Different types of cleft lip and palate

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

What causes cleft lip and palate?

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. We are actively researching factors associated with cleft formation. In most cases, clefts occur sporadically; there are no known external factors that contribute to cleft differences.

Is cleft lip and palate genetic?

While there are genetic factors associated with orofacial clefts, prenatal genetic testing and meeting with a genetic counselor are helpful in determining the possibility of having a future child with cleft, and of your child having potential future children with cleft. If a family has no prior history and has a baby with a cleft without an identified cause, the chance of having another child with a cleft is typically less than 15%.

While most of the time there is no known cause for a cleft, there are a few genetic diagnoses that are associated with orofacial clefts, including Stickler syndrome and Van der Woude syndrome.

Complications with cleft lip and palate

Your baby will be thoroughly assessed at birth, focusing on breathing and feeding. 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

Feeding is the immediate concern for a baby with cleft lip and palate. It is difficult for a baby to form suction around the nipple when the roof of the mouth has an opening. Although babies with cleft may appear to be efficient with feeding, the difficulty in making a seal can cause the baby to get fatigued and fall asleep without completing the meal. When this occurs, the baby is at risk for dehydration, weight loss and failure to thrive, in which case the baby will need to be admitted to the hospital. Our experienced cleft program will minimize the risk of this complication by working with you to understand the proper bottles and feeding position. 

If your baby has an incomplete cleft of the lip, the feeding concerns are minimized.

When your baby has a cleft, you may see some milk come out of their nose during or after feeding (this is called nasal regurgitation). When mothers receive education and support, 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. Our program has resources that can help with feeding a baby with a cleft palate.

Ear infections and hearing loss

Children with clefts are more prone to having fluid in the ear, which can result in an ear infection. The muscles of the palate have an important role in helping to drain fluid from the middle ear. When a cleft palate is present, this fluid does not drain normally and can lead to more frequent ear infections. 

After birth, it is common for babies with cleft palate to fail the initial hearing test due to fluid in their ears.

Recurrent ear infections can lead to hearing loss, so children with cleft palate may be referred to our colleagues in Otolaryngology (ear, nose and throat specialists), who may recommend the surgical placement of ventilating tubes in the eardrum. Ear tubes can prevent fluid build-up and lessen the occurrence of ear infections and associated hearing loss. Because our surgical teams collaborate to optimize the care of your baby, the ear tubes are often placed at the time the child is undergoing cleft lip or palate surgical repair. The tubes may need to be replaced as necessary.

Speech and language difficulties

When your child is 18 months old, a visit with our team will include having your child evaluated by a skilled speech and language pathologist. The purpose of this is to identify any developing speech patterns to assess language development. When there are differences noted, your child will be referred to Early Intervention or other resources.

Associated risks with cleft lip and palate

Cosmetic abnormalities

Our cleft team will follow your child throughout their childhood and adolescence. At any of these annual visits, our cleft team will discuss surgical options to treat any concerns that you or your child raise.

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 common for children born with a cleft lip to have a deviated septum, a problem that can make it difficult to breathe out of both sides of the nose. We can perform a surgical procedure called a septoplasty that straightens the septum and can help restore the nasal airway. We can also perform a procedure called a rhinoplasty to round out the tip of the nose and restore symmetry of the nostrils.

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 differences. We follow families closely over time, as babies grow and become adolescents, and help them through the different stages of school and development. We firmly believe that every child born with a cleft of the lip and palate will thrive and not be identified by what nature by chance has given them. Instead, the children we care for will be defined by the team members and families who have loved and nurtured them.

Dental issues

Most children born with a cleft will require orthodontic treatment to help to align their teeth and address any difficulty with proper chewing. The Cleft Lip and Palate Program team includes board-certified craniofacial pediatric orthodontists who address these issues.

Diagnosis of cleft lip and palate

Cleft lip and palate are often identified at the 20-week ultrasound. If your baby is prenatally diagnosed, you will be offered to meet with CHOP’s Center for Fetal Diagnosis and Treatment, our plastic surgeon, our nurse navigator and our advanced practice provider. Together, our team can assess the best treatment options for each child’s unique situation. Our collaborative team focuses on:

• Personalized attention during the pregnancy with a focus on feeding education — even before baby is born.
• Making sure families are connected with the Cleft Lip and Palate Program’s nurse navigator well before delivery to make for seamless care coordination between all specialists and help families prepare for what to expect.

When to see a doctor for cleft lip and palate

Early diagnosis and treatment are 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 the womb allows both the family and the healthcare team to prepare, anticipate potential challenges and assemble appropriate resources to optimize the outcome for the newborn.

Prenatal evaluation

If orofacial cleft is suspected on a routine ultrasound study, expectant mothers may be referred to CHOP’s 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 presence of other anomalies, including findings that might suggest an underlying syndrome.
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. This is important in a case of suspected cleft lip and palate because the development of the brain drives the development of the face. In conjunction with an ultrasound, an MRI also allows for more accurate assessment of cleft palate and aids in detection of soft palate defects, which can be difficult to visualize on sonography.

All families are given the option to meet with a board-certified genetic counselor who completes a detailed review of family, medical and pregnancy history. The genetic counselor discusses potential screening and diagnostic testing options that may aid in the individualized care of each child. Information from this evaluation can also help assess the recurrence risk for future pregnancies.

Planning for delivery and course of treatment for cleft patients

After the results of the genetic evaluation are reviewed, families are counseled by a multidisciplinary team that includes maternal-fetal medicine specialists (MFM), reproductive geneticists, plastic and reconstructive surgeons, and nurse coordinators. The team develops individually tailored prenatal and postnatal management plans in partnership with your family’s physicians.

If prenatal evaluation suggests a potential underlying syndrome, genetic testing may be performed through amniocentesis.

In some cases, if both mother and baby are candidates based on the initial evaluation and overall health, babies may be delivered in CHOP’s Garbose Family Special Delivery Unit (SDU). By being delivered in this specialized birthing unit located within CHOP, newborns have immediate access to pediatric plastic surgeons and other specialists who can assess feeding in the early hours after birth.

Receiving a diagnosis of cleft lip and palate is a challenging time for any family. There are currently no interventions that can be performed before the baby is born. Together with other specialists from throughout CHOP, 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.

Surgery to treat cleft lip and palate

At 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, orthodontists and other specialists to provide the most comprehensive care for every child. We also work closely with the Neonatal Craniofacial Program to manage the care of these newborns.

The surgical procedures required for each patient with cleft lip and palate will vary depending upon the type and severity of the defect. 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 (4 to 6 months)
3. Cleft palate repair (9 to 14 months)
4. Palatal expansion (5 to 7 years, if needed)
5. Alveolar bone graft and fistula repair (5vto 8 years)
6. Tip rhinoplasty (varied timeframes)
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)

Long-term care for cleft lip and palate patients

Your child will be scheduled for an annual cleft team evaluation beginning at 18 months of age. 

From the moment your child enters our program, our multidisciplinary team partners with your pediatrician or primary care team 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 see your child annually to monitor their facial growth and to continue to follow surgical healing and palatal motion. 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, your child will be seen by a speech and language pathologist to evaluate your child’s feeding skills and oral-motor development. This will 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 we will make any appropriate referrals for early intervention.

An initial language evaluation will occur at the first team visit at 18 months of age. After that initial evaluation, your child will be seen annually through adolescence. 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 and orthodontist to plan individualized treatment needed to improve your child’s communication skills and make recommendations for speech therapy or surgery as needed.

Hearing assessments

Your child will have an annual hearing test (audiogram). If the audiologist discovers the presence of ear fluid or any other concerns, you will be referred to an ear/nose/throat surgeon for further evaluation.

Dental and orthodontic care

Routine dental care can be provided by your local dentist and should begin at age 1. Your child will be evaluated by our team orthodontist annually. Our 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, local dentist 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 social challenges that may accompany a craniofacial condition. Our specialists can provide guidance in coping with medical and surgical procedures, navigating visible differences, and facilitating social and school functioning. We can help with appropriate referrals for comprehensive evaluation and intervention when more specific or local support services are needed.