Cleft lip and palate, a condition occurring in approximately 1 in 1000 births, is an anomaly resulting from developmental disorders that can be observed during the 8th-12th weeks of fetal development in the womb.

The biggest problems seen in babies born with this anomaly in the early stages (the first few months after birth) are slow development due to feeding difficulties and frequent upper and lower respiratory tract infections. This fairly common anomaly can be completely corrected with a good and organized multidisciplinary team approach, and children born with this anomaly can lead completely normal lives.

Causes of Cleft Lip

The causes of cleft lip and palate have been studied in great detail, and it has been observed that both genetic and environmental factors play a role in its formation. Genetic factors play a role in 5-10% of all clefts. If there is a history of cleft lip and palate anomalies in the family, the risk of the baby having it also increases. If there is no such history in the family, it may occur as an isolated genetic anomaly along with split mutant genes and chromosomal deletions, or syndromal anomalies such as trisomy D and trisomy E.

Environmental factors play a more active role in cleft formation, and often no specific cause can be found. Experimentally, high doses of cortisone can cause cleft lip and palate in the offspring of pregnant animals. Similarly, a rubella infection during the first three months of pregnancy can cause cleft formation. Smoking and certain vitamin deficiencies have also been shown to have an effect on cleft formation. Furthermore, the risk of cleft lip and palate is higher in individuals with low socioeconomic status.

It should be carefully emphasized that in many cases, parents, especially the mother, are not to blame for cleft lip and palate formation. After birth, parents often blame each other and themselves for giving birth to such a child, but this approach is not only incorrect but also has no positive effect on the child’s treatment.

Epidemiology

Cleft lip and palate are roughly:

Pure cleft lip: 25%
Pure cleft palate: 25%
Cleft lip and palate together: 50%
Cleft lip and palate occur in approximately 1 in 1000 births, while isolated cleft palate occurs in 1 in 2500 births. Cleft lip and palate are twice as common in boys, while isolated cleft palate is twice as common in girls. This is because the mesodermal projections inside the mouth fuse one week later in female infants during the embryonic period; if these mesodermal projections do not fuse, cleft palate occurs. Furthermore, cleft palate and lip are more frequent in East Asians and rarer in African Americans, whereas there is no such distinction in isolated cleft palate.

Cleft Lip Anatomy

Cleft lip anomalies are seen in the upper lip.

Unilateral incomplete cleft lip
Unilateral complete cleft lip
Bilateral incomplete cleft lip
Unilateral complete cleft lip
Bilateral incomplete cleft lip
Midline cleft lips (rarely seen)

Cleft Palate

Soft palate clefts
Hard palate clefts
Submucosal clefts
Presents as bifid uvula (separation of the uvula into two)

These are the general classifications of cleft lip and palate that families should be aware of; Plastic Surgeons use a more anatomical classification.

Cleft Lip Treatment

Surgical repair of cleft lip aims to normalize the anatomy of facial features. Since the lip is both a cosmetic and functional organ, the result should achieve both cosmetic and functional results. Since nasal deformity is always present in cleft lip cases, the goal is to symmetrically reconstruct the lip mucosa and skin along with the functioning lip muscles, while aesthetically creating the phytum columns, vermillion tubercle, and Cupid’s bow. Symmetry of the nostrils, sufficient nasal lining and columella length, symmetrical and adequate nasal tip projection, and symmetrical nostrils are the aims of nasal treatment.

Age for Surgery

It would be more accurate to say the time to begin treatment rather than the age for surgery. Treatment for babies with cleft lip and palate begins within the first 48 hours after birth. Some surgeons perform cleft lip surgery within the first week after birth, while others perform it between 2 and 6 months after birth, taking into account the child’s development. Some surgeons perform a temporary lip operation (lip adhesion) before the actual surgery to prepare the baby’s anatomical structures for the main operation. These methods generally do not differ significantly from each other. The plastic surgeon prefers the method they learned during their training.

Families do not need to panic about this. On the other hand, regardless of the degree of deformity, the baby should be evaluated by an orthodontist immediately after birth, and the anatomical structures should be developed regularly with various intraoral or extraoral devices depending on the type of deformity. While pre-operative orthodontic treatment is very well practiced in the Far East, where this deformity is common, unfortunately, in our country, this is only available in certain centers. In addition, these babies should be examined by a pediatrician immediately after birth to investigate whether there is any additional anomaly. Meanwhile, parents should also be provided with psychological support (especially if a cleft palate and lip diagnosis was not made before birth). Since the sucking function is insufficient in these babies, the mother should be informed about the feeding method (in the mother’s lap with a spoon or special bottle, and using an intraoral obturator in babies with cleft palate).

Performing the surgery within the first week is somewhat more difficult. This is because the baby is not yet stable at this stage and carries risks in terms of anesthesia. Also, since the lip structures have not yet reached sufficient size, performing surgery on them is quite difficult. Babies with cleft lips are most often operated on 2-3 months after birth. It is important that the baby’s weight gain and development are improving before the operation. In babies with poor development, it is best not to perform surgery until the problem is resolved. Before the operation, the baby’s blood tests should be performed to show that values ​​such as hemoglobin, hematocrit, and bleeding clotting time are at normal levels. Since these cases are always operated on under general anesthesia, thorough pre-operative preparation of the baby is essential.

Post-Cleft Lip Care

Patients are generally kept in the hospital on the day and night of the surgery and discharged the next morning. Babies are fed with a spoon in a sitting position starting from the day of the surgery. Contamination of the incision lines by nasal discharge and formula should be prevented. For this purpose, the incision is dressed daily with antibiotic ointments. Keeping the wound lines dry is important. All sutures are removed 5-7 days after surgery.

Cleft Palate Treatment

Cleft palate anomalies vary from complete clefts where the oral and nasal cavities are fully open to clefts only in the dental arch, and clefts only in the soft palate or uvula. Another type of cleft palate is the submucosal cleft. These clefts, which can only be diagnosed by plastic surgeons, are clefts that create a mucosal barrier between the nasal and oral cavities. However, because the muscles beneath this mucosa are separated, the infant develops rhinolalia aperta, or “mumbling” speech where sounds come from the nose. Even though there is no visible cleft in these infants, repair of the submucosal cleft is necessary for the infant to develop proper speech later in life.

Goals of Cleft Palate Treatment

Creating an airtight and watertight velopharyngeal valve
Preserving hearing
Preserving midfacial growth
Creating a functionally and aesthetically adequate upper dental arch
Ensuring proper speech in the future.

Age in Cleft Palate Surgery

Even in infants with only a cleft palate, treatment begins with orthodontic treatments within the first 48 hours after birth. Various intraoral appliances reduce the size of the cleft, facilitating future surgery. As with lip surgery, the appropriate age for cleft palate surgery varies according to different theories. While one group considers 6 months to be suitable, another group finds 12-18 months appropriate. As various studies have shown, these are merely academic debates, and each group claims that their own surgical method is better. If the operation is performed well, it is not actually that important. Nevertheless, a large majority of plastic surgeons recommend waiting at least 9 months for surgery.

Postoperative Care for Cleft Palate Surgery

Postoperative care for babies with cleft palate is more important and challenging than for those with cleft lip. Early postoperative bleeding may occur as a leakage from the mouth, and this bleeding must be closely monitored as it can cause asphyxia (suffocation). Similarly, a swollen tongue can also cause choking if it falls back. In the early period, and at least for the first 48-72 hours, the baby’s formula should be free of particles and should be clear. Although initially difficult to care for, the incisions inside the mouth heal rapidly within 3-4 days.

Cleft lip and palate surgeries are performed by plastic surgeons. While families expect a completely flawless face without scars, a normal nose, and speech similar to that of a child without a cleft lip and palate, the results are often far from perfect. Scarring in the cleft area is unavoidable, especially after lip surgery. Nasal deformities are inevitable in these patients, and these deformities necessitate rhinoplasty after development is complete (17-18 years old). The closure of the palate may not be complete, leading to speech difficulties, food regurgitation through the nose, and potentially requiring the use of dentures. The likelihood of such complications increases because many patients do not have the opportunity to receive orthodontic treatment immediately after birth. However, families should know that these children are completely normal (aside from their deformities) and are not abnormal.