Cleft Lip and Palate
Cleft lip and palate is the result of abnormal fusion of the facial structures during pregnancy. Patients are diagnosed at birth by the presence of a gap or crack of the lip and/or palate. The range of severity for this defect can be quite wide. Cleft lip and/or palate occurs in approximately 1 in 1000 births.
Children can have cleft lip, cleft palate, or both. The most common type is cleft lip and palate (46%), followed by isolated cleft palate (33%) and isolated cleft lip (21%).
In addition to cosmetic problems, these children can have difficulty with feeding and development of speech. Early referral to a craniofacial clinic is needed to ensure the best outcomes for these children.
Patients can be seen by Texas Children's experts in Cleft Lip and Palate Clinic.
Causes & Risk Factors
Smoking during pregnancy and fetal exposure to phenytoin (a drug used to treat seizures) can cause cleft lip and palate. Children of Asian descent have a higher incidence of cleft lip and palate.
A family history of cleft lip and/or palate is also a risk factor for the condition. If the parent or sibling has a cleft lip and palate, the risk that your next child will have the disorder is 4%. If two siblings have cleft lip and palate, the risk of a subsequent child increases to 9%. If the parent and a child have cleft lip and palate, then the risk of the next child having the disorder goes to 17%. For isolated cleft palate, the risk of subsequent pregnancies with the disorder is 2% with one affected child and 6% if the parent had a cleft palate.
Symptoms & Types
The easiest way to classify cleft lip is either as a unilateral or bilateral cleft.
- A unilateral cleft lip only involves one part of the lip that extends toward one of the nostrils.
- A bilateral cleft lip involves two parts of the upper lip and extends towards both nostrils.
Clefts can be considered complete or incomplete.
- Complete cleft lips are clefts that extend up and into the nostril and will involve the tooth bearing structures in the mouth (known as the alveolus).
- An incomplete cleft lip extends toward the nostril, but the skin of the upper lip is not fully separated.
Complete cleft lips usually have a more severe deformity than incomplete clefts. Complete and incomplete clefts can be seen in both unilateral and bilateral cleft lips. A microform cleft lip is the least severe form of cleft lip and is usually only noticeable as lip notching.
Cleft palates occur with cleft lips or by themselves. A cleft palate can involve the uvula (the small tissue that hangs down in the back of the mouth), or a bifid uvula (one that looks like it has split in 2). It can involve only the soft palate, or extend into the soft and hard palate.
If a cleft palate involves both the soft and hard palate and extends into the tooth baring portion of the mouth (alveolus) it is considered a complete cleft palate. Just like in a cleft lip, there can be unilateral and bilateral cleft palates. Unilateral cleft palates involve only one side of the hard palate and alveolus, while bilateral cleft palates involve both sides.
A submucous cleft palate is a unique type. Although the roof of the mouth appears structurally intact, the muscles of the submucous cleft palate are not in the correct position or orientation. This may contribute to speech problems as a child grows.
Diagnosis & Tests
The diagnosis of cleft lip and/or palate is a clinical one, made by physical examination. Genetic testing is frequently done to screen for syndromes that can be seen associated with cleft lip and palate.
Treatment & Care
Care of patients born with a cleft lip or cleft palate begins at birth and continues until they are fully grown. These patients are best treated in by multidisciplinary team.
Treatment can begin as early as 1 week of age for severe cleft lip patients. A technique known as Nasoalveolar Molding (NAM) is used for very severe clefts to decrease the severity of the deformity, provide for an easier repair and increase chances for good outcomes. The craniofacial orthodontist on the cleft team will perform this operation.
Surgical repair of the cleft lip is usually performed in the first 3-6 months of life. The precise timing depends on whether the child needs NAM treatment or not and whether she has any other medical issues that may prevent her from being operated on at such a young age. The cleft lip surgery usually is a day surgery or an overnight admission. Children can resume feeding with a bottle after the procedure.
Doctors usually recommend cleft palate repair when the child is 9 -12 months old. Because this surgery is performed on the child’s airway, proper precautions are taken. These precautions include overnight hospital admission and continuous monitoring of patient oxygenation. The cleft palate surgery is also delayed until this age because the child must be old enough to handle the surgery safely, but not too old where they have begun to produce many sounds.
The cleft palate operation is a functional: its goal is to repair the hole in the palate as well as the muscles in the palate so that appropriate speech production can be achieved. After this repair, constant speech therapy is needed so that any speech problems can be identified early and addressed. Secondary speech surgery is sometimes necessary if the cleft palate is not functioning so that all appropriate sounds can be produced.
After the cleft lip and palate have been repaired, each patient is followed by the Cleft Lip and Palate Clinic on to make sure all issues associated with the cleft have been addressed. These issues can include speech production, feeding issues and sleeping problems.
Further surgical intervention is not necessary until the permanent teeth begin to replace the baby teeth. This usually occurs around 7-9 years of age. The craniofacial orthodontist will work closely with the cleft surgeon to determine the appropriate age for alveolar bone grafting. The cleft that is in the tooth baring segment of the mouth needs to be filled with bone so that when the adult teeth emerge, they have bone to hold them in place. If this is not done, the teeth will simply become loose and fall out. Proper timing of this intervention is critical to preserving as many permanent teeth as possible.
After alveolar bone grafting is done, the craniofacial orthodontist will work to straighten all of the teeth. This is done with braces, just as with children who do not have a cleft.
Future surgical intervention is delayed until adulthood is reached, usually around 16-18 years old. Further surgery to straighten the nose and open the airway should be considered. Some cleft patients need to have their jaw bones adjusted so that their teeth will come out straight. This type of surgery is called oral surgery or orthognathic surgery. Not every cleft patient needs this surgery, so it is important to be evaluated by the clinic team members so that all information can be gathered.
Patients who are in the Cleft Lip and Palate Clinic are usually discharged from care after the age of 18 or when they go to college. All of their care should be concluded by this time. If, however, there are things that they are unhappy with, such as the appearance of their lip or nose, surgical revisions can always be considered.
Living & Managing
Living with cleft lip and/or palate can be challenging for children. The stigma of an abnormal appearing lip or speech production can be difficult to cope with. With attentive team care and intervention (usually surgical), these children do very well and develop normally. It is a long road of treatment, and it is important for both the patient and family to understand that they are not alone.
The Cleft Lip and Palate Clinic team has the resources and time required to help them through the process. We offer a support group for parents of children with cleft lip and palate and a camp for children with cleft lip and palate.
References & Sources
Pediatric Plastic Surgery – Clefts, In E.P. Buchanan, & L. Hollier (Eds.), Seminars in Plastic Surgery; Vol 26; 4, 2012.
Hopper RA, Cutting C, Grayson B. “Cleft Lip and Palate”. Grabbe and Smith’s Plastic Surgery, 6th Edition, Charles H. Thorne. Philadeplhia: Lippincott, Williams and Wilkins, 2007. 201-225. Print.