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Click photo to enlarge Cleft
Lip What
is cleft lip and palate?
One in every 600 to 700 children is born with a cleft of the lip and/or palate. Extensive experience at correcting this deformity and its related dental and speech problems indicates that, with your help, your child can be expected to be no different from other children. The most important thing to remember is that it is what you do now and in the future for yourselves and your child that will determine whether your baby will develop into a normal, productive, happy individual.
Your attitude towards your child and towards each other should be discussed
openly. You are not responsible, and most importantly, your child is
not responsible for the cleft. Do not blame yourself and do not at any
time feel guilty for what you might have done or have neglected to do. The face of the infant begins to develop at approximately seven to nine weeks after conception. The lip develops, about the seventh week and the roof of the mouth, which is the hard and soft palate, at approximately nine weeks of life. The lip and palate develop independently; it is therefore possible to have either a cleft of the lip or a cleft of the palate separately or together. Lip: The degree of the cleft can vary from a slight notching of the red portion of the lip to a complete separation of the lip. The portion of the bone where the teeth grow through the gum may also be involved. The lip can be cleft to any degree on one or both sides. Palate: Clefts of the hard and soft palate may also vary in degree from a slight notching of the end of the soft palate to a complete separation through the roof of the mouth. Sub mucous Cleft : This is a cleft of the palate, which is not visible at birth. In the sub- mucous cleft the muscles of the soft palate are not joined together even though the lining of the roof of the mouth is intact. Though the palate may appear structurally normal on appearance there can be serious speech disturbances if not investigated and treated correctly.
Most investigators feel that there is sometimes a hereditary factor in the formation of cleft palate. Research has also demonstrated that the non-hereditary factors including nutritional deficiencies, infection and various drugs may also cause similar defects. Parents of the cleft lip and palate children often question whether they should have more children. If the mother also had a cleft lip with or without a cleft palate, she will have more of a chance of having another child with a cleft palate than if the father had this deformity. Racial and sexual differences affect such considerations. While one in 600 to 700 newborns in the Caucasian population may have cleft lips and palates, only one in 2,000 newborns in the Negro population are affected. Clefts of the lip with or without the cleft palate are more frequent in males, whereas clefts of the palate alone are more frequent in females. In general, clefts occur more often in males. If you are parents with no cleft history and give birth to one child with a cleft lip or palate, your chances would be about one in 25 of having another child with a cleft lip or palate. If you are a mother with a cleft lip and palate and give birth to a child with the same deformity, then your chances of having another child with a cleft lip and palate are approximately about one in ten. Your infant's face has the potential for growing and developing normally. The cleft is not due to something missing; rather it is due to something that has not joined together during early development. Although a wide cleft Have the lip and palate may be present at birth, the two separate portions of the palate can still grow and develop normally. After the cleft lip is repaired, the cleft in the palate will narrow. This may be evident soon after lip surgery while in other cases the narrowing occurs slowly: one, two or three years later. In a rare few the cleft palate will become wider. Growth increases the size of the palate and thus will aid in the closure of the cleft space. Facial growth changes in a child with a bilateral cleft lip and palate deserve special comment. The protruding middle section of the lip and bone (containing the front teeth) may appear to be forward of the remaining part of the face. However, as the face grows and develops, all the parts will begin to fit together more naturally. The protrusion of the profile of the child is lessened as the rest of the face grows. A plastic surgeon should be consulted to examine the newborn infant. Usually the cleft lip is repaired shortly after birth. Lip closure aids in feeding and improves facial appearance. The intact lip also helps mould the underlying bone. The physical condition of your child will determine the timing of the first operation. Your surgeon will review with you the nature of the deformity and His plans for correction. The number of operations necessary to accomplish a good final result depends on the type and degree of the cleft and associated problems. Surgery on the lip is usually performed under general anesthesia, but some surgeons prefer local anesthesia with sedation. After surgery, you will notice that sutures are in your child's lip. He may have an appliance taped to his cheeks to lessen the tension on the sutures. To keep the infant from rubbing the repaired lip with his hands, he may have his arms restrained. The baby will be offered water by mouth and then is usually back on formula feedings shortly after surgery. At the discretion of the surgeon, he may be fed with a nipple, a medicine dropper, a syringe, or whatever he has been accustomed to using. Whichever method is used, he should be held in a sitting position and fed slowly and carefully, allowing ample time for burping. After each feeding he should be given sufficient water to cleanse his mouth. To promote healing and prevent undue scarring, the suture line is cleansed and freed Of crusts which may form. This is usually done using sterile cotton applicator sticks or gauze squares saturated with hydrogen peroxide solution. This may be followed with a special ointment to keep the area lubricated. Care should be taken to see that the child does not rub his face on the pillow or the side of the crib. Insofar as possible, the baby should be kept from crying as this may cause undue pull on the suture line; therefore, sedatives may be used. Frequent holding, cuddling and feeding will usually keep crying at a minimum. Many infants are discharged on the second or third day after lip surgery. Within a week the sutures will be removed at the hospital or surgeon's office and the metal guard holding the lip, if used, will be changed. Some physicians will keep their patients in the hospital until the sutures are removed. You should take extra care during the first three weeks, continuing to clean the lip and restrain the child's hands. Be sure that your plastic surgeon clearly lists his desires for your baby with a schedule of events to expect. The scar from the repair of the cleft lip will remain red in children for up to a year's time. It is only after this period that one can tell what further surgery may be required. Very often what looks like a perfect result at the time of surgery will disclose need for some revision prior to his going to school. What seems to be an imperfect result at the time of surgery may, with growth, appear improved at a later age. Revision of the scar is often preferable before the child enters school, but that gives you a grace period of four to five years for this decision to be made. If there is a bilateral cleft of the lip, the plastic surgeon may repair the lip either in one or two stages. He may operate on both sides or he may do one side as in the unilateral cleft; then three to four weeks later, close the other side. Much depends on the nature of the clefts. Again, the same instructions apply in this situation as in the single cleft. You may be advised to use an external appliance (supplied by your surgeon or orthodontist), which will help mould the protruding central portion of the lip and bone before lip surgery is performed. The tip of the nose is usually pulled down following bilateral lip closure. This may require a later operation before school age. The initial results in the bilateral cleft lip are rarely as satisfactory as in the single cleft and later additional surgery is often the rule.
The
cleft of the hard or soft palate is usually not repaired until the child
is over one year of age. There are three aims of cleft palate surgery.
The most important is to help the patient speak well (this requires
a palate that is movable and long enough). The second aim is to help
re-establish palatal-Eustachian tube- muscle function related to normal
hearing. The third is to establish normal dental function and appearance.
Before closure of the cleft palate, the orthodontist may take dental
impressions. This will allow him to watch changes that come from growth
and surgery. X-ray films of the head (cephalometric studies) may be
taken for the same purpose. Usually, all of the palate is repaired at
one time although the surgeon may plan two operations in order to get
the most favorable closure and permit normal growth of the face and
palate. After
surgery your baby is placed on his side or abdomen. He may require gentle
sectioning to remove mucus from his nose or mouth. His arms are restrained
so that he does not place his fingers in his mouth. After surgery he
is usually given sips of water. You should remove the arm restraints one at a time at frequent intervals to exercise the arms and then place them on again. A convenient time to do this is during feeding. The child, after the palate surgery, should be kept from crying and receive a great deal of attention, affection and diversion. Children may have a fever after surgery, which may require antibiotics. The child may be placed in a plastic tent to increase the moisture of the air he breathes. His lips may be dried or cracked from having his mouth open during the operation. You may find a suture at the end of your child's tongue during the first day. This is only to be sure that the tongue can be pulled out of the mouth should he have trouble breathing. For the first few weeks after surgery, no foods harder than mashed potatoes should be given. Certainly, potato chips, hard cookies or candy should be kept out of reach for as long as the surgeon requests. Any of these could damage the newly repaired palate. Earaches may occur after cleft palate surgery from swelling of the throat. The surgeon or pediatrician should check these. If your child has a bilateral cleft lip and palate, there is a procedure to make the front gum firmer with a bone graft at the time the lip is closed, right after the lip is closed, or after further facial growth. Dental casts may be taken and special plastic appliances used to shape the palate and dental arches prior to and after such surgery. The bone graft is usually taken from a portion of one of the child's ribs. The results are too recent to be certain if this more extensive treatment is any better than other standard procedures. This is, however, considered an acceptable form of early treatment. Dental Care: Routine dental care is important in all children. The condition of the baby teeth or their absence can affect the success of the child's total cleft lip and palate treatment. A
full set of baby teeth is normally present by age three. You should
see a dentist by that time. When there is a cleft in the bone, which
supports the teeth, changes generally occur in the structures of the
teeth next to the cleft. In some instances, extra teeth are present.
Any of these teeth may be malformed or grow out- side of their normal
position. They may interfere with feeding to the extent that removal
may be required. It is usually important that all baby teeth be kept
until the growing second teeth normally replace them. Orthodontics: The orthodontist straightens the teeth and jaws. Most children with clefts of both the lip and the palate require two stages of orthodontic treatment.
The first stage of treatment may begin as early if age two. The roof
of the mouth on either side of the cleft may overlap after the lip is
repaired. This creates a "dental cross bite," a condition
where the upper teeth are inside of the lowers when the teeth are together.
The overlap can be corrected with a "spreading action" appliance,
which establishes a more normal relationship between the palate and
the upper and lower teeth. After the palate and teeth are in better
relationship to one another, they are held this way with a special orthodontic
appliance called a "retainer," until the permanent teeth erupt.
In some instances, surgery using bone grafts to maintain this correction
may be recommended. Cleft Palate Prostheses: Removable or permanent dental plates are necessary to replace missing teeth, to assist in chewing, improve the appearance of the mouth and aid in speaking. Special dental plates (obturators) are used to close spaces that may be present in the roof of the mouth. There are other forms of appliances, which assist in speech. All appliances should be checked periodically to be certain they fit properly and are still performing the task required of them. These appliances are made by a dental prosthodontist, on the recommendation of other members of the cleft palate team.
EAR DISEASE IN CHILDREN WITH CLEFT PALATE Cleft
lip children do not have ear problems any more than other children.
However, more children with cleft palates have a decrease in hearing
than do similar groups of children without cleft palate. Since it is
very difficult to see the ear drum in small infants, the physician and
family may not be aware of the presence of ear disease and changes may
occur which might have been prevented by early treatment. The part of
the ear that we are most concerned with in the cleft palate patient
is the middle ear. Since these changes occur early in life, your child may not show obvious symptoms but may learn to live with the disease. It is difficult but not impossible to evaluate hearing in infants. A specialist in the testing of hearing (audiologist) may work with the ear specialist (otolaryngologist or otologist) in evaluating your child's hearing as he grows. Moderate hearing loss may not be noted until the baby is older when it is obvious that he/she does not hear properly. Occasionally, the infection will be severe enough to cause a perforation of the eardrum with a discharging ear. Ear disease can exist with minimal symptoms in the infant. The ears should be examined regularly and the wax that interferes with visualization of the ear drum removed. Hearing tests are scheduled as early as possible. When the cleft palate child has a cold in the nose, throat or chest, the ears are examined to see if there is an associated ear infection. If this is present, it is usually treated with the appropriate antibiotic. When indicated, the eardrum may have to be "lanced" in order to allow fluid to escape from the middle ear. Opening the eardrum will not result in bad scars or permanent holes. It may sometimes be necessary to insert a plastic or metal tube through the eardrum to prevent it from healing too rapidly, to allow a longer time for drainage. If permanent changes in the ears can be prevented during infancy, the need for corrective surgery in later life may be avoided. If changes do occur, newer- techniques are available to allow the surgeon to try to reconstruct the middle ear in hopes of restoring proper function. As the child gets older, it is easier to examine him than to depend on his reporting symptoms. Periodic hearing tests are valuable throughout childhood to determine the function of the ear. SPEECH AND YOUR CLEFT PALATE CHILD There
is general agreement that the major potential handicap for a child with
cleft palate is a "speech defect." Defective speech was a
common accompaniment in cleft palate children in the past.
Inadequate Palatal Function: A definite relationship exists between
speech and the function of the soft palate. Muscles of the soft palate
and the throat must work together to close off the back opening to the
nose for your child to be able to make most speech sounds in the mouth. Infants normally learn speech in the first three years of life. Children without cleft palate or other physical problems sometimes learn to speak abnormally. Remember that poor learning or psychological problems can also cause defective speech in a young child. The chance of such problems occurring is greater for any child born with any physical handicap. Parental anxiety for an infant with a con- genital malformation may lead to overprotection. This can cause a lack of normal speech development. A speech pathologist may help your child attain normal speech early in life mainly by evaluating and understanding his special problems in learning speech. He can guide you in early training when it is needed. THREE DEVELOPMENTAL PERIODS TO CONSIDER In the preschool years there are three stages of development related to the time of surgical correction of the cleft palate during which parents can help. Before the Palate is closed: The first period is from birth to the time of surgical closure of the palate. If the cleft involves the lip alone, no special problem in speech should be anticipated. After the Palate is Closed: The second period is the time immediately after complete closure of the cleft palate to the time the doctors can tell if the palate muscles can function for speech. After the Palate Works for Speech to Eight Years of Age: The third period is from the time the palate performs properly for speech up to eight years of age at which time clear speech is usually well-learned by all children. This period will not be discussed further in this text. It is essential to understand the special learning problems at each of the stages. I) From Birth to Closure of the Palate: You will have four things to encourage
in your child's development during this period. These are:
Encourage Normal Speech Development: Your child's
wish to talk will be the same as any other child's. He will make sounds
(babble) in his early months of development. It is important that you
encourage him to make sounds. Do not discourage his early attempts at
sounds and speech. Some parents of cleft palate infants try to delay
their child's use of speech until after the operation on the palate
thinking he might form bad speech habits. However, since speech is the
natural form of language learning in the early years, holding back speech
can lead to increasing the speech handicap of your child. Simplified language at this stage does not mean "baby talk." Avoid this but attempt to shorten your speech by using only easy sentences. The non-cleft child from age one to three often leaves out final sounds of words. He will usually use easy sounds for harder sounds like W for R ("I wan to wide" for "I want to ride"). This is acceptable at this age and should not be criticized in any child. SOME FIRST WORDS WHICH CAN BE SPOKEN CLEARLY BEFORE PALATE SURGERY
All words in your child's early vocabulary should be encouraged and rewarded. These words have a phonetic structure which should enable specially clear production of first words as compared to other words. Attempt to understand all the words your child uses. Do not try to correct his speech at this stage. This becomes particularly important should the surgery be postponed. Encourage the child but do not frustrate yourself or the child. II After surgical closure of palate It is now necessary to determine if the repaired palate is sufficient for speech. The help of a trained speech pathologist would be most helpful at this juncture. There are certain things that parents can do to assist in developing palatal function. You can stimulate your child to use simple words that require coordinated use of the palate with other speech organs. SOME WORDS TO STIMULATE THE SOFT PALATE USE AFTER SURGERY
There are a number of simple games that parents can use after surgery to help their child use the muscular potential of the soft palate. As soon as the surgeon advises, foods, which require a good deal of chewing and frequent swallowing, should be given to exercise the muscles. The easiest way to start the closing off of the back of the palate and throat is to start with activities requiring blowing out of the mouth. Children can be encouraged to attempt to blow light objects such as feathers or ping pong balls as a game. When your child can speak words starting with P or B, the speech pathologist can better evaluate adequate function of the palate for speech. These words force a child to produce a puff of air to be produced. If air escapes through the nose on these words, the muscles of the soft palate did not close. It often takes many weeks of practice for the soft palate muscles to move in coordination with the lips on these words. A speech pathologist should evaluate your child's palate to see if it is adequate for speech as early as possible. He will discuss with you the specific questions, which arise regarding speech development. The need for formal speech therapy training before school can be determined. If circumstances do not allow for direct speech therapy in your community when this is recommended, you may help your child to produce words clearly through activities at home. Show and guide your child in naming pictures using words beginning with the Alphabet letters P, B, F and T at the start. Later, words with the consonant sounds D, V, K, 0, the, and she can be used. Words with S and eh or consonant blends are usually most difficult and should not be stressed early.
Give your child every opportunity to speak for himself in the family
and in social situations. Let him gain self-confidence and assurance
by answering questions addressed to him. Allow him every chance to recognize
himself as an important part of the family group. Expect him to speak
at the usual time and with the usual skills at each age level. SECONDARY SURGICAL PROCEDURES TO IMPROVE SPEECH Should speech be inadequate after the first operation because the palate cannot move properly, the surgeon will most likely wish to perform a second operation to improve the function of the soft palate. The operation may involve making the palate longer by a "lengthening procedure." A "pharyngeal flap" procedure is another operation used to improve the function of the palate for speech. In this operation some of the tissue at the back of the throat is attached to the palate to help close off the escape of air through the nose. Occasionally holes (fistulas) in the palate due to poor healing after the first operation require later closure to improve function for speech. Nasendoscopy and lateral video fluroscopy are two investigations that are done simultaneously to decide the requirement and the type of pharyngoplasty. Cleft palate patients having a second operation require the same consideration mentioned under the original palate surgery. After second operations, the help of a speech therapist to change poor speaking habits and to make the patient more efficient with his newly created structures is usually required. The later in life the second operation is performed, the more difficult the re-learning and, therefore, the harder the obtaining of normal speech. SECOND OPERATIONS IN CLEFT LIP PATIENTS As the child grows, it sometimes becomes apparent that his external appearance requires surgical revision. This may involve only a simple revision of a scar which is cosmetically unsatisfactory. It may also involve bone grafting the gap in the alveolus so that the permanent dentition can erupt normally. An orthodontist routinely observes these children in their post operative phase when the new teeth are erupting and usually advises Alveolar bone grafting at 9 to 11 years of age. Secondary surgery may be a correction of an asymmetry of one side of the nose. The tip of the patient's nose may be flatter and require nasal tip surgery. Some children, as they reach their teens, need an operation on the nose (rhinoplasty) to improve their appearance. There may be nasal obstruction because the nasal septum is deviated. This can also be corrected by surgery. Approximately two-thirds of the children with complete cleft lips and palate will have a deviated nasal septum. These are all things that must be evaluated in periodic examinations by your plastic surgeon. During teenage years, further surgery for cosmetic purposes may be indicated. This Improvement in appearance can be very meaningful and personally satisfying to the patient. EMOTIONAL ASPECTS OF THIS DEFORMITY It is important that you help your child to be socially mature and responsible in keeping with his age. If both parents accept the fact that the child has an obvious cleft deformity but that correction is possible, you are then best able to help him. Be encouraging but do not over-protect. Do not hesitate to consult with a psychologist or psychiatrist who might help prevent the development of negative or harmful attitudes in your family. Most importantly, be aware that a cleft lip or cleft palate does not limit a child's future capabilities. The preschooler may benefit from early association with other children such as in nursery schools and kindergartens. After school age, it is often better to schedule later surgery during the summer vacations so as not to miss school. Consult with his teacher when he starts school to be certain she understands his medical history and what treatment or special training is still needed. Most important for healthy emotional development is that your child, your relatives and your neighbours know you accept and love him. The
velo pharyngeal sphincter is situated between the oral and nasal
cavities and helps in separating the two cavities while we attempt
speech. A competent velo pharyngeal sphincter (VPS) is essential for
normal speech. The soft palate forms the anterior part of the sphincter
and the lateral and posterior pharyngeal walls form the rest of it.
Closure is achieved by tension in the velum and its elevation towards
the posterior pharyngeal wall. The lateral and posterior pharyngeal
walls move towards the rising velum, diminishing the lumen of the
pharynx and assisting in its eventual shutting. As one prepares to speak
the velum is partially raised and held in ready position. It only moves
to closed position once phonation starts. Vowels do not require a closure of the VPS and neither do the nasal sounds. Closure of the VPS allows compression of the air proximal to the point of constriction so that consonants, especially plosives and fricatives, can be produced with sufficient strength. For a voice that has quality, richness and carrying power, and for the consonants to be clear and precise, a good closure of the VPS is an essential prerequisite. If the air escapes into the nose through an open VPS the produced consonants lack precision and clarity, may get distorted, may be associated with pharyngeal or glottal sounds. Compensatory habits like excessive tension in the vocal tract, increased breath flow, articulatory substitutes further compromise the clarity and quality of speech. The speech distortions – misarticulations, hypernasality, hyponasality, and nasal escape interfere with the intelligibility of the individual words or sentences. Causes of V.P.I. Velo-pharyngeal
incompetence (VPI) can be caused by idiopathic insufficiency of
musculature surrounding the VPS, Congenital palatal insufficiency, Cleft
Palate, Sub-mucous clefts, following repair of Cleft Palate and after
Pharyngeal flap or Pharyngoplasty. It also presents after adenoidectomy,
after mid face advancements, because of enlarged tonsils, neurological
conditions like Myasthenia Gravis and Bulbar Polio mylitis and on rare
occasions it is Functional or Hysterical. The
reported incidence of speech disorders in Cleft Palate patients vary
widely, depending upon how closely the speech is scrutinized. Primary
effects include hypernasality, nasal turbulence and articulation
disorders like absence or distortion of plosives. Secondary effects
include abnormal articulations due to glottic stops, pharyngeal
fricatives and nasal grimace produced by attempts to interrupt or
constrict the flow of air in vocal tract. Additionally there may be
omission or substitution of phonemes. So adequacy of the VPS is the most
important determinant of articulation and listener understanding. Other
factors include dentition, the hypoplasia of palatal muscles, their
neurological input and the severity of hearing loss. The abnormal
compensatory patterns of speech production learned before palatoplasty
and velo-pharyngeal competence usually are hard to give up
post-operatively despite quality speech therapy. Speech
pathologists often find marked difference in articulation proficiency
between single responses and connected speech. In addition, observation
of patient’s articulation in conversational speech provides an
estimation of the speaker’s intelligibility level and degree of
communication handicap. Other features of VPI include facial grimace
associated with production of plosives, audible nasal emission of air,
and an improved articulation when the nostrils are manually occluded. Management of VPI This
involves
three steps
The direct visualization of the velo-pharyngeal sphincter with a nasendoscope together with synchronized lateral video fluoroscopy provide almost exact information of the sphincter deficiency as well as the correct choice of procedure to correct it. Nasendoscopy provides a clinical assessment of the depth of nasopharynx, length of palate, estimation of degree of palatal mobility during phonation, extent of palatal and pharyngeal wall motion and the exact extent and pattern of velo-pharyngeal gap during speech. Lateral video fluoroscopy is used to evaluate the degree of antero – posterior palatal movement and velo-pharyngeal closure during conversation and during production of a speech sound like a sustained s…, which essentially requires a total velo-pharyngeal closure. Thus these two procedures together provide information on the degree of palatal elevation, posterior wall movement, degree and pattern of sphincter closure obtained in antero-posterior and end on views during production of isolated words and during actual conversation. Surgery
for V.P.I. Surgery for VPI involves a combination of Palatal lengthening procedures, Pharyngeal flaps, Augmentation of posterior pharyngeal wall and reconstruction of velo-pharyngeal sphincter. Non-surgical management revolves around palatal prosthetic obturators and speech therapy. The demand on VPS varies from language to language, from place to place, and certainly across socio-economic groups. Acceptable speech standards also vary accordingly and the ultimate aim of all management protocols should be to achieve a speech for a patient that is easily understood by and that compares well with his peers. |
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