complete documentation should be carried out around 5 years that includes impressions of both dental arches, radiographs (panoramic X-ray of teeth and skull teleradiography) and photographs of the face, mouth and teeth.
Children may need sessions for prevention, care for any tooth decay, tooth extraction and for sealing the grooves of permanent teeth and, of course, orthodontic treatment and orthopedic appliances.
The specialist in orthodontics should follow children with cleft lip and palate during the growth in order to assess the need for alignment of the teeth, enlargement of the palate to create space for permanent teeth, monitor the growth of the maxilla and decide, together with the surgeon , additional surgical corrections of advancement of the maxilla that had a poor growth (Fig. 4).
Both parents and children may need a professional to take care of the mental well-being. Following the birth of a child with a cleft, parents may experience shock, anger, feeling a sense of guilt and anxiety, depressions. Stress may develop following the economic efforts and care related to the situation and at the time of the surgery practiced on the child. Brothers and sisters of a child with a cleft may have mixed feelings towards this new family member.
Parents may be called to face new problems regarding the management and education of children. A psychologist can help parents in dealing with this situation. Children with clefts may have difficulty adapting in a psychological manner which may occur around the age of two. They can be a result of the reactions of the people to the way they talk or the way they look. Psychologists can help children develop self-confidence and adequately address any ridicule.
From the age of three, children can learn to respond to simple questions about their appearance or the way they talk. Children aged two to four years may be afraid of hospital and surgery and cry or fuss when they go to the hospital. Sometimes children try to cope with these problems by hiding behind the shyness to prevent people acting so immature compared to their age or seeking refuge with the parents: they can help by requiring a play-therapy for the child or advice regarding the ‘ education for themselves.
In all these cases, the psychological response of young patients or their family members may become in itself a clinical problem.
It is good that children with cleft attend kindergarten because young children learn through play. They need to experience new, exciting, pleasant experiences with their peers, with materials and with adults.
The nursery gives children the opportunity to interact with others outside of their domestic space and become part of a group with a daily routine. The child can make the experience of being “special” outside the family.
The experience of kindergarten provides benefits to parents, who can observe the child in a group of other children and observe his behavior and that of other children of the same age.
They can also see how other adults behave with their child and get advice from experienced professionals to understand children. In addition, the nursery provides parents time to work or spend time with other children who may feel neglected by the attention given to the brother.
The first visit to our center can usually be fixed by booking at 055/5662900 (CUP, One Centre Bookings) on advice of the family doctor or the Department of Obstetrics and Gynecology, where the young patient was born, doctors will fix another appointment.
In case you are unable to keep your appointment and want to move or the appointment was after a long time and doctors could not program it, call CUP already carring a referral from a pediatrician.
To see your child you must be in posses the required documentation of the child’s pediatrician, with indication of maxillofacial surgical visit.
You will be made an appointment at a time that may vary depending on the workload of the day and emergency management. We ask courtesy to have some patience. When you arrive you will have to carry out the bureaucratic process of acceptance at the counter. Even having an exemption, this procedure is essential.
Bring with you all that can be of assistance during your stay in the center. For a reminder, we list a few things you use most frequently: the need to feed and change the baby, adhesive paste and / or patches if your child uses the plate.
Approaching the date of the operation, you will be contacted by telephone to perform pre-operative exams. On this occasion, the child will be subject to removal, the appropriate examinations and necessary specialist visits for intervention.
At the date of admission you must present at the time you were communicated by the administrative staff. Remember to bring all medical records in your possession. A parent can sleep next to the baby during hospitalization and will always be next to the child. During the recovery period, which is usually of 3/5 days, in the department you will find the basic needs.
If your child has special needs, such as taking special medication, report to the medical department, in any case bring an extra, but you probably do not need it. Do not forget your favorite games for your child and what else can be used for its serenity during the period of hospitalization.
At the end of hospitalisation information about the process after surgery and the date of the next visit will be provided. You will also receive a letter of resignation, on which are briefly described all the information about the hospitalization and surgery. Do not forget to bring your letter to the appointments.
Children with cleft or facial deformities are entitled to exemption from charges as a result of a medical certificate.
5 – Staff
6 – Glossario