Cleft Lip and Palate
Cleft lip and palate occur as a result of a fusion during different phases of development inside the mother’s womb (in the embryological stage). The cleft lip and palate manifest in different races at different rates. On average, one in 750-1000 births in Europe exhibit this disorder. It is most common in Japan (1 in about 600 births) and it’s seen the least in black Americans (about 1 in 2200 births).
In this illness, called rabbit lip, 80% of the cases have a lip cleft, on one side and predominantly on the left side. The left / right ratio is 2/1. This condition results from the use of certain medications, vitamin deficiencies, virus infections, cigarette smoking and genetics (familial).
The development of the face in the mother’s womb occurs between the 5th and 8th weeks. It completes by the 12-17th week. In this period, according to the significance of the problem of the gemmules coming together to form the face, a lip cleft anomaly may occur. The cleft ranges from a small indentation on the lip to a completely single or double-sided cleft. An anomaly also can develop from a small indentation on the palate to a single and double cleft of the upper jaw. Embryologically, lip palate development consists of two parts as primary and secondary. The primary part is the part containing the upper lip and teeth of the upper jaw, while the secondary part is the hard and soft palate part.
Clinical Forms of the Illness (Types)
Cleft Lip and Palates are classified according to their embryological development. The cleft may be unilateral, bilateral, symmetrical on both sides, or asymmetrically slit. They are generally classified in 3 groups:
1-Cleft Lip: it is the clefts seen on the upper lip. There are different forms. The upper lip has complete or incomplete clefts. They can be unilateral or bilateral. These clefts may also continue in the teeth part of the palate.
2- Cleft lip and palate: the group which includes the upper lip, the hard palate and the soft palate.
3- Cleft palate: this is the group where the upper lip is fine, but the hard palate or the soft palate are cleft separately or together.
Facial clefts are visible in advanced (morphological) examinations performed during ultrasounds in pregnancies. They are difficult to confuse with another condition. The cleft and lip palate is easy to recognize. After the diagnosis, it should be examined to see whether there’s another accompanying syndrome. Skull base clefts affecting the central nervous system are visible in the rare mid line lip clefts. The rare Trisomie 13, Trisomie 18, Vander Wood syndrome, Treacher Collins syndrome, Stickler syndrome, Shprintzen syndrome, Goldenhar syndrome, Pierre Robin syndromes should be investigated.
The treatment of cleft lip and palate is performed by a team during a long period of time. The team includes pediatricians, orthodontists, orthophonists (speech specialists), ENT specialists, psychologists, and specialists in the field (in coordination with a plastic – reconstructive surgeon experienced in the facial clefts). Treatment begins with improving the approach of the family to the child. Characteristics of the child’s care, nutrition and psychology are taught to the family. Pediatricians regularly follow-up on the weight and height of the child. Lung infections due to food escaping into the lungs and hearing problems related to fluid collection in the middle ear (KBB specialists) are closely monitored in these children.
Immediately after babies are born, they are evaluated by an experienced orthodontist and if necessary, they are given a closing epidermis for the palate (in the palate) to make feeding easier. In cleft lips, the nose and the teeth bones are prepared for surgery with prothesis called Mold (specially prepared for each patient) to stop it from slanting towards the cleft or the shape distorting (due to lack of bone).
The timing of the surgeries can be summarized as follows.
1- During the 1st week of birth, the baby is evaluated in terms of nutrition and nose-to-palate molding (prosthesis for restoring the nose and palate position).
2- Nutrition, lung and hearing problems are evaluated until week 6.
3- If general development is favorable, cutting the lip is carried out in the third month.
4 -Between 6-12 weeks, the palate is repaired.
5- From the 18th month to the 5th year, speech therapy is done with an orthophonist followed up and supported by the family. In this period, the small openings called fistula, which is a complication of the alveolar palate, are surgically repaired.
6- Bone deficiencies are repaired (if any) in the palate or teeth bone between the ages of 7 and 11.
7- With the permanent removal of teeth between the ages of 11 and 16, orthodontic treatment starts, and the teeth are aligned in the best way. Jaw development is followed up on. If there is lagging in the development of the lower or upper jaw, an orthodontic preparation is made for a possible future operation.
8 – After 18 years of age, the necessary corrections are made. A correction will be performed on the lip and nose if needed. If there is a biting disorder, corrective jaw surgery is performed. Deficiencies in the teeth are corrected by an appropriate method.
9- At any stage of the treatment, at the request of a family member or a specialist from the team a psychologist can be brought in for support.
Feeding characteristics in cleft lip and palate
Babies with cleft lip and palates are fed in different ways as they have difficulty breastfeeding. The priority is to try to get the baby to breastfeed. When inadequate, babies with isolated cleft palates can breastfeed with the help of epiphysis closures. Mother’s milk can be given in a bottle. The best tip can be found by testing bottle tips of different lengths. The Baby’s feeding position is very important. The baby should be in a position higher than 45 degrees that is, it should be in a half-sitting position so that the food cannot enter the lungs. Otherwise, the baby will constantly cough and will have lung infections due to escaping of food into the lungs.
Cleft Lip Surgery
When the baby is 3 months old, it can have cleft lip surgery. Firstly, the baby’s condition is evaluated in terms of general anesthesia. Babies with appropriate development and weight (hemoglobin 10 mg / dl, weight 4.5-5 kg) are operated on for lip correction. There are many surgical techniques. The Plastic and Reconstructive surgeon can choose one according to his personal experiences. The most commonly used is Millard’s repair technique. Corrections are made in three plans. Mucosal repair, muscle repair, and skin repair.
In Millard’s technique, the skin muscle and the mucosa on the cleft edges are repaired as a whole by reciprocal rotation and advancement methods. The operation lasts about 1-2 hours and the hospital stay is 1 day. Recovery takes an average of 10 days. The most important thing to know is that the scars on the lips will never go away and will always be a little visible.
Cleft Palate Surgery
After the baby is 6 to 12 months old, a repair operation is done by evaluating the general condition and the width of the ward. Generally, the existing palate tissues are sufficient for repair. Von Langenbeck (simple palatoplasty) is the most commonly used repair technique. This is a method of palate repair and push-back palatoplasty. In these surgeries, the palate tissues on both sides of the palate are repaired in 3 planes (repair of nasal mucosa, repair of soft palate muscles and repair of oral mucosa). The surgery takes 1-2 hours on average, and the hospital stay is 1 day. Recovery time is about 2 weeks. During this time, caretakers must be especially careful not to damage the baby during feeding.
Possible problems and solutions
Problems are minimized when the treatment of infants with cleft lip palate is performed by an experienced team. When we look at details of common problems, the following problems are encountered in each region:
There may not be enough muscle unity in the after cleft lip repair. In this case, the lip may look thinner in the area repaired and may not be able to fully pucker up the lips by making whistling movements. This can result in indentations and irregularity of the scars. The M shape of the mouth may not be fully formed. The solution is to perform a revision surgery and repair the muscles and improve the scars.
Nasal deformities: in cleft lip palates, there are deformities in shape and function of different sizes. Due to the deficiencies of the bones, cartilage and soft tissues on the side of the cleft, the nose lies to one side which may lead to abnormal (formal and functional) developments causing impairments in the septum and conchae. The axis of the nose deviates and the tip droops down. The solution is to resolve these problems with cosmetic nose surgery after 18 years of age. Even if the surgery is performed in the best way, it is important to remember that slight differences from a normal nose will remain due to structural tissue disorders.
Dental and dental bone (alveolar) problems are common problems. Between the lip, palate and nose may be holes (fistulas) that develop due to soft tissue and bone healing problems. They cause liquids to come out of here. Fusion of the alveolar bone and developmental retardation can lead to loss of teeth and impairment in biting. The solution is to close the fistulas with surgery and, if necessary, bone grafts from the hip region, i.e. repair of the alveolar bone, followed by remediation of missing teeth with implants if possible.
The most common problems in palate patients are voice and speech disorders (pharyngeal nausea). These are caused by the fact that the soft palate of the throat area (pharynx) cannot close the throat adequately because of unfilled holes (fistulas) in the palate area. The solution depends on the problem. If there is a fistula, closure of the fistula with surgery is sometimes difficult. If there is midfacial growth retardation, the upper jaw can be brought forward after 18 years of age following orthodontic treatment. Speech disorders can be improved by reducing the soft palate or by eliminating the escape of the soft palate (inadequacies) using various techniques after performing an endoscopic speech examination.
Cavities and misalignments that may occur in teeth are followed-up by dentists and they are treated at every age. Finally, lip and cheek implants are put in to make the cheek bone more distinctive for aesthetic purposes.