Cleft Palate

INTRODUCTION Head and neck formed by some of the bumps and curves, among other frontonasalis processus, processus Nasalis medial and lateral, processus maxillaries, and mandibular processus. The failure of the unification processus processus Nasalis medial maxilla and will lead to openings in the lip (labioschisis) the unilateral or bilateral. When the processus medialis Nasalis, which forms part of two segments of the maxilla, then there is a gap failed to converge on the roof of the mouth or langitan called palatoschisis.1 Cleft palate is a congenital anomaly or palatoschisis on the face where the roof / langitan from the mouth of the palate does not develop normally during pregnancy, resulting in the opening (cleft) palate do not fuse until the Nasalis cavity area, so there is a relationship between the nasal cavity and mouth. Therefore, in palatoschisis, children usually drink at frequent choking and nasal voice. Cleft palate can occur on any part of the palate, including the front of the mouth of sky that is the hard palate or the back of the mouth is the soft palate. 2.3 Cleft palate has a large number of functional and aesthetic implications for patients in their social interactions, especially their ability to communicate effectively and their facial appearance. Correction preferably before the child begins to talk in order to prevent disruption of speech development. Counseling for the child's mother is very important,

especially on how to give drink to the child adequate nutrition when children will undergo reconstructive surgery. Congenital abnormalities should be handled by a team of experts is comprised of surgeons, pediatricians, experts who will follow the development of orthodontic jaw with teeth, and experts who oversee and guide logaoedic ability bicara.1 EMBRYOLOGY Facial tissues, including the lip and palate derived from migration, penetration, and fusion of mesenchymal cells cranioneural head. The third major protrusion on the face (nose, lips, palate) in embryology from facial processus pooling bilateral.4 Palate embryogenesis can be divided into two separate phases of primary palate formation which will be followed by the formation of the secondary palate. Growth of the palate begins approximately at day 35 of pregnancy or week 4 of pregnancy that is characterized by the formation of facial processus. The unification of the processus medialis processus Nasalis maxillaries, followed by unification of the processus lateralis processus Nasalis Nasalis medial, enhance the formation of the primary palate. Failure or damage that occurs in this processus pooling process leading to formation of a gap in the primary palate. 3 Secondary palate formation starts after primary palate perfectly shaped, approximately 9 weeks of pregnancy. Secondary palate is formed from the growing bilateral side of the medial part of processsus maxillaries. Then both sides will meet in the midline with the lifting of this side. When it

develops towards the superior side, the process of unification started. The failure of this union will lead to the formation of a gap in the secondary palate. 3

ANATOMY Palate consists of the hard palate and soft palate (velum) which together form the roof of the mouth cavity and nasal cavity floor. Os maxilla and palatine processus horizontal lamina of the palatine os forming the hard palate. Soft palate is a fibro musculer network formed by several muscles are attached to the posterior hard palate. There are six muscles attached to the hard palate is m. Levator Veli palatine, m. pharyngeus Constrictor superior, m. uvula, m. palatopharyngeus, m.palatoglosus and m.tensor Veli palatini. 3 These three muscles that have the greatest contribution to velopharyngeal function is m.uvula, Veli m.levator palatine, and m. constrictor pharyngeus superior. M.uvula biggest role in lifting the velum during the contraction of this muscle. Veli M.levator palatine velum push towards the superior and posterior to the posterior pharyngeal kedinding attach vellum. The movement of the medial wall of the pharynx, conducted by m. constrictor pharyngeus superior form velum toward the posterior pharyngeal wall to form a strong sphincter. M.palatopharyngeus function under the direction of moving the palate and medial direction. M.palatoglossus primarily as a depressor palate, which plays a role in the formation of nasal venom by allowing a controlled flow of air

through the nasal cavity. The latter muscle is m.tensor Veli palatine. These muscles do not play a role in the movement of the palate. The main function of this muscle resembles the function of the tympanic m.tensor ensure ventilation and drainage of the tuba auditiva. 3 Its blood supply primarily from major a.palatina entering through the major palatine foramen. While a.palatina minor and minor m.palatina passing through the minor palatine foramen. Innervasi palate from the maxilla n.trigeminus branches that form a plexus which menginervasi palate muscles. In addition, the palate also received cranial nerve innerves of VII and IX, which runs adjacent to the posterior of the plexus.

INCIDENCE The incidence of various types of cleft in the report by Veau. Overall incidence of cleft-reported by Fogh Andersen, which is 1 of 655 births and the Ivy which is 1 of 762 births, which is more common in males than females. Palatoschisis increased risk increases with increasing maternal age and the presence of family history of suffering from the same congenital disease. Ethnic factors also affect the incidence palatoschisis. Palatoschisis most often found in Asian races than African race. Palatoschisis on racial incidents Asia about 2.1 / 1 000, 1 / 1000 on the white race, and 0.41 / 1000 on the black race. According to 2004 data, in Indonesia found around 5009 cases of cleft palate of the total population. Palatoschisis without labioschisis have a relatively constant ratio of 0.45 to 0.5 / 1000 births. The most common type is

the uvula bifida with incidence of about 2% of the population. After that followed by a left unilateral complete palatoschisis. 3,5,7,8,9

Etiology In 1963, Falconer put forward a theory that the etiology is multi factorial palatoschisis where the formation of cracks on the palate associated with hereditary factors and environmental factors involved in growth and development processus.4

1. Hereditary factors About 25% of patients who suffer from palatoschisis have a family history of suffering from the same disease. Parents with palatoschisis have a higher risk for having a child with palatoschisis. If only one parent who suffers palatoschisis, then the child may palatoschisis is about 4%. If both parents do not suffer palatoschisis, but it has a single child with palatoschisis the risk of the next generation suffer from the same disease is also about 4%. Conjecture on this subject supported the fact, have successfully isolated an X-linked genes, ie at Xq13-21 locus 6p24.3 in patients with cleft lip and of sky. Another fact that supports, that so many disorders / syndromes with cleft lip and langitan (especially bilateral type), involving the skeletal anomalies, as well as other birth defects. 2. Environmental factors The drugs consumed during pregnancy, such as phenytoin, retinoids (vitamin A group), and steroids are at risk of causing palatoschisis in infants. Infection during the first half of pregnancy such as

rubella and cytomegalovirus infection, associated with the formation of cracks. Alcohol, circumstances that cause hypoxia, smoking, and deficiency of food (such as folic acid deficiency) can cause palatoschisis.3, 4.10

PATHOPHYSIOLOGY Patients with impaired developmental palatoschisis face, velopharyngeal incompetence, abnormal speech development, and impaired function of the fallopian eustachi. All of which gives the pathological symptoms include difficulty in food intake and nutrition, recurrent middle ear infections, deafness, abnormal speech development, and disturbances in facial growth. The existence of the relationship between oral and nasal cavity cause a reduction in the ability to suck on bayi.3 Abnormal insertion of the palatine Veli m.tensor causing incomplete emptying of the middle ear. Recurrent ear infections have been associated with the onset of hearing loss that worsens speech in patients with palatoschisis. Intact velopharyngeal mechanism is important in generating non-nasal sounds, and as a modulator of air flow in the formation of other phonemes that require nasal coupling. (Manipulation of the complex anatomy of this mechanism and difficult, if not successfully performed in early speech development, can cause reduced normal pronunciation) .3

CLASSIFICATION Palatoschisis can be shaped as palatoschisis without labioschisis or accompanied by labioschisis.

Palatoschisis itself can be classified further as the gap is only on the soft palate, or just a gap in the submucosa. Gaps in the overall palate is divided into two, namely the complete (total), which covers the hard palate and soft palate, starting from the foramen insisivum to the posterior, and incomplete (subtotal). Palatoschisis can also be unilateral or bilateral. 2.11 Veau cleft divides into four categories: 1. Cleft soft palate 2. Cleft soft palate and hard palate 3. Cleft lip and palate unilateral complete 4. Cleft lip and palate bilateral complete Line-Y classification for cleft lip and palate is based on modification of the Millard Kernohan. Small circle indicates the foramen incisive; triangle indicates the nasal tip and nasal base.

MANAGEMENT Handling defects in cleft lip and cleft palate is not simple, involving several elements, among others, a Plastic Surgery, orthodontic specialists, ENT specialists to prevent the deal with the onset of otitis media and hearing controls, and anesthesiologist. Speech therapist for speech function. Each specialization has a role that does not overlap but complement each other in dealing with CLP in plenary. 16

1. Non-surgical therapy Palatoschisis is a surgical problem, so there is no specific medical therapy for this condition. However, complications from palatoschisis the problem of food intake, airway obstruction, and otitis media requires

medical treatment first before diperbaiki.3 General Maintenance In Cleft palate In the neonatal period are some things that are emphasized in the treatment of infants with cleft palate that is: 1. Intake of food Food intake in children with cleft palate is usually difficult because of inability to suck, even though the baby can make sucking movements. The ability to swallow should have no effect, adequate nutrition may be given when the milk and soft foods if through the posterior part of the cavum oris. in infants who are breastfed, should be given milk through other tools / special dot unnecessary inhaled by the baby, which when reversed milk can radiate out alone with the optimal amount that is not too large so as to make the patient become choked or too small to make the intake of nutrients become insufficient. Milk bottle made a big hole so that the milk can flow into the back of the mouth and prevent regurgitation into the nose. At the age of 1-2 weeks may be paired obturator to close the gap on the palate, in order to suck milk, or with a spoon with a half-sitting position to prevent the milk through the ceiling splits or wear a hole towards the bottom dot, or using a pacifier that has a hose that length to prevent aspiration. (5) 2. Maintenance of airway Breathing can be a problem child with a cleft, especially if the chin with retro possition (short chin, micrognatic, lower jaw (undershot jaw), genio glossus muscular function is lost and the tongue falls backward, causing partial or total obstruction during inspiration (The Pierre Robin Syndrome)

3. Middle ear disorders Otitis media is a common complication that occurs in the cleft palate and often occurs in children is not inoperable, so that recurrent suppurative otitis is often a problem. The primary complication of persistent middle ear effusion is hearing loss. This problem should receive serious attention so that complications of hearing loss does not occur, especially in children who are at risk of impaired speech due to cleft palate. The main treatment is the incision for the ventilation of the middle ear so that the problem of speech disorders due to conductive deafness can be prevented. (5)

2. Surgical Therapy Surgical treatment in palatoschisis not an emergency case, carried out between the ages of 12-18 months. At that age will provide optimal results to talk functionality by allowing network until cooked on postoperative wound healing process, so before people start to talk so soft palate to function properly. There are some basic surgical techniques that can be used to repair the palate gap, namely: 1. Von Langenbeck technique This technique was first introduced by von Langenbeck surgical technique which is the oldest still in use today. This technique uses a flap technique bipedicel muco periosteal on the hard palate and soft palate. To correct existing abnormalities, the basis of this flap adjacent to the anterior and posterior medial extended to palate to close the gap.

2. VY push-back technique

VY push-back technique involves two uni pedicel flap flap with one or two uni pedicel palate with essentially adjacent to the anterior. The flap is advanced anteriorly and medially rotated while the posterior flap was transferred back to the V to Y technique will increase the length of palate repair. 3. Technique double Opposing Z-plasty This technique was introduced by Furlow to extend the soft palate and create a function of m.levator.

4. Technique Schweckendiek This technique was introduced by Schweckendiek in 1950, in this technique, the soft palate closed (at age 4 months) and followed by the closure of the hard palate when the child approached the age of 18 months. 5. Two-flap palatoplasty technique Introduced by Bardach and Salyer (1984). This technique includes the creation of two pedicle flap with essentially the posterior extends to other parts of the alveolar. This flap is then rotated and advanced medially to correct existing abnormalities.

Speech therapy is needed after the surgery began at the age of palatoplasty ie 2-4 years to train to speak properly and miminimalkan incidence because after the surgery, nasal voice, nasal voice, nasal voice can still occur because children are accustomed to pronounce sounds wrong, there have been compensation mechanism to position the tongue in the position wrong. If after palatoplasty and speech therapy is still obtained pharyngoplasty, nasal voice is done to minimize the sound nasal (nasal escape) is

usually performed at the age of 4-6 years. At the age of children 8-9 years expert orthodontic arch alveolar repair in preparation for alveolar bone graft measures and age 9-10 years plastic surgeons do bone graft surgery on alveolar bone gap as the dentition caninus.16 Care after surgery, immediately after the conscious patient is allowed to drink and liquid food up to three weeks and then are encouraged to eat regular food. Keep oral hygiene if the child already understands. When children are still small, get used to after eating liquid food followed by drinking water. Give antibiotics for three days. At the patient's parents could also be given education, patients should be sleeping position tilted / stomach to prevent aspiration if there is bleeding, should not eat / drink that is too hot or too cold which would cause vasodilation and may not suck / suck for one month post surgery for avoid the post operasi.

COMPLICATIONS Children with palatoschisis potentially suffering from the flu, otitis media, deafness, speech disorders, and abnormal dentition. Moreover, it can cause psychosocial problems. 13 Postoperative complications commonly arise are: a. Airway obstruction As mentioned earlier, postoperative airway obstruction is the most important complications in the period immediately after surgery. This situation arose as a result of prolapse of the tongue into the oro pharynx while the patient was put to sleep by the anesthetist. Intra operative placement of traction

sutures tongue helps in dealing with this condition. Airway obstruction can also be a problem of protracted due to changes in the dynamics of the airway, especially in children with a small mandibula. In some instances, the manufacture and nursing of tracheotomy necessary to have a perfect palate repair b. Bleeding Intra operative bleeding is a complication that occurs's potential. Because the rich blood that is given in palate, intra operative hemorrhage is a potential Complication. Because of the rich blood supply to the palate, which means moving to the bleeding does transfusions. This can be dangerous in infants, in a total volume of blood low. Preoperative assessment of the amount of hemoglobin and platelet count is very important. Epinephrine injection before the incision and use of intra operative do than go to fast oxymetazoline hydrochloride reduces blood loss can occur. To keep from post-operative blood loss, the area containing the palate mucosa should be given avitene or other haemostatic agents. c. Palate fistula Palatal fistula can occur as a complication in the period immediately after surgery, or it could be a pending problem. A fistula in the palate can occur anywhere along the side of the cleft. The incidence has been high enough been reported as much as 34%, and severity of cleft has been reported by that it is associated with the risk of fistula. Postoperative fistula cleft palate can be handled in two ways. In patients without any symptoms, dental prosthesis can be used to cover existing defects with good results. Patients with symptoms required for surgical therapy.

At least the blood supply, especially supply to the anterior is the main reason for the failure of closure of the fistula. Therefore, the anterior and posterior closure of the fistula should be persistent in trying to not more than 6-12 months after surgery, when the blood supply has had the opportunity to stabilished himself. Currently, many centers wait until the patient becomes older (at least 10 years) before attempting to repair the fistula. If a simple closure method fails, such as tissue flap flap anterior tongue may be needed to perform the closing. d. Mid face abnormalities Cleft palate in the handling of several agencies have focused on surgical interventions in advance. One negative effect of growth is retriction maksilla in a few percent of patients. Palate is repaired at an early age can cause a reduction in anterior and posterior dimensional, narrowing of shaft teeth, or abnormally high. Considerable controversy on this topic because the cause of the hypoplasia, whether it is an improvement or effect of the cleft at primary and secondary growth on the face, it is not clear. As many as 25% of patients with unilateral cleft palate repair has been done to require orthognathic surgery. Lefort I osteotomies can be used to repair mid face hypoplasia resulting in a malocclusion and deformity dagu.3 e. Wound expansion Wound expansion is also the result of excess tension. When this happens, the child is allowed to develop until the late stage of the reconstruction of sky, at which time the improvement of scar tissue can be performed without requiring a separate anesthetic.

f. Wound infection Wound infection is a complication that is quite rare because the face has a large enough blood supply. This can occur due to postoperative contamination, unintentional trauma of a child who is active where the sensation on her lips can be reduced postoperatively, and local inflammation that can occur due to node sets. g. Mal position Premaccilar Premaccilar mal position such as slope or retrusion, which can occur after surgery. h. Whistle deformity Whistle deformity is a deficiency of vermilion and may be associated with the retraction along the lip line correction. This can be avoided by use of a total of lateral muscle segment orbicularis. i. Abnormality or asymmetry thick lips This can be avoided with the precise intra operative measurements of the distance of the important curve anatomical.3

Prognosis Although the anatomical correction has been performed, the child continues to suffer from speech disorders necessitating speech therapy that can be obtained in school, but if the child is speaking slowly or carefully it will sound like a child normal.