About Cleft Lip and Palate

  • What is cleft lip and palate

    Cleft lip and palate are birth defects of the mouth and lip, also known as oral-facial clefts. A normal fetus has a split lip and palate, but early in pregnancy, the sides of the lip and the roof of the mouth should fuse. Failure of fusion results in cleft lip and/or cleft palate.

    • About 6,000 to 8,000 kids are born in the United States with oral-facial clefts every year
    • Cleft defects are more common in boys
    • Cleft defects are more common in Asians and certain groups of American Indians and are less common in African-Americans
    • Less than 13 percent of patients have other birth defects

    What is cleft lip?

    Cleft lip is an abnormality in which the lip does not completely form during fetal development. The degree of the cleft lip can vary greatly, from mild (notching of the lip) to severe (large opening from the lip up through the nose), in which case the cleft can be very noticeable.

    What is cleft palate?

    Cleft palate occurs when the roof of the mouth does not completely close during fetal development, leaving an opening that can extend into the nasal cavity. The cleft may involve either side of the palate. It can extend from the front of the mouth (hard palate) to the throat (soft palate). Often the cleft will also include the lip.

    Cleft palate is not as noticeable as cleft lip because it is inside the mouth. It may be the only abnormality in the child, or it may be associated with cleft lip or other syndromes. In many cases, other family members have also had a cleft palate at birth.

    Children can also have a less noticeable form of cleft palate, known as a submucuous cleft palate. This is a cleft covered by the mucous menbrane that lines the roof of the mouth.

    What are the different types of cleft lip and palate?

    The different types of cleft defects are categorized according to the location and how much of the lip is involved:

    • unilateral incomplete and complete Unilateral incomplete (l) and complete (r) cleft lip Unilateral defects – the most common type of cleft, affecting one side of the lip, with or without a cleft palate
      • Incomplete unilateral cleft lip – a notching of the lip that does not extend into the nose
      • Incomplete unilateral cleft lip and palate – a notching of the lip that does not extend into the nose, accompanied by a cleft palate
      • Complete unilateral cleft lip and palate – an opening that extends from one side of the lip into the nose and palate
    • bilateral complete Bilateral complete Bilateral defects – affecting both sides of the lip and/or palate, forming two clefts
      • Incomplete bilateral cleft lip and palate – a notching of both sides of the lip that does not extend into the nose
      • Complete bilateral cleft lip and palate – an opening that extends from both sides of the lip into both nostrils
    • Children can also have less noticeable form of cleft lip than those depicted above, known as a form-fruste cleft lip. A form-fruste cleft lip is a small, subtle indentation of the lip.
  • Causes

    The cause of cleft lip and palate is not completely understood, though we know that a combination of genetic and environmental factors contribute to cleft lip and palate. If parents without clefts have a baby with a cleft, the chance they will have another baby with a cleft ranges from 2 percent to 8 percent, which suggests a genetic factor. If clefts run in your family, genetic consultation is recommended.

    In addition, there are several syndromes commonly associated with cleft lip and palate:

  • Complications

    Beyond the cosmetic abnormality, there are other possible complications that may be associated with cleft lip and cleft palate. Our Cleft Lip and Palate Program brings together a team that specializes in providing treatment and long-term support for the condition and related issues.

    Feeding difficulties

    Frequently, the most immediate concern for a baby with a cleft palate is proper nutrition. Sucking is difficult for a child with cleft palate because the roof of the mouth is not formed completely, making it difficult for your baby to create enough suction to pull milk from a standard bottle or the breast. Your baby may appear to be working very hard at feeding, but end up taking only a very small amount of breast milk or formula. When this happens, your baby does not gain weight properly and may require readmission to the hospital. Feeding difficulties occur more with cleft palate abnormalities. Children with cleft lip but without cleft palate do not usually have feeding difficulties.

    Issues of extra air intake and nasal regurgitation (milk coming from the nose) may also occur. With proper teaching, support and patience, most babies with cleft palate go on to do very well with feeding. In some cases, extra support from a feeding team may be recommended. Children’s Hospital has resources that can help with feeding your baby with a cleft palate.

    Ear infections and hearing loss

    Patients with cleft palate in particular are more susceptible to ear infections due to a dysfunction of the tube that connects the middle ear and the throat, causing fluid collection behind the ears.

    Recurrent infections can lead to hearing loss, so patients with cleft palate are often seen by an otolaryngologist (ear, nose and throat specialist) who may recommend the surgical placement of ventilating tubes in the eardrum to prevent fluid build-up and lessen the occurrence of ear infections and associated hearing loss. These tubes are often placed at the time of cleft lip or palate repair, and may need to be replaced as necessary to preserve normal hearing.

    Speech and language delay

    Due to the opening of the roof of the mouth and the lip, muscle function may be decreased, which can lead to delayed or abnormal speech. Referral to a speech therapist should be discussed with your child’s physician.

    Proper speech is dependent upon many factors, but the most common problems associated with cleft lip and palate are related to articulation and nasal escape during speech. Nasal escape may be caused by a short palate or by palate dysfunction due to problems with the palatal muscle. Patients significantly impacted by palatal insufficiency (also called velopharyngeal insufficiency) may require secondary procedures and can be treated in coordination with our Velopharyngeal Dysfunction Program.

    Cosmetic deformity

    As your child grows, he might develop irregularities or asymmetries in the face, either due to clefting or abnormal facial growth. We can help to maximize normal appearance through surgery and other treatments.

    Upper and lower jaw discrepancy

    Some children with cleft lip and palate have jaws that grow at different rates, sometimes leading to difficulties with chewing food, improper alignment of teeth, and even early jaw-joint arthritis. Our team specializes in treating these issues.

    Difficulties with nasal breathing

    It is not uncommon for some children to have a deviated septum, a problem that can cause significant functional impairment. A septoplasty, a surgical procedure that straightens the septum, can help restore the nasal airway.

    Psychosocial issues

    Our specialists in pediatric psychological counseling have helped thousands of children and their families cope with the emotional and social issues that can accompany facial deformities.

    Dental problems

    As a result of the abnormalities, teeth may not erupt normally and orthodontic treatment is usually required. The Cleft Lip and Palate Program team includes board-certified pediatric orthodontists and support from a pediatric dentist who consults with our team.

  • Diagnosis

    unilateral cleft lip ultrasound A child with unilateral cleft lip diagnosed with prenatal ultrasound Cleft lip and palate is generally diagnosed after birth, but in some cases, the diagnosis can be made prenatally. The Cleft Lip and Palate Program works closely with CHOP’s Center for Fetal Diagnosis and Treatment to provide early and accurate diagnosis, allowing our team to assess the best treatment options for your child’s unique situation.

    Early diagnosis and treatment is important to achieve optimal outcomes and correction of cleft lip and palate. The discovery of cleft lip and cleft palate while a baby is still in utero allows both the family and the healthcare team to prepare, anticipate potential challenges and assemble appropriate resources to optimize the outcome for the newborn.

    Cleft abnormalities are most often prenatally diagnosed around or after 30 weeks gestation. At this stage of fetal development, the lip and palate have normally fused, so a failure to fuse may be evident in advanced prenatal imaging tests after this time.

    In most cases, babies born with cleft lip and palate will be transferred to Children’s Hospital after birth, where they will undergo comprehensive imaging and evaluation to determine the most appropriate, individualized course of treatment. We work closely with the Neonatal Craniofacial Program to manage the care of newborns with cleft lip and palate.

    Prenatal evaluation

    If orofacial clefting is suspected on a routine ultrasound study, expectant mothers may be referred to the Center for Fetal Diagnosis and Treatment for a comprehensive, full-day prenatal evaluation. The evaluation includes three diagnostic studies:

    1. A targeted, high-resolution ultrasound evaluates the extent of the cleft as well as overall fetal growth and development.
    2. A fetal echocardiogram closely examines the structure and function of the fetal heart.
    3. An ultrafast fetal MRI allows for a detailed evaluation of the fetal brain, important in a case of suspected cleft lip and palate because development of the brain drives development of the face.

    A detailed review of family, medical and pregnancy history, performed by a board-certified genetic counselor, completes the evaluation process. This information is important in assessing recurrence risks for future pregnancies.

    Planning for delivery and course of treatment for cleft patients

    The results of the evaluation are reviewed and families are counseled by a multidisciplinary team that includes maternal-fetal medicine specialists (MFM), reproductive geneticists, plastic and reconstructive surgeons and nurse coordinators. Individually tailored prenatal and postnatal management plans are developed in partnership with referring physicians.

    If prenatal evaluation suggests a potential underlying syndrome, genetic testing may be performed through amniocentesis. While the diagnosis of an isolated cleft does not necessarily alter delivery planning, babies with multiple prenatally diagnosed birth defects might benefit from delivery in a specialized center such as the Hospital’s Garbose Family Special Delivery Unit (SDU).

    Diagnosis of a birth defect like cleft lip and palate is a challenging time for any family. Together with other specialists from throughout the Hospital, the Cleft Lip and Palate Program and the Center for Fetal Diagnosis and Treatment provide a wide range of resources to support families throughout pregnancy, delivery, and treatment after birth.

  • Treatment

    At The Children’s Hospital of Philadelphia, children with cleft lip and cleft palate receive coordinated care through our Cleft Lip and Palate Program. The multidisciplinary program brings together plastic surgeons, pediatricians, orthodontists and other specialists in order to provide the most comprehensive care for your child.

    The surgical procedures required for each patient with cleft lip and palate will vary depending upon the type and severity of the deformity. Timing and treatment will be adjusted based on each patient’s overall medical needs, but treatment typically includes a combination of the following procedures and stages, performed within general time frames based on development.

    1. Nasoalveolar molding (NAM) (1 week to 3 months of age, if needed)
    2. Cleft lip repair (3 to 6 months)
    3. Cleft palate repair (9 to 18 months)
    4. Palatal expansion (5 to 7 years, if needed)
    5. Alveolar bone graft and fistula repair (6 to 9 years)
    6. Tip rhinoplasty (6 to 9 years)
    7. Phase I orthodontics (6 to 9 years)
    8. Phase II orthodontics (14 to 18 years)
    9. Orthognathic surgery (jaw surgery) (14 to 18 years)
    10. Final touchup surgery (adolescence or adulthood)

    Learn more about what to expect during the course of your child's treatment »

  • Long-term care

    After successful surgical repair of cleft lip and/or cleft palate, Cleft Lip and Palate Program patients will return to our clinic for yearly appointments that will include meetings with specialists, an overall health assessment, and surgical follow-up. Once a patient has reached maturity and completed all of the procedures and treatment, he is generally discharged from annual clinic visits.

    From the moment your child enters our program, our multidisciplinary team partners with your pediatrician or primary care physician to provide coordinated care, making treatment recommendations according to each patient’s unique needs.

    Our program brings together a variety of pediatric specialists, all with extensive experience treating children with cleft lip and palate. Your child’s long-term care will include:

    Surgical follow-up

    Your plastic surgeon will monitor for potential complications (such as formation of fistulas or openings of palate into nose) to determine if any additional palatal procedures are needed. Because the nature of cleft lip and palate makes it possible that a patient will require surgery later in life, we continue to follow our patients in the long term.

    Speech and language assessments

    Shortly after birth, a speech and language pathologist will evaluate your child’s feeding skills and oral-motor development to determine the need for any specific treatment or procedures. In the early years, our team will partner with your pediatrician to monitor for delays in speech and language development, and make any appropriate referrals for early intervention.

    Around 3 years of age, when children have the ability to follow directions and speak in sentences, we begin yearly speech and language evaluations during clinic visits to assess palatal function. Due to changes in the growth of the face and oral structures as your child grows, routine evaluations continue through the teen years to monitor language development, articulation and vocal quality.

    Our internationally recognized speech pathologists work with your plastic surgeon, orthodontist and dentist to plan individualized treatment needed to improve your child’s communication skills and make recommendations for speech therapy or surgery as needed.

    Audiology assessments

    A hearing specialist will provide periodic reports that are used in coordination with evaluations by an ear, nose and throat (ENT) specialist to care for repeated ear infections or hearing loss that frequently accompany cleft palate. The ENT specialist may recommend the surgical placement of ventilating tubes in the eardrum to prevent fluid build-up and lessen the occurrence of infections and hearing loss.

    Dental and orthodontic care

    Our dental and orthodontic specialists keep track of your child’s dental growth and development, making recommendations regarding proper care for developing teeth, as well as the timing of any necessary surgical procedures or corrective orthodontics. Our specialists will partner with your primary care physicians and other specialists to meet each patient’s dental and orthodontic needs.

    Psychosocial counseling and support

    A dedicated psychologist is available to evaluate patients for potential academic, psychological and personal difficulties that may accompany a craniofacial condition. Our specialists can offer supportive therapy and provide guidance in dealing with peers and relatives, as well as make appropriate referrals for comprehensive evaluation and intervention.

Reviewed by Jesse A. Taylor, MD on July 31, 2013