Things to Know During National Cleft & Craniofacial Awareness Month

• More than 7,000 babies are born annually with a cleft lip or palate.  
• The cleft lip, or palate, is one of several craniofacial anomalies in which the lip doesn’t form completely.  
• Surgery is the only treatment for craniofacial anomalies.  

In the U.S., around 600,000 individuals are diagnosed with a craniofacial condition, according to Children’s National. Meanwhile, around 7,000 babies are born with a cleft lip or palate annually, according to Weill Cornell Medicine, meaning one in every 600 babies has a cleft lip or palate. 

Craniofacial disorder is a broad term to describe malformations of the skull and face, which are often birth defects but also can result from disease or trauma. Craniofacial anomalies, or CFA, are deformities in the growth of the facial or head bones. The craniofacial anomalies can range from mild to so severe surgery is required.   

What Causes Craniofacial Anomalies?

Several factors can lead to the formation of craniofacial anomalies, including alterations in DNA sequencings at conception or maternal folic acid deficiency. Low folic acid intake by the mother during conception can lead to anomalies in the child, so ensure that your pregnant patients maintain adequate folic acid intake, especially during the first trimester.  

Common Types of Craniofacial Anomalies

The most common types of craniofacial anomalies include:   

Cleft lip and palate: A cleft lip occurs when the lip does not form completely. The degree of the condition can vary from child to child. In some babies, it is mild, causing a notching of the lip. In extreme cases, there will be a large opening from the lip up through the nose. A cleft palate is when the mouth does not close completely, leaving an opening that extends into the nasal cavity. The aperture can be on either side of the palate and extend from the front of the mouth (or hard palate) to the throat (or soft palate). Sometimes, cleft palate may also include the lip, causing a cleft.   

Craniosynostosis: Naturally, baby sutures tend to grow for some time after birth. However, in craniosynostosis, the sutures, also known as soft spots of the skull, close too early in the infant developmental stages, causing problems with normal skull and brain growth. Premature suture closure can cause pressure buildup inside the head, causing an increase in size. Moreover, it can also cause the facial or skull bones to appear abnormal, deviating from a symmetrical appearance. 

Hemifacial Microsomia: Hemifacial microsomia is a condition in which the tissues of one side of the infant’s face are underdeveloped, primarily affecting the aural, oral, and mandibular areas. In some cases, both sides of the face may get affected, involving the face and the skull. Other names for the condition include: Goldenhar syndrome, Brachial arch syndrome, Facio-auriculo-vertebral syndrome, Oculo-auriculo-vertebral spectrum, and Lateral facial dysplasia.

Vascular Malformation: A birthmark, or growth, composed of blood vessels present at birth, that causes problems with facial appearance or functions. Other vascular malformations may involve multiple body systems, named according to the type of blood vessel affected. Vascular malformation also is known as Lymphangiomas; Arteriovenous malformations; and Vascular gigantism. 

Hemangioma: Also known as port wine stain, strawberry hemangioma and salmon patch, hemangioma is an abnormal growth of the blood vessel under the skin. It manifests as faint red marks at birth or in the initial months after birth.   

Deformational Plagiocephaly: Deformational, or positional, plagiocephaly means, quite literally, “oblique head.” In this condition, a persistent flat spot develops on one side or the back of the infant’s head, primarily because of sleeping in the same position. It is a common issue after birth and affects around one in 10 infants in the United States. Repositioning and exercises can help eliminate the side’s flatness. In some cases, plagiocephaly occurs before delivery. It’s also more common in twins when two babies grow together and exert pressure on each other.  

How is Craniofacial Anomaly Diagnosed?

In most cases, a craniofacial anomaly is apparent at the time of birth. However, in some cases, it may take some time to show up when the baby’s head starts to grow disproportionately. You can identify a craniofacial anomaly by examination alone. However, to finalize the diagnosis, order an MRI or CT scan of the skull and brain.

The baby’s head grows fast in the first year of life. Therefore, craniofacial anomalies are diagnosed in the first few years when timely treatment is available. You may need to refer the patient to a neurosurgeon who can examine the baby and decide on a future treatment plan.   

Treatment for Craniofacial Anomalies

Craniofacial and minimally invasive surgery are available options for treating craniofacial anomalies. A pediatric neurosurgeon will collaborate with a pediatric plastic surgeon to operate on the patient. Surgical procedures that help correct craniofacial anomalies are:  

Craniofacial Surgery: Craniofacial surgery is performed on patients with craniosynostosis and other syndromes. It helps correct the physical formation of the skull and facial bones while maximizing the baby’s functioning. It can be a complex, intensive surgery in which parts of the skull are removed, replaced, and reshaped. 

Minimally Invasive Surgery: In some cases, when the anomaly is diagnosed before the baby is 3 months old, the surgical team may be able to perform minimally invasive procedures. These are endoscopic procedures that carry a lower risk of blood transfusions and surgery-associated complications. Moreover, they guarantee fast patient recovery. 

Nursing Assessment Priorities

Children with craniofacial abnormalities encounter difficulty with feeding, speech and language, ear infections and hearing, and breathing and nasal resonance, along with dental and orthodontic issues. 

The structural abnormalities can make breastfeeding a challenge, as the infant cannot suck the nipple properly. At that stage, ensuring adequate nutrition and overcoming feeding challenges is crucial for their growth and development.

For speech concerns, early intervention with a speech therapist is suggested to help monitor the child’s progress.

In infants with cleft lip and palate, the alignment and development of teeth may get impacted, leading to common dental problems, such as missing teeth, dental decay, and malocclusion. This could require an orthodontist or a dentist to intervene and ensure proper oral health and function.  

Infants with craniofacial abnormalities also are at risk of developing otitis media and hearing loss because of the Eustachian tube dysfunction. To prevent potential hearing impairment, timely intervention with frequent monitoring is necessary.

Babies born with cleft palates may have nasal resonance, which can lead to nasal airway obstruction. To manage this, nasal surgery, speech therapy, and continuous nasal function monitoring are necessary. 

Nursing Interventions in Craniofacial Abnormalities

Babies diagnosed with cleft lip and palate may have difficulty feeding, which can lead to aspiration. To prevent that, nurses can take the following steps:   

• Monitor and check the infant’s breath rate, depth, and effort. Assess the baby’s skin color and capillary refill, which indicate low oxygenation.  
• Check the baby for abdominal distention caused by swallowing extra air during bottle feed. Abdominal distention can cause upward pressure on the lungs and the diaphragm, eventually compromising respiration.  
• Place the infant in a position of 30 degrees to 45 degrees. This prevents the tongue from falling back and obstructing the airway. The semi-upright position also helps limit fluid regurgitation and burping and prevents the entrance of milk into the Eustachian tubes and middle ear space, hence lowering the incidence of ear infections. 
• Allow the baby to swallow milk during nursing sessions and provide oral care with sterile water. Use a saline solution to gently clean the suture line.  
• Provide adequate nasal and oral suctioning to maintain airway breathing and to improve oxygenation by getting rid of excess fluids and secretions in the nasal or oral cavities.   
• Frequent burps can help reduce excessive swallowing air and prevent spitting up. Hence, feed them slowly and burp frequently.  

With cleft lip and palate, the baby’s sucking ability may be impaired, leading to poor feeding and low body weight. To manage that, nurses can: 

• Check the child’s sucking and swallowing abilities and ensure they have adequate food intake. 
• Document the baby’s daily weight and check if they are underweight.   
• Guide the mother in nursing sessions. Educate them to apply pressure to the areola using her fingers, insert the nipple inside the infant’s mouth, and hold it during the feeding session. Also, encourage them to burp the baby after every nursing session. Moreover, if she finds it difficult to feed the baby, ask the mother to pump the milk and feed it through the bottle.   
• Assess the family member’s anxiety and help them cope with the situation. 

While there are no guidelines to help prevent craniofacial abnormalities, the Centers for Disease Control and Prevention (CDC) suggests that mothers follow a healthy lifestyle before getting pregnant. They should avoid excess sugar consumption, quit smoking, and consume a diet rich in folic acid or take folic acid supplements, especially in the first trimester.   

The Bottom Line

Craniofacial abnormalities can affect an infant’s mental status and facial features and cause speech and feeding difficulties. Surgical intervention can help correct craniofacial abnormalities to some extent. When parents see their baby with an anomaly, they are apprehensive. You should counsel them, help lower their anxiety, and answer their questions patiently. Also, help the baby with feeding sessions and monitor their respiratory status closely. 

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