Clefts of the lip and palate occur in roughly 1 in 1000 births, making clefts one of the most common congenital anomalies that we see in our center. Clefts occur when embryologic components of the face fail to merge and fuse together as they should, resulting in an abnormal separation of the lip and/or the palate (or roof of the mouth). The lip typically forms by 6 to 8 weeks gestation. The palate forms a little later, generally by 9 weeks gestation. Because the lip and palate form at different times, it is possible to have a cleft only of the lip, only of the palate or of both the lip and the palate. In addition, it is possible for clefts to be partial or complete and involve only one side of the mouth or both.
Cleft Lip and Palate
Causes of cleft lip and palate
Although up to one third of clefts occur where there is a family history of clefts, the majority of cases occur sporadically. Clefts have been associated with a variety of drugs, including alcohol, cigarettes, vitamin A derivatives and some siezure medications. However, none of these medications has been shown to cause clefts. In addition, a number of genes have been identified which may make a cleft slightly more likely to occur. However, no single gene causes clefts. In fact, it is thought a number of genetic and environmental factors must occur simultaneously in order for clefts to occur.
Diagnosis of cleft lip and palate
Since clefts of the lip and palate occur during the first 10 weeks of gestation, it is possible that a cleft may be diagnosed by prenatal ultrasound. When a diagnosis of clefting is made before the baby is born, we recommend that families meet with a member of our team to help them learn what to expect and prepare for the care of their new baby. If the cleft is not seen by prenatal ultrasound, the diagnosis is typically made at the time of birth.
Because babies with clefts of the lip and palate are missing some of the normal structures of the lip and mouth, they may have difficulty with feeding. In particular, babies with clefts may have a difficulty sucking and may require specialized bottles or nipples to be sure they are able to get all of the nutrients they require. In addition, as babies grow, they may also have difficulties with speech. Given these potential problems, it is important that babies with clefts be evaluated early following birth so that parents can receive the education and support they will need to help their baby thrive.
Children with clefts may require a number of surgical procedures throughout childhood and adolescence, depending on the type and extent of the cleft. It should be emphasized that there is no uniform treatment plan that may be applied to all children. Just as each child is unique, the treatment plan for each child with a cleft is tailored to that child's individual needs. An overview of common interventions and treatment is found below.
Feeding evaluation: Since children with clefts frequently have difficulty feeding, any newborn with a cleft should be evaluated by a craniofacial team and a feeding specialist in the first 1-2 weeks of life. At that time, parents learn strategies to help their baby feed well and thrive. Clickfor information on feeding an infant with cleft lip and palate. Nasoalveolar Molding, or NAM, is a technique in which the gum ridges (alveolus) and/or the nose are molded with a specially formed applicance, similar to an orthodontic retainer, prior to surgery to repair the cleft lip. The baby wears the NAM 24 hours a day soon after birth for a period of time (weeks or months) to help mold the soft tissues of the mouth in preparation for the surgery.
For a short video by the parents of one of Dr. van Aalst's patients on the correct way to put in a NAM device on a cleft infant, click here.
For information on arm restraints for your child following surgery, click here.
Hearing evaluation: Babies with clefts of the palate are more likely to develop fluid behind their eardrums (serous otitis media). This benign condition, if left untreated, may progress to ear infections and hearing problems that may affect speech development. Consequently, babies with cleft palate should be seen early by a pediatric otolaryngologist (ENT) to assess whether they may need ear tubes to help drain the fluid and prevent infection. When ear tubes are needed, they are easily placed at the time of the lip or palate repair. We highly encourage coordination of surgical proceedures between the different surgeons who care for babies with clefts in order to minimize the number of trips to the operating room.
Lip repair: Repair of a cleft lip typically occurs at around 3 months of age. Although a variety of methods have been described for repairing clefts of the lip, the most commonly performed repairs recruit lip tissue from the sides in order to close the cleft and lengthen the lip. At the time of lip repair, some surgeons also choose to surgically reshape the nose which is often asymmetric as a result of the cleft. It is our practice to use absorbable sutures in our lip repairs so that no sutures need to be removed following surgery.
Palate repair: The palate is typically repaired when the baby is between 9 and 12 months of age. Some surgeons advocate earlier repair, citing the potential for improved speech outcomes later in childhood. Others point out that earlier repair may adversely affect the way in which the palate and mid portion of the face grow over time. Although the ideal timing of palate surgery continues to be controversial, it seems that most surgeons now agree that choosing a time between 9 and 12 months of age balances those competing concerns regarding speech and facial growth. A variety of repair techniques have been described, and most of the commonly performed repairs lead to good results. The specific choice of technique is dictated by the type and extent of palate clefting observed in a particular baby.
Lip and nose revision surgery: Revision surgery refers to smaller procedures aimed at fine-tuning symmetry and balance of the nose and lip. Not all children require lip and nose revision surgery. The need for this type of surgery is determined by how the child heals from his or her initial surgery and how growth occurs over time. Typically, lip and nose revision surgery (when needed) is first performed as children are preparing to enter mainstream school. Additional revision surgery may be performed at later times to address specific concerns of the child.
Alveolar bone grafting: For children who have clefts going through the gum line, bone must be placed in the gap in order to provide a stable platform for the canine tooth to erupt. During this procedure, a small amount of bone is taken from the hip and packed into the gap. This surgery is typically performed when the child is between 7 and 9 years of age. The precise timing is guided by a pediatric dentist who uses x-rays to determine when the canine tooth is forming and preparing to descend.
Jaw surgery: For some children with clefts, the upper jaw does not grow properly. This leads to an abnormal relationship between the upper and lower teeth, characterized by the lower jaw growing far forward of the upper jaw. When this occurs, the teeth don't fit together properly, and surgical correction may be required. Surgical correction typically involves cutting the upper jaw and allowing it to slide forward, then holding the upper jaw in this new position with small titanium plates and screws. This procedure, called a LeFort I, is typically deferred until the child is nearing completion of his or her facial growth, generally in the middle teenage years. The procedure is also generally preceded by a period of orthodontic preparation with braces.