At Plastic and Craniofacial Surgery for Infants and Children, we provide compassionate care for children with congenital disorders and birth defects. Cleft lip and cleft palate deformities can be successfully corrected through advanced surgical techniques. If your child has a cleft lip or cleft palate, request a consultation online or call us at (469) 375-3838 to schedule a time to meet with our skilled team. We take a deep personal interest in the overall wellness of the children who visit our practice, caring for their spirit as we heal their bodies.
Types of Cleft Lip
A cleft lip can range in severity from a slight notch in the red part of the upper lip to a complete separation of the lip extending into the nose. Clefts can occur on one or both sides of the upper lip. The term “incomplete” refers to deformity that does not go through the floor of the nose and the incisive foramen, while the term “complete” includes the floor of the nose and incisive foramen and the entire primary and secondary palates. The laterality of the anomaly is described as right, left, or bilateral. Cleft lip and/or palate may present in any combination. The least affected lip is known as a “forme fruste” or microform cleft lip and may be exhibited as a depression in the lip and slight alteration of the nostril floor or alar shape. This variant may still require definitive lip and nasal repair. Surgery is generally done when the child is 10-12 weeks old.
Types of Cleft Palate
A cleft palate may occur as part of the cleft lip deformity (cleft lip/cleft palate) or as an isolated midline entity involving the secondary palate (isolated cleft palate or ICP). These two occurrences of cleft palate are distinctly different embryologic events (see embryology below) and should be viewed as different congenital anomalies even though the surgical correction and goals of treatment are very similar.
It is important to recognize that the risk of occurrence for ICP and CL/CP is very different (see etiology) with CL/CP being far more common. Furthermore, multiple malformation syndromes are far more likely in ICP (42%) as compared to CL/CP where multiple malformation syndromes occur in only 14%.
By far the most common associated disorder with ICP is the Pierre Robin variant while the most frequently occurring syndrome is Stickler Syndrome (17.5% of syndromic clefts, autosomal dominant, severe myopia, retinal detachment, and glaucoma).
Embryology
Normal facial development is completed in the first three months of life. The events that occur in-utero and result in clefting are not clear. The cleft anterior to the incisive foramen (Primary) and that posterior to the foramen (Secondary) define the anatomical delineation of cleft palate. The primary palate refers to that area that forms the upper lip, columella, maxillary alveolus, and the hard palate anterior to the incisive foramen. The secondary palate forms the soft and hard palate posterior to the incisive foramen. Between the fourth and eighth week, the primary palate formation is usually complete, while the secondary palate formation is completed between the eighth and twelfth weeks. These distinctly different times for the development of the primary and secondary palate form the basis for considering cleft lip with cleft palate and isolated cleft palate as different developmental deformities. The significance of this distinction is underscored when one envisions the difference in familial inheritance as well as the difference in risk for inheriting associated disorders with other organ systems.
Etiology
The exact cause for cleft lip and cleft palate is not known. It is postulated that environmental, genetic chromosomal factors may be responsible for the occurrence of this anomaly with all its variations. Animal experiments have produced clefting in the offspring by varying diets, drug exposures, radiation and vitamin dosages. A protective influence in the prevention of clefting with folic acid supplementation before and during the first trimester is now being postulated. Families known to be at risk for clefting have been used for these studies. Environmental factors in humans have been limited to rubella, in which offspring may demonstrate clefting.
The following table summarizes the risk of CL/P in several family situations.
| Situation | Cleft lip/cleft palate | Isolated cleft palate |
| Frequency of defect in the general population | 0.1% | 0.04% |
| My spouse and I are unaffected. | ||
| 1. We have an affected child. What is the probability that our next baby will have the same condition if: |
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| a. We have no affected relative: | 4.0% | 2.0% |
| b. There is an affected | 4.0% | 7.0% |
| c. Our affected child also has another malformation: | 2.0% | 2.0% |
| d. My spouse and I are related? What is the probablility that our next baby will have some other sort of malformation: |
same as general population | same as general population |
| 2. We have two affected children. What is the probability that our next baby will have the same condition? |
9.0% | 1.0% |
| I am affected (or my spouse is). | ||
| 1. We have no affected children. What is the probability that our next baby will be affected? |
4.0% | 6.0% |
| 2. We have an affected child. What is the probability that our next baby will be affected? |
17.0% | 15.0% |