#133: Cleft Lip & Palate

February 12, 2025 | By Sam Masur

Audio

Summary:

Is there a large cleft in your knowledge of craniofacial anomalies? Dr. Matthew Blessing (Seattle Children’s) joins us to break down this common condition, treatment strategies, and palate-able pearls for the PCP.

Cleft Lip & Palate Pearls

1. Cleft lip and palate abnormalities are often detected on prenatal imaging. However, there are some cases diagnosed postnatally, or even after discharge from the birth hospital. If a newborn presents with failure to thrive and/or reflex/emesis frequently coming out of the nose- keep this on the differential! 
2. Infants  with cleft lip/palates often have feeding difficulties, and this should be normalized with caregivers! Most babies with this condition need to utilize a bottle with a one-way valve for effective feeding. 
3. Surgical repair timelines vary by center, but most cleft lips are repaired at approximately 3-6 months, while cleft palates are often repaired at approximately 9-15 months.

Cleft Lip and Palate Notes

Presentation

Types

Patients can be born with a cleft lip, cleft palate, or both. This can be a unilateral or bilateral defect. Notably, there is also a condition called submucous cleft palate, where the cleft is less overt and the oral mucosa appears intact. Most cleft abnormalities are detected on prenatal imaging, especially those involving a cleft lip. However, there are certainly some children diagnosed in the immediate postnatal period, or even later in infancy. An isolated cleft palate is the most likely to be missed in the prenatal workup.

Exam

Trying to get a good exam of a newborn’s mouth? Dr. Blessing shares this tip: With a gloved hand, apply gentle pressure on the anterior portion of the upper and lower gum lines.. Babies often reflexively open their mouths wide, and you can get a great look at the palate without using a tongue depressor! (You may still need a tongue depressor to get a good look at the uvula, though.) Babies with a cleft palate will often have a V-shaped cleft. If the cleft has a more U-shaped appearance, this is more common in babies with Robin sequence (see below!).

Genetics

Some cleft lip/palate abnormalities occur in isolation. However, if your patient has 2 or more genetic differences, consider broadening your workup to rule out an underlying etiology. (Note: a cleft lip and palate does not count as 2 distinct defects; this would be grouped together as one!) If the patient has an isolated cleft palate, this increases the odds that the patient has a genetic diagnosis. Multi-disciplinary craniofacial centers often include genetics evaluations if clinically indicated. The most common genetic diagnoses with associated clefts are 22q.11.2 microdeletion (aka DiGeorge syndrome) and Robin sequence. 22q.11.2 is the most common microdeletion- patients often also have hooded upper eyelids, a broad nasal bridge, and small external ears. Neonatal hypocalcemia can be an issue in these patients, so check chemistries if there are enough concerning exam findings.

Management

Feeding Strategies

Patients born with cleft abnormalities have an increased incidence of feeding difficulties, especially as infants. Normalize this with parents! Encourage use of bottles with a one-way valve that prevents liquid from going back up into the bottle. Other feeding strategies include the use of lip tape to make it easier for the baby to generate suction from the bottle. In a unilateral defect, positioning the bottle to the unaffected side can also be helpful. In the case of an isolated cleft lip, patients can often still direct breastfeed with lactation support and lip tape. As an added bonus, lip tape can also help bring the premaxilla & skin edges together. Once purees are developmentally appropriate, these patients usually do quite well with those. Counsel parents that acidic purees may be bothersome to some patients.

Surgical Repair(s)

Surgical time will vary by surgical center. In general, cleft lip repair is done between 3-6 months of age, and cleft palate repair is done between 9-15 months of age. Post-operative swelling can be more severe following a palate repair. Children with Robin sequence often undergo a sleep study pre-operatively to evaluate their degree of upper airway obstruction. Several factors go into surgical timing, including the presence of a NG tube (which often precludes lip repairs), nutritional status (wound healing), and corrected gestational age. There have recently been studies that suggest that non-white patients undergo repair at a later age. This is likely multifactorial. Access to an accredited craniofacial center is crucial, even if a patient is followed by another team closer to home as well.

Eustachian Tubes

Eustachian tube dysfunction is extremely common, especially in patients with abnormal clefts. Routine hearing screens are part of the multi-disciplinary craniofacial clinic workflow. Newborns typically pass their hearing screen, but show evidence of conductive hearing loss by their first birthday. This is treated with tympanostomy tubes that are placed at the same time as the palate repair. Fun fact: Tubes placed prior to palate repair are not terribly effective, and often drain excessively.

Toddlerhood & Beyond!

Developmental Concerns

Children with cleft abnormalities often need speech therapy – encourage participation with early intervention programs. A global developmental delay is not expected with this condition – so broaden the differential if there are additional delays in achieving milestones. Patients need to continue to be followed by an experienced craniofacial speech therapist to identify velopharyngeal insufficiency. This happens in a minority of children post-repair, but is important to recognize because this cannot be treated with speech therapy alone.

Teeth

Dental care is extremely important for these patients. Poor dentition can prohibit wound healing if the patient ultimately needs bone grafting. There is also a higher incidence of natal teeth, sometimes near the gum line of the cleft. If these teeth are loose, they often require removal to prevent risk of aspiration. Primary teeth are generally not intervened on, unless they are causing the patient discomfort. As patients develop skeletal maturity, they are at increased risk for underbites (class iii malocclusions).

Citations

Lewis, C. W., Jacob, L. S., Lehmann, C. U., Krol, D., Gereige, R., Karp, J., … & Segura, A. (2017). The primary care pediatrician and the care of children with cleft lip and/or cleft palate. Pediatrics, 139(5).

Mullen, M. C., Yan, F., Ford, M. E., Patel, K. G., & Pecha, P. P. (2023). Racial and ethnic disparities in primary cleft lip and cleft palate repair. The Cleft Palate Craniofacial Journal, 60(4), 482-488.

Reilly, S., Reid, J., Skeat, J., Cahir, P., Mei, C., & Bunik, and the Academy of Breastfeeding Medicine, M. (2013). ABM Clinical Protocol# 17: Guidelines for breastfeeding infants with cleft lip, cleft palate, or cleft lip and palate, Revised 2013. Breastfeeding medicine, 8(4), 349-353. 

Links

To find the closest center accredited by the American Cleft Palate Craniofacial Association, click here.

Goal

Listeners will explain the type of cleft lip and palate abnormalities, as well as the immediate and long-term management of these patients. 

Learning Objectives

After listening to this episode listeners will…  

1. Recall the common types of cleft lip and palate diagnosis, as well as when to consider an underlying genetic diagnosis. 
2. Be familiar with the common newborn feeding strategies in patients with craniofacial clefts. 
3. Recognize the importance of early referral to an accredited, multidisciplinary craniofacial center.
4. Describe the common surgical repair timelines. 
5. Learn about associated diagnoses, such as eustachian tube dysfunction.

Disclosures

Dr Blessing reports no relevant financial disclosures. The Cribsiders report no relevant financial disclosures. 

Citation

Wells E., Blessing M., Berk J, Chiu C, Masur S. “#133: Cleft Lip & Palate”. The Cribsiders Pediatric Podcast. https:/www.thecribsiders.com/ February 12, 2025.