16.5 Cleft Lip & Cleft Palate

Cleft lip and palate are common congenital defects that occur during fetal development. A cleft lip is failure of the lip tissue to join together, leading to an opening between the sides of the upper lip. A cleft palate refers to an opening in the roof of the mouth. A cleft lip and cleft palate often occur at the same time, but it is possible to have one without the other. These are not only cosmetic defects but also affect nutritional status because they affect the ability of the infant to swallow. Risk factors for the development of cleft lip and/or cleft palate include maternal smoking, gestational diabetes, or exposure to teratogenic substances. Cleft lip and cleft palate also have a genetic component and are associated with other congenital disorders. They are more common in males and the Asian population.^[1],[2]

Pathophysiology

Cleft lip and/or cleft palate develop when these tissues fail to adequately fuse together during fetal development. The lips and palate develop around gestational Weeks 4 to 5. When they fail to develop properly, the result is a cleft lip and/or cleft palate.^[3],[4]

Assessment (Recognizing Cues)

Physical Exam

Cleft lip and/or cleft palate can be visually observed when assessing a newborn client. A cleft lip can range greatly in size from a small hole to a large opening that extends up to the nose of the client. See Figure 16.9^[5] for an image of a cleft lip. A cleft palate can affect the hard and/or soft palate and can be seen in Figure 16.10.^[6]

Due to the effects of cleft lip and/or cleft palate on the infant’s ability to feed, the following signs and symptoms may be present^[7],[8],[9]:

• Fatigue
• Long feeding times
• Respiratory distress
• Difficulty latching

A cleft lip and/or cleft palate can be diagnosed by direct observation or by placing a finger in the mouth of the infant to feel the palate to determine if it is intact or not. Potential complications of a cleft lip and/or cleft palate are speech difficulties, teeth issues, chronic otitis media, and hearing loss. Scarring may also occur due to surgical interventions.^{[10],[11],[12]}

Common Laboratory and Diagnostic Tests

Cleft lip and/or cleft palate may be diagnosed after a prenatal ultrasound, with 3D ultrasounds offering a more accurate diagnosis than 2D ultrasounds. Ultrasounds may be followed by a prenatal MRI to further evaluate the cleft lip and/or cleft palate and to screen for additional congenital defects because cleft lip and/or cleft palate are commonly associated with other congenital syndromes.^[13],[14]

After the infant is born, due to the risk for chronic otitis media and hearing loss, the client should have their hearing evaluated. Evaluation of the teeth should also occur at an early age. Parents of children with cleft lip and/or cleft palate are encouraged to undergo genetic testing and counseling due to the increased risk of having another child with a cleft lip and/or cleft palate.^[15],[16]

Nursing Diagnosis (Analyzing Cues)

Nursing priorities for clients with cleft lip and/or cleft palate include optimizing nutrition and preventing complications.

Nursing diagnoses for clients with cleft lip and/or cleft palate are created based on the specific needs of the client, their signs and symptoms, and the etiology of the disorder. These nursing diagnoses guide the creation of client-specific care plans that encompass client outcomes and nursing interventions, as well the evaluation of those outcomes. These individualized care plans then serve as a guide for client treatment. Possible nursing diagnoses for clients with cleft lip and/or cleft palate are as follows^[17],[18]:

• Risk for impaired verbal communication r/t disease process
• Imbalanced nutrition: less than body requirements r/t difficulty feeding
• Anxiety r/t surgical procedures
• Risk for aspiration r/t disease process

Outcome Identification (Generate Solutions)

Outcome identification encompasses the creation of short- and long-term goals for the client. These goals are used to create expected outcome statements that are based on the specific needs of the client. Expected outcomes should be specific, measurable, achievable, relevant, and timebound (SMART) within a set time frame based on the application of appropriate nursing interventions. Sample expected outcomes for clients with cleft lip and cleft palate include the following^[19]:

• The client will be able to effectively speak and make their needs known after surgery and consultation with a speech therapist.
• The client will maintain their weight within a healthy range that is appropriate for their height until the next follow-up appointment.
• The parents or caregivers of the client will verbalize reduced anxiousness after the teaching session.
• The client will not experience aspiration during the course of treatment.

Interventions (Generate Solutions & Take Action)

Medical Interventions

Initial treatment of cleft lip and/or cleft palate consists of ensuring adequate nutrition. A speech therapist should be involved to teach parents/caregivers how to safely feed the client with assistive devices such as unique bottles or nipples to prevent the reflux of formula or breastmilk into the respiratory system. Oral prosthetics may also be used.^[20]

Definitive treatment for cleft lip and/or cleft palate consists of surgical repair that is typically performed in stages with the lip being fixed prior to the palate. Cleft lip repair can begin after the infant is over ten pounds and ten weeks old, but cleft palate repair will not begin until they are 9-15 months old. Prior to repair of the cleft lip, the lip may be taped in place, or a prosthetic may be placed to help enhance post-surgical symmetry.^[21]

Nursing Interventions

Registered nurses develop interventions based on the expected outcomes of the client. Prior to implementation, the nurse must determine if all previously planned interventions are still suitable based on the current situation of the client. When caring for a client with cleft lip and/or cleft palate, nursing interventions can be divided into nursing assessments, nursing actions, and client teaching, as described in the following subsections.^[22],[23]

Nursing Assessments

• Assess the client for feeding difficulty and signs of aspiration because the client with cleft lip and/or cleft palate can easily aspirate formula or breastmilk.
• Assess the client for signs and symptoms of an ear infection because these are common in those with cleft lip and/or cleft palate.

Nursing Actions

• Ensure adequate nutrition by promoting the use of alternative feeding methods such as the use of special nipples. Breastfeeding may still be possible as breast tissue can be used to close the gap in the lip. The newborn should be fed in an upright position.
• Ensure appropriate referrals are in place with a speech therapist, audiologist, and dietician.
• Perform postoperative wound care per provider orders.
• Encourage caregivers to join support groups or refer to counseling as indicated.

Client Teaching

• Educate the family about surgical repair of the cleft lip and/or cleft palate and encourage questions to help reduce parent/caregiver anxiety.
• Educate the family about postoperative care and provide written handouts.
• Encourage parents/caregivers to provide opportunities for verbal communication by the child with cleft lip and/or cleft palate

Evaluation (Evaluate Outcomes)

Evaluation of client outcomes refers to the process of determining whether or not client outcomes were met by the indicated time frame. This is done by reevaluating the client as a whole and determining if their outcomes have been met, partially met, or not met. If the client outcomes were not met in their entirety, the care plan should be revised and reimplemented. Evaluation of outcomes should occur each time the nurse assesses the client, examines new laboratory or diagnostic data, or interacts with another member of the client’s interdisciplinary team.