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Genetic Factors One in every 700 births results in a cleft lip and/or palate. Cleft defects develop very early in pregnancy. They result from an abnormality in the genes or chromosomes. The causes of clefts could be genetic, environmental or nutritionally derived In most children born with cleft lip and/or palate, this is the only congenital abnormality. However, approximately 15% percent of all children with a cleft have multiple congenital abnormalities. 300 craniofacial (head and face) syndromes involve cleft lip and/or palate as part of the syndrome. Most of these syndromes have been identified and named. Proper and complete assessment of the child with a cleft is necessary to determine the presence of other abnormalities, to establish realistic expectations for treatment and to plan the best course of treatment. Top
ANATOMY OF NORMAL FACENormal Anatomy of lip and palate The ultimate goal in treating cleft patients is to perform reconstructive surgery that creates a normal appearance and restores function. The following will describe the normal anatomy of the lip, nose and palate. The Upper lip Vermilion: The lower margin of the upper lip is called the vermilion and is characterized by its rosy color. The line or ridge between the skin of the upper lip and the vermilion is called the vermilion border. The Upper lip is composed of orbicularis oris muscle covered by skin on the outside and mucous membrane on the inside. Many other muscles are attached to the orbicularis oris muscle, and together they work to create the movements and power required for speaking, eating and forming facial expression. The orbicularis oris muscle constitutes the major bulk of the upper and lower lips.
The Nose
The Palate. The hard palate is a composite of several bony structures. The arch of the hard palate is called the palatal arch. The hard palate constitutes the anterior portion of the entire palate and lies directly behind a horseshoe-shaped bony arch which supports the teeth. This arch is called the alveolus. The teeth protrude from a ridge known as the alveolar ridge. The hard palate is immobile. The flesh that covers the hard palate is called mucoperiosteum. It is used in cleft palate repair to close the hard palate defect. The soft palate lies behind the hard palate. It ends with a little flap that hangs down from the soft palate called the uvula. The soft palate is composed of several muscles and fibrous tissue (all of which is attached to the posterior edge of the bony palate). The soft palate is mobile and plays a decisive role in speech production. This primary function of the soft palate is dependent on the levator (veli palatine) muscle. Reconstruction of this muscle is an important part of cleft palate surgery. Behind the palate lies the pharynx or throat. The pharynx begins behind the mouth is called the oropharynx. The soft palate performs many functions. One of its roles is to close off the back of the nose during swallowing. This keeps food and fluids from being forced through the nose when a person eats and drinks. The soft palate also plays a major role in speech. Since it acts like a veil over the pharynx, it is sometimes called the velopharynx. When the soft palate does not close completely or properly while making certain sound, the patient is said to have velopharyngeal insufficiency or velopharyngeal incompetence. This functional impairment in cleft patients is marked with a nasal quality of speech called hypernasality. Top The cleft is a splitting of tissue that occurs in the upper lip, alveolus and/or palate. The degree to which these structures are deficient, malformed or displaced depends on the form and severity of the cleft. The nose, which is not spilt, is severely affected by the split of anatomic structures at its base. If the split is one side of the lip it is called a unilateral cleft lip and if the split is on both sides it is known as bilateral cleft lip. The cleft lip may be associated with cleft of alveolus and palate. The cleft could also be isolated to the palate only. This is known as isolated cleft palate. Unilateral Cleft Lip and/or alveolus and/or palate The unilateral cleft lip defect can be complete or incomplete. It can be associated with cleft of alveolus and/or cleft of palate. The unilateral cleft lip defect is formed by the improper fusion of the orbicularis oris muscle and its accessory muscle on one side. In a complete cleft the whole orbiclaris oris muscle does not fuse but in an incomplete cleft part of the orbicularis oris develops normally.
Bilateral Cleft Lip and/or alveolus and/or palate
Cleft Palate Cleft palate may be present without clefting of the lip and alveolus. It may involve only the soft palate, the soft and part of the hard palate, or the entire palate, soft and hard. The clefts, especially complete clefts of the palate, may vary in width. The cleft palate is divided into the hard palate and the soft palate as mentioned earlier. Cleft of the hard palate involves the bony shelf of the palate that does not fuse leaving a gap down the center of the palate. The cleft of the soft palate involves non-fusion of the soft palatal muscles, which are then abnormally attached.
While parents often request that the cleft be repaired soon after birth and before the child goes home, surgery on the newborn is difficult because it is dangerous and usually results in a less-than-optimal repair. Therefore, most cleft lip and palate treatment centers prefer to wait to perform the initial lip repair until the child is approximately four months old. There are several reasons for this delay. First, when the child weighs between six and eight kilograms and is healthy and vigorous, he or she is better able to tolerate the anesthesia and surgery. Second the waiting period allows ample time to thoroughly diagnose the extent and characteristics of the problem and any additional or associated problems that might be present, as well as to adequately plan an immediate and long-term course of treatment. Third, the child at four months of age is significantly larger than the newborn which facilitates the technical requirements of the surgery and results in a much better aesthetic and functional outcome. During the period before surgery, parents are generally concerned with the problems of feeding and the issue of bonding. A number of feeding approaches are available to the parents of children with clefts. Some trial and error may be necessary in finding the method that works best for you. Nearly all babies experience some weight fluctuation after birth. Your pediatrician can help you determine the significance of the weight fluctuation and the need to change or supplement your child’s diet. A rule of thumb that may be helpful is that a child should take about 90 to 120 ml of milk or formula in about thirty minutes of feeding. It is myth that all babies with a cleft are unable to breast feed. While some babies may not have the ability to suckle sufficiently to breast feed, others do. If you have made a commitment to breast feed your child, the presence of the child’s cleft should not deter you from trying. Remember that many mothers with normal children have difficulty learning to breast feed. Some of the problems all mothers must solve in learning to breast feed are how to hold the baby, how to place the breast nipple in the baby’s mouth, waiting for the breasts to become engorged with milk, knowing how much milk the child has taken etc. For the mother of a child with a cleft, it is tempting to attribute early problems to the cleft when they actually may be problems all mothers face during the learning process. When feeding your baby, cradle him or her in your arms in a semi-sitting position with the infant’s body upright and tilted slightly backward. This position helps the child swallow and also prevents the flow of formula or milk into the nose. The child with a cleft may swallow more air when feeding than a child without a cleft. Therefore, he or she should be burped often. Some parents worry that their infant will choke during feeding. Choking is usually caused by milk striking the back of the throat and is often the result of feeding too rapidly. Be sure the nipple opening is just large enough to allow the milk to flow freely with very little pressure against it. If milk pours out when the bottle is inverted, the opening is probably too large. Weight Gain In general, your child should double his or her birth weight during the first two and a half to three months. The growth and development of your child will be monitored carefully by your pediatrician. If the baby does not gain weight as rapidly as desired, nutritional supplementation or other measures be necessary. Whenever possible, many centers prefer to delay the initial surgical treatment of the cleft until the child weight at least six kilograms. A weight of eight kilograms is desirable before treating a child with bilateral cleft. This weight is usually achieved between three to six months of age. In general, it is desirable to perform the initial surgery before the age of six months at approximately four months of age. Other considerations in assessing your child’s readiness for surgery are his or her general health and a hemoglobin count of greater than ten. Hemoglobin is the component of red blood cells that carries oxygen to body tissue. Top PLEASE VIEW PREOPERATIVE AND POSTOPERATIVE PICTURES OF PATIENTS IN THE SURGICAL SPECTRUM PAGE OF THIS WEBSITE. (Link this line to surgical spectrum) Top Surgery is performed in the hospital under general anesthesia. The child is admitted to the hospital one day before surgery. The surgical procedure for closure of a unilateral cleft lip and nose deformity generally requires between one to two hours. The hospital stay is usually 48 hours post-operatively. After the surgery is completed, the child is taken to the recovery room for careful monitoring. When your child has safely recovered from anesthesia and is waking up, you will be able to be with him or her before returning to the child’s hospital room. During the rest of your child’s stay, you will learn how to care for the operative wound. It is also important to keep your child from rolling over on his or her side or stomach since pressure against the surgical area may cause injury to the freshly sutured lip. Pressure can even break down the sutures. Sutures in the lip are removed 10 days after surgery. Specific instructions regarding this procedure will be given to you at the appropriate time. It will take two and a half to three weeks for the operative wound to gain sufficient strength to resist damage. The Unilateral Cleft lip Unilateral cleft lip is a cleft involving only one side of the lip. It may be only a notching of the lip, or the cleft may extend into the nose or though part or all of the palate. Surgical repair of the cleft is done at age 4 months and weight 6 kilograms Classification of Unilateral Clefts Incomplete Unilateral Cleft Lip: Cleft lip without cleft alveolus
Type I Submucous cleft Type II Involving vermillion but not extending beyond white roll
Type III Extending beyond white rol l Type IV Extending beyond whiteroll Complete Unilateral Cleft Lip: Cleft lip with cleft alveolus
Type I a without Simonart’s band Type I b with Simonart’s band
Type II a without complete obliteration Type II b with complete obliteration of the of the alar curve on cleft side alar curve on cleft side
Surgical Repair of the Unilateral Cleft Lip A number of techniques are available in the repair of the unilateral cleft lip. The method used in our center is the Afroze technique which uses “C” flap on the non-cleft side and the Pfiefer wave on the cleft side. This provides adequate rotation and lengthening of the tissue to get a good lip form. The objective of surgery is to reconstruct the functioning aspects of the lip so it will look as normal as possible. The cupid’s bow on the cleft side is recontoured and matched with non-cleft side to simulate the philtral colum. Lengthening of the lip balances out the cleft and non-cleft sides. Repairing the Unilateral Cleft Nose. The Primary cleft nasal deformity can be markedly improved by reconstructing the nose at the same time as the primary lip repair. This is the single most important operation on the nose, although there will probable be secondary surgery at a later age. Here the septum of the nose is carefully dissected and repositioned and the nasal cartilages are repositioned with the nasalis muscle (this is the muscle that gives the shape to the under side of the nose).
Afroze Incision Minimal muscle dissection ensuring dissection of transverse nasalis muscle
Wide sub-periosteal dissection from the Minimal muscle dissection is done on the
SEPTUM IS KEY Periosteoplasty is done to exert more medial
Ala of nose stabilized symmetrically to Muscle approximation and closure is done
The Bilateral Cleft Lip Bilateral cleft lip involves clefting on both the right and left sides of the lip. Fifteen percent of all children with a cleft lip deformity have a bilateral cleft. In the bilateral cleft, a portion of tissue projects from the bottom of the nose. This central portion of the lip (prolabium) is attached to a segment of bony gum (premaxilla). This tissue is used to surgically reconstruct the entire central portion of the lip. Classification of Bilateral Clefts Incomplete Bilateral Cleft Lip: Cleft lip without cleft alveolus
Type I a Symmetrical cleft involving Type I b Symmetrical cleft involving
Type II a Asymmetrical cleft involving Type II b Asymmetrical cleft involving Complete Bilateral Cleft Lip: Cleft lip with cleft alveolus
Type I a Complete cleft on both sides Type I b Complete cleft on one side and incomplete cleft on the other
Surgical Repair of the Bilateral Cleft Lip Surgical repair of the bilateral cleft lip is performed in one stage. The surgical procedures for repairing bilateral cleft lip are carefully planned for each case. As with the unilateral cleft, the incisions are made so that the sides of the muscle are freed form their abnormal attachments to the sides of the cleft. This allows the surgeon to swing the muscle into its normal position for reconstruction, lengthening of the lip and providing for normal lip movement. The incisions are closed so simulate the philtral columns with reconstruction of the philtral dimple and the Cupid’s bow. Repairing the Bilateral Cleft Nose Unlike the surgery in a unilateral cleft nose, the bilateral cleft nose is usually obscured by the projection of the premaxilla and prolabium. The nose though is repaired like tat of the unilateral lip with the repositiong of the nasal septum and nasalis muscle. The shape of the nose starts to show once the lip muscles act on the premaxilla to rein it into the palatal and alveolar arch form. The bilateral cleft nose will require revision surgery once the child grows up.
Afroze Incision of transverse nasalis muscle
Nasal sill is closed bilaterally Ala of the nose is stabilized syymetrically.
Vestibule formed with tissue from Muscle approximation and closure is done The Cleft Palate Clefts of the palate can occur alone, as an isolated deformity, or in combination with a unilateral or bilateral cleft of the lip. In twenty-five to thirty percent of all individuals with a cleft deformity, the cleft palate is the only cleft problem. Clefts of the palate may vary considerably. They appear as a single narrow cleft, as a wide cleft which is complete or incomplete, or as a bilateral complete or incomplete cleft in association with a complete or incomplete cleft lip. Treatment of the child with cleft palate is complex for several reasons. First, the tissue and bony structures of the hard and soft palates are contained within the alveolar arch which means that the only tissue available for closure of the palatal defect must come from the sides of the defect. Second, development of the teeth and alignment of the jaws can be effected. Third, both the hard and soft palates play a vital role in speech development. Fourth, persons with cleft palate often have associated problems with the middle ear. Classification of Cleft Palate
Type I a Type I b Type II a Type II b
Type III a Type III b Type IV a Type IV b Surgical Repair of the Cleft Palate. To offer the best chance for normal speech development, we perform the complete repair of the palate in one procedure at the age of twelve months. With this approach, most patients have normal development of the velopharynx which is essential for normal speech. The Some patients may require future speech therapy and/or and additional operation called pharyngeal flap or pharyngoplasty. The need for additional procedures cannot be determined until the child reaches the age of four to five years and can be adequately assessed for speech development. The surgeical procedure requires about one and a half hours. The main muscle in the soft palate is called the levator muscle. Normally it forms the soft palate and connects to either side of the throat to help form the velopharynx. It is abnormally attached to the sides of the posterior edge of the hard palate and to the sides to the cleft itself. The next step in the procedure is to carefully isolate the muscle from their abnormal attachments and to reconstruct the normal muscle sling and configuration in the back of the soft palate The third and final step of this operation is the complete closure of both nasal and oral layers to create a well-functioning soft palate and to completely close the defect within the hard palate. This type of closure helps to prevent the occurrence of fistulas which are small holes between the oral and nasal cavity within the area of the reconstructed palate. Fistulas result in the leakage of air and fluid into the nasal cavity. The presence of palatal fistulas requires surgical closure since they not only cause leakage, but also hamper normal speech.
Medial Incisions: uvula to anterior. Nasal layer carefully preserved and detached
Detached muscle sutured to the Adaption sutures placed to hold down hard Postoperative care after cleft palate surgery Several months are required for complete healing of the reconstructed palate. By four weeks, however, the repair is strong enough to resist damage caused by the baby’s fingers or by eating. Feeding your child after surgery requires special care. It is imperative to keep your child from sucking during the initial four weeks after surgery. Fluids must be carefully dropped in the mouth from a cup or syringe for the first four weeks after surgery. The child may be offered any fluid that flows form a cup, including pureed food. If the mixture is too thick, it may be diluted with milk. Follow each feeding with water to cleanse the palatal area. The child will remain in the hospital 24 – 48 hours after surgery. Since the sutures used in palate repair are dissolvable, there is no need to remove them. However, it is important that the child be seen in the hospital for a follow-up visit approximately four weeks after surgery. aa Top It is common for children with cleft palate to have associated problems with the middle ear. Most of these problems are caused by a malfunctioning of the Eustachian tubes which are small passages that lead from the middle ear to the throat. Their function is to allow pressure to equalize on the inside of the eardrum or tympanic membrane. They also serve to drain any fluid that may accumulate in the middle ear. Yawning allows air to pass through the Eustachian tubes to relieve pressure on the eardrum. When the Eustachian tubes are not functioning properly, fluid can build up in the middle ear, causing discomfort and temporary or even permanent hearing loss. Treatment of Eustachian tube problems is by surgical placement of “ear tubes” called grommet tubes. These small plastic tubes are placed in the ear drum to allow normal ventilation and to drain fluid from the middle ear. With surgical treatment and continued regular assessment and care hearing loss can be prevented . Top Special Problems with Teeth and Jaws Because cleft palate affects the form and growth of the upper jaw, the teeth become a special consideration. Development and alignment of the teeth and their relationship to the jaw needs to be addressed during infancy and throughout the child’s growing years. Age Five to Eight Years. As the child grows, the bony arch that supports the teeth, the alveolus, may become too narrow or incorrectly shaped. This condition is called collapse of the alveolar arch and requires orthodontic treatment. A prosthesis called an expansion device is inserted in the mouth which gradually expands the arch. Once the arch is expanded, its shape is maintained as the child grows and develops either with continued use of the expansion device or with a different type of prosthesis which is temporarily inserted into the mouth. As the permanent cuspid (canine or eye) teeth develop and begin the process of eruption, usullay between seven to nine years of age, it is necessary to correct the bony defect in the alveolar ridge. The surgical procedure used is a bone graft. It involves filling the space between the edges of the alveolus with a small amount of bone taken surgically from the hip. To prepare the area for bone grafting, the orthodontist applies orthodontic bands and wires(braces) to the teeth; he or she will work with your child until the teeth are properly positioned. Ages Eight to Twelve Years. Ages eight to twelve are called the period of mixed dentition because children lose their primary (baby) teeth, and their secondary (permanent) is used to move the teeth into a more normal position. This enables the orthodontist to create a normal arch form and a good positioning of the upper and lower teeth. If there is underdevelopment of the upper jaw, overdevelopment of the lower jaw or a combination of two, further recommendations for treatment will be made. In about ten percent of all cases, surgical treatment may be necessary to correct skeletal deformities of the upper and/or lower jaws. Treatment is delayed until orthodontic alignment of the teeth is completed, and this surgery is performed no earlier than fourteen to sixteen years of age. Upper jaw surgery involves incisions in the soft tissues inside the mouth which cover maxilla, allowing the surgeon to advance or move it forward. The new position of the bone is then secured with miniplate fixation. While this type of surgery is not frequently required, it can enhance the function and appearance of the teeth and jaws while speech and nasal breathing. Surgery on the lower jaw may be necessary to correct the skeletal deformity. It is performed when the jaw appears to be overgrown and must be set back to balance both the upper and lower lower jaws. This setback procedure is called a mandibular osteotomy. Final Orthodontic Treatment Final orthodontic treatment focuses on the proper positioning of the teeth in the upper and lower jaws. Absent teeth are corrected with a bridge, a removable partial denture or the newer technique of dental implants. Depending on the particular cleft deformity and your child’s unique problems, any one, or a combination of these techniques, may be recommended. Top Many factors influence the proper development of speech. Defects in the palate can certainly impair this process. Thus, an important aspect in cleft palate treatment is thorough speech assessment, evaluation and speech therapy, if needed. As discussed earlier, an important consideration when surgically closing the palatal defect is the effect it will have on normal speech development. Assessment of speech performed by a speech pathologist, who specializes in cleft and craniofacial patients. Part of the evaluation involves obtaining a speech sample. This simply means that the child is asked to say various sounds, words and phrases that represent the spectrum of normal speech. The child must be old enough to cooperate in the assessment. Usually, this age is four to five, although occasionally it is possible to assess children as young as three years old. Early evaluation is important. If there are problems regarding the child’s speech, therapy may be prescribed by the speech pathologist. Parents play a very important role in working with the child to encourage the development of normal speech. The speech pathologist will provide guidance for you throughout this process. If any abnormality in speech is suspected, certain diagnostic studies may be necessary. Like nasopharyngoscopy. Naospharyngoscopy is a technique used by the physician to visualize or look at the back of the throat and the velopharynx. It can be performed in children as young as four to five years of age and is done while the child is awake and talking. Nasopharyngoscopy involves placing a small tube through the nose and into the throat. The physician and / or speech pathologist can look through this tube to analyze the movements of the soft palate and throat as the child talks. Recordings of these movements are made on a videotape synchronized with the speech sounds. Top Many adults cleft deformities have not had the benefit of being treated with the modern techniques described in this booklet. To help these individuals, may surgical techniques are available to improve their appearances and / or to achieve better speech production. One is never too old to benefit from recent advancements in the multidisciplinary management of clefts. Secondary Correction of the Lip Deformity This surgery can be performed at any age and will benefit the patient aesthetically and functionally. Among the surgical procedures that may be used to correct secondary lip deformities, it is important to mention the Abbe flap procedure. It may be necessary for people who have a tight upper lip and who do not wish to undergo maxillary adavancement surgery. This operation helps to relieve lip tightness which may be present after advancement procedures. It also increase the fullness and length of the upper lip by moving a portion of the lower lip tissue into the center of the upper lip. At the same time, surgery may be helpful in lengthening a short columella.
Marking and minimal muscle dissection is Lower lip dissection is done to raise the
Abbe Flap is sutured to create a columella Flap is depediclised after three weeks Secondary Correction of Nose This surgery can be performed at any age to improve the appearance of the nose and / or nasal breathing.
Incision marking Dissection of columella
Dissection of lower lateral cartilages and Dissection of upper lateral cartilage and
Harvesting septal graft and septoplasty Repositioning of lower lateral cartilages and approximating upper lateral cartilage
Placement of strut and wedge incision to Final suturing and finishing Secondary Surgery of the Palate Careful evaluation by a highly experienced plastic surgeon and by a speech pathologist may determine the possibility of performing palatal surgery to achieve complete closure of the palatal defect and, at the same time, to retain normal speech production. Individuals who have had cleft palate surgery, but have palatal fistulas, may have the fistulas closed at any time in life. At an older age, palatal fistulas present a serious nuisance and can be closed with a prosthesis to avoid fluid leakage and to keep food from entering the nasal cavity. Fistulas may also affect speech production.
Anterior palatal fistula Closure of the nasal layaer and extent of tongue flap
Tongue flap raised Tongue flap sutured in place and depediclised after three weeks Surgery for Improvement of Speech. Surgical procedures such as pharyngeal flap or pharyngoplasty can correct long-standing cases of velopharyngeal insufficiency. Combined with intensive speech therapy, even adults can achieve more normal speech. Orthognathic Surgery Surgery on the jaws (orthognatic surgery) can help to reduce the flattening of the face which is characteristic of severe cleft deformity. By using modern osteotomy techniques along with bone and cartilage grafts, the upper and lower jaws and facial skeletal framework are moved and appropriately reconstructed. Presurgical orthodontic treatment is usually recommended. Distraction Surgery The latest development in correcting the facial skeletal framework is by using devices known as Distractors. These devices work by incrementally pulling or pushing the bone 1mm at a time over a period of two to three weeks. This slow increase in bone width will result in fresh bone forming in the gap created while the distraction is going on. Other Therapies A Number of surgical, orthodontic and speech related therapies are available to adults who desire additional treatment. In addition, psychological and social consultations can help people deal with the impact of cleft lip and/or palate problems. PLEASE VIEW PREOPERATIVE AND POSTOPERATIVE PICTURES OF PATIENTS IN THE SURGICAL SPECTRUM PAGE OF THIS WEBSITE. (Link this line to surgical spectrum aaaaaa Top |
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