Cleft Lip and Cleft Palate
Comprehensive Nursing Notes
Introduction
Cleft lip and cleft palate are common congenital anomalies that occur when tissues in the baby’s face and mouth don’t fuse properly during embryonic development. These conditions can occur separately or together and may present various challenges including feeding difficulties, speech problems, dental issues, and psychosocial concerns.
Cleft Lip
A cleft lip is a physical split or separation of the two sides of the upper lip and can extend beyond the base of the nose. It appears as a narrow opening or gap in the skin of the upper lip.
Different types of cleft lip: unilateral incomplete, unilateral complete, and bilateral complete
Cleft Palate
A cleft palate is a split or opening in the roof of the mouth. It can involve the hard palate (bony front portion of the roof of the mouth), the soft palate (soft back portion of the roof of the mouth), or both.
Different types of cleft palate: soft palate only, unilateral complete, and bilateral complete
Embryology and Development
Understanding the embryological development of facial structures helps explain the etiology of cleft lip and palate.
Facial Development Timeline
4-6 Weeks
Five facial prominences develop: frontonasal, paired maxillary, and paired mandibular. These will form the face.
6-7 Weeks
Medial nasal prominences merge with maxillary prominences to form the upper lip and primary palate.
Critical period for cleft lip formation
8-12 Weeks
Secondary palate develops as palatal shelves grow medially and fuse, separating oral and nasal cavities.
Critical period for cleft palate formation
Failure of proper fusion during these critical periods results in cleft lip and/or cleft palate.
Key Developmental Concept
Cleft lip results from failure of the maxillary and medial nasal prominences to fuse properly during weeks 6-7 of embryonic development. Cleft palate results from failure of the palatal shelves to fuse properly during weeks 8-12 of embryonic development.
Classification and Types
Cleft lip and palate can be classified in several ways to guide treatment planning and communication between healthcare providers.
Veau Classification
| Type | Description | Clinical Presentation |
|---|---|---|
| Type I | Cleft of the soft palate only | The defect involves only the soft palate, with the hard palate intact. |
| Type II | Cleft of hard and soft palate | The defect extends from the soft palate to the incisive foramen, with intact alveolus. |
| Type III | Complete unilateral cleft | The defect extends from the soft palate through the hard palate and alveolus on one side. |
| Type IV | Complete bilateral cleft | The defect extends from the soft palate through the hard palate and alveolus on both sides. |
LAHSHAL Classification
The LAHSHAL system is a comprehensive classification system that describes the location and extent of the cleft:
L = Lip (right)
A = Alveolus (right)
H = Hard palate (right)
S = Soft palate
H = Hard palate (left)
A = Alveolus (left)
L = Lip (left)
Uppercase letters indicate complete clefts, while lowercase letters indicate incomplete clefts. A period (.) is used in place of a letter when there is no cleft in that area.
Descriptive Classification
Cleft Lip Types
- Unilateral vs. Bilateral: Affects one or both sides of the lip
- Complete vs. Incomplete: Extends into the nose or stops short of the nostril
- Microform: Minor form with minimal notching of the lip
Cleft Palate Types
- Soft Palate Only: Affects only the soft palate
- Hard and Soft Palate: Affects both hard and soft palate
- Submucous Cleft: Muscle defect covered by intact mucosa
- Bifid Uvula: Split uvula, may indicate submucous cleft
Comprehensive illustration of different types of cleft lip and palate
Epidemiology and Risk Factors
Epidemiology
- Cleft lip with or without cleft palate occurs in approximately 1 in 700 live births worldwide
- Isolated cleft palate occurs in approximately 1 in 2,000 live births
- Prevalence varies by geographic location and ethnicity:
- Highest in Asian and Native American populations (1 in 500)
- Intermediate in Caucasians (1 in 1,000)
- Lowest in African populations (1 in 2,500)
- Cleft lip with or without cleft palate is more common in males (2:1)
- Isolated cleft palate is more common in females (2:1)
Risk Factors
- Genetic factors:
- Family history increases risk by 2-4%
- Associated with over 300 syndromes (e.g., Pierre Robin sequence, Treacher Collins, Stickler syndrome)
- Environmental factors:
- Maternal smoking (increases risk by 30-50%)
- Alcohol consumption during pregnancy
- Maternal diabetes
- Certain medications during pregnancy:
- Anticonvulsants (phenytoin, valproic acid)
- Retinoids
- Corticosteroids
- Nutritional deficiencies (folic acid, zinc, vitamin B6)
- Advanced maternal age
Mnemonic: “CLEFTS”
Risk factors for cleft lip and palate:
- C – Cigarette smoking during pregnancy
- L – Lack of prenatal vitamins (especially folic acid)
- E – Environmental toxins exposure
- F – Family history of cleft lip/palate
- T – Teratogenic medications (anticonvulsants, retinoids)
- S – Syndromes (over 300 syndromes associated with clefts)
Clinical Presentation
The clinical presentation of cleft lip and/or cleft palate varies based on the type and extent of the cleft.
Cleft Lip Presentation
- Visible gap or split in the upper lip
- May be unilateral (one side) or bilateral (both sides)
- May be complete (extending to the nose) or incomplete
- Asymmetrical appearance of the nose and nostrils
- Flattening of the nasal tip on the affected side
- Visible notch or gap in the vermilion border (red part of lip)
- In microform cleft: small notch in the lip or vertical furrow
Cleft Palate Presentation
- Opening in the roof of the mouth (may not be visible externally)
- May affect soft palate, hard palate, or both
- Visible gap between the oral and nasal cavities
- Bifid uvula (split uvula) – may indicate submucous cleft
- Submucous cleft: zone of translucency in midline of palate
- Alveolar ridge may be affected in complete clefts
Associated Clinical Features and Complications
- Feeding difficulties:
- Poor suction due to inability to create negative pressure
- Nasal regurgitation of milk/formula
- Excessive air intake during feeding
- Extended feeding times
- Poor weight gain
- Respiratory issues:
- Nasal airway obstruction
- Increased risk of aspiration
- Sleep apnea (especially with associated syndromes)
- Otologic problems:
- Eustachian tube dysfunction
- Recurrent otitis media
- Conductive hearing loss
- Speech and language issues:
- Hypernasal speech
- Articulation disorders
- Velopharyngeal insufficiency
- Delayed speech development
- Dental anomalies:
- Missing or supernumerary teeth
- Malformed teeth
- Malocclusion
- Enamel defects
Diagnosis and Assessment
Prenatal Diagnosis
- High-resolution ultrasound can detect cleft lip as early as 13 weeks’ gestation (detection rate 70-80%)
- 3D/4D ultrasound improves detection of cleft lip
- Cleft palate is more difficult to detect prenatally
- MRI may be used for more detailed assessment
- Genetic testing may be recommended if:
- Family history of clefts
- Associated anomalies are present
- Suspicion of syndromic cleft
Postnatal Assessment
- Physical examination at birth:
- Visual inspection of lip and oral cavity
- Palpation of palate using gloved finger
- Assessment of nasal deformity
- Evaluate for bifid uvula
- Assessment for associated anomalies:
- Cardiac evaluation
- Hearing screening
- Evaluation for Pierre Robin sequence (micrognathia, glossoptosis)
- Classification and documentation
- Referral to multidisciplinary cleft team
Comprehensive Assessment by Multidisciplinary Team
Initial Assessment
- Detailed history
- Family history
- Documentation of cleft type and extent
- Anthropometric measurements
- Feeding assessment
- Parental counseling
Specialized Assessments
- Genetic evaluation
- Audiological assessment
- Speech and language evaluation
- Dental and orthodontic assessment
- ENT evaluation
- Psychological assessment
Nursing Assessment
- Feeding abilities
- Growth parameters
- Respiratory status
- Family coping and support
- Parent education needs
- Care coordination needs
Important Assessment Considerations
Always assess for airway compromise, especially in infants with Pierre Robin sequence or other syndromes. Signs of respiratory distress include nasal flaring, retractions, cyanosis, and stridor. These require immediate medical attention.
Management
Management of cleft lip and palate requires a multidisciplinary approach to address the various aspects of care. Treatment is typically staged throughout childhood development.
Multidisciplinary Team Approach
Mind map illustrating the multidisciplinary approach to cleft care
Treatment Timeline
| Age | Intervention | Purpose |
|---|---|---|
| Birth – 1 month |
|
Address immediate feeding needs, begin alignment of segments |
| 3-6 months | Cleft lip repair | Restore lip continuity and nasal symmetry |
| 9-18 months | Cleft palate repair | Separate oral and nasal cavities, create functional soft palate for speech |
| 18 months – 3 years |
|
Address speech development, prevent hearing loss from otitis media |
| 4-7 years |
|
Address velopharyngeal insufficiency and speech issues |
| 7-9 years | Alveolar bone graft | Stabilize maxillary arch, provide bone support for permanent teeth |
| Adolescence |
|
Correct dental alignment, improve nasal appearance, correct jaw discrepancies |
| Ongoing | Psychosocial support | Address self-esteem, social integration, and emotional well-being |
Feeding Management
Feeding Challenges
- Inability to create adequate suction for breastfeeding or standard bottle feeding
- Nasal regurgitation during feeding
- Excessive air intake
- Longer feeding times
- Fatigue during feeding
- Risk of aspiration
- Difficulty with weight gain
Specialized Feeding Equipment
- Specialized bottles:
- Mead Johnson Cleft Palate Nurser
- Medela SpecialNeeds/Haberman Feeder
- Pigeon bottle and nipple
- Dr. Brown’s Specialty Feeding System
- Other feeding devices:
- Feeding plates/obturators
- Soft squeeze bottles
- Syringes with soft tubing
Feeding Techniques
Positioning
- Semi-upright position (45° angle or greater)
- Ensure proper head alignment
- For unilateral clefts, position infant with cleft side facing down
- Support cheeks to enhance seal around nipple
Feeding Method
- Keep nipple pointed downward, away from cleft
- Direct flow toward side of mouth without cleft
- Use gentle compression on specialized bottles
- Frequent burping (every 1/2 to 1 ounce)
- Pacing to prevent fatigue
- Limit feeding time to 30 minutes
Feeding Tips for Parents
- Tilt the bottle so the nipple is always filled with milk
- Some milk leakage through the nose is normal and expected
- Keep feeding times under 30 minutes to prevent excessive energy expenditure
- Burp frequently to reduce air accumulation
- Keep baby upright for 20-30 minutes after feeding
- Clean the cleft area with clear water after each feeding
Surgical Management
Cleft Lip Repair
- Typically performed at 3-6 months of age
- Based on “Rule of 10s”:
- Weight ≥ 10 pounds
- Hemoglobin ≥ 10 g/dL
- Age ≥ 10 weeks
- White blood cell count < 10,000/mm³
- Rotation-Advancement (Millard) technique
- Triangular flap (Tennison-Randall) technique
- Straight-line (Fisher) technique
- Restore lip continuity
- Reconstruct philtrum
- Improve nasal symmetry
- Create functional muscle repair
Timing:
Common Techniques:
Goals:
Cleft Palate Repair
- Typically performed at 9-18 months of age
- Balance between:
- Early repair for better speech development
- Delayed repair to minimize growth restriction
- Two-flap palatoplasty (Bardach)
- Furlow double-opposing Z-plasty
- Von Langenbeck technique
- Veau-Wardill-Kilner push-back
- Separate oral and nasal cavities
- Create functional soft palate for speech
- Enable normal feeding
- Minimize growth restriction
Timing:
Common Techniques:
Goals:
Post-Operative Care
After Cleft Lip Repair
- Elbow restraints to prevent touching the surgical site
- Suture line care (keeping clean and moist)
- Lip taping or Logan bow application
- Pain management
- Feeding modifications:
- Spoon or syringe feeding initially
- Transition to soft-tipped syringe or speciality bottle
- Avoid straws, pacifiers, or spoons that could disturb suture line
After Cleft Palate Repair
- Airway monitoring for first 24 hours
- Elbow restraints
- Pain management
- Positioning (side-lying or prone)
- Oral hygiene (gentle rinsing with water after feeding)
- Feeding modifications:
- Liquid or pureed diet for 7-10 days
- No hard or sharp foods for 6 weeks
- No straws, spoons, forks, or pacifiers
- Cup feeding or soft-tipped syringe
Post-Operative Complications to Monitor
- Airway obstruction (especially after palate repair)
- Bleeding from surgical site
- Wound dehiscence (separation of sutured area)
- Infection (redness, swelling, discharge, fever)
- Palatal fistula formation
- Inadequate pain control
- Poor oral intake
- Vomiting or aspiration
Nursing Management
Nurses play a crucial role in the care of infants with cleft lip and palate, from initial assessment and feeding support to pre- and post-operative care and ongoing management.
Nursing Process for Cleft Lip and Palate
Mind map illustrating the nursing process for cleft lip and palate care
Nursing Assessment
Initial Nursing Assessment
Physical Assessment
- Detailed assessment of cleft type and extent
- Airway patency and respiratory status
- Vital signs
- Anthropometric measurements (weight, length, head circumference)
- Associated anomalies
- Feeding abilities
- Hydration status
Psychosocial Assessment
- Parental knowledge and understanding
- Parental emotional response
- Family support system
- Resources available to family
- Cultural factors affecting care
- Financial concerns
Nursing Diagnoses
| Nursing Diagnosis | Related Factors | Defining Characteristics |
|---|---|---|
| Ineffective Breastfeeding |
|
|
| Imbalanced Nutrition: Less Than Body Requirements |
|
|
| Risk for Aspiration |
|
|
| Impaired Oral Mucous Membrane |
|
|
| Acute Pain |
|
|
| Anxiety (Parental) |
|
|
| Risk for Impaired Parenting |
|
|
Nursing Interventions
Mnemonic: “CLEFTS CARE”
Key nursing interventions for infants with cleft lip and palate:
- C – Coordinate with multidisciplinary team
- L – Learn family needs and provide education
- E – Establish effective feeding techniques
- F – Facilitate surgical preparation and recovery
- T – Track growth and development milestones
- S – Support family coping and adaptation
- C – Comfort measures and pain management
- A – Airway management and monitoring
- R – Recognize and prevent complications
- E – Educate on home care and follow-up
Pre-operative Nursing Care
- Nutritional Management:
- Establish effective feeding methods
- Monitor growth parameters
- Ensure adequate nutritional intake
- Teach parents specialized feeding techniques
- Surgical Preparation:
- Educate parents about surgical procedures
- Verify NPO status as ordered
- Complete pre-operative checklist
- Implement pre-surgical protocols (e.g., antibiotics if ordered)
- Family Support:
- Provide emotional support
- Address parental concerns and anxiety
- Encourage parent-infant bonding
- Connect with support resources
Post-operative Nursing Care
- Airway Management:
- Position to maintain airway patency
- Monitor respiratory status
- Suction as needed (using caution near surgical site)
- Elevate head of bed
- Pain Management:
- Assess pain using age-appropriate scale
- Administer analgesics as ordered
- Implement non-pharmacological comfort measures
- Monitor effectiveness of pain management
- Surgical Site Care:
- Maintain wound cleanliness
- Apply prescribed ointments
- Monitor for signs of infection or dehiscence
- Apply elbow restraints as ordered
- Feeding Management:
- Implement post-operative feeding plan
- Use appropriate feeding devices
- Monitor intake and output
- Gradually progress diet as tolerated
Ongoing Nursing Care
Growth and Development
- Monitor growth parameters
- Track developmental milestones
- Assist with early intervention as needed
- Support age-appropriate activities
Family Education
- Home care instructions
- Feeding techniques
- Developmental stimulation
- Recognition of complications
- Follow-up appointments
Care Coordination
- Facilitate referrals
- Coordinate appointments
- Liaise with multidisciplinary team
- Connect with community resources
- Advocate for family needs
Discharge Teaching Priorities
- Proper feeding techniques and positioning
- Surgical site care and monitoring
- Use of elbow restraints
- Signs and symptoms requiring medical attention
- Medication administration (if prescribed)
- Follow-up appointment schedule
- Contact information for healthcare team
Evaluation
Expected Outcomes
- Infant demonstrates adequate nutritional intake and appropriate weight gain
- Surgical wounds heal without complications
- Pain is effectively managed
- Infant achieves developmental milestones
- Parents demonstrate competence in care techniques
- Family demonstrates positive coping and adaptation
- Complications are minimized or prevented
- Appropriate follow-up care is maintained
Complications and Long-term Follow-up
Potential Complications
- Feeding-related:
- Failure to thrive
- Malnutrition
- Dehydration
- Aspiration pneumonia
- Surgical complications:
- Wound dehiscence
- Infection
- Bleeding
- Scarring and contractures
- Palatal fistula formation
- Ear-related:
- Recurrent otitis media
- Eustachian tube dysfunction
- Conductive hearing loss
- Speech and language delays secondary to hearing loss
Long-term Concerns
- Speech and language:
- Velopharyngeal insufficiency (VPI)
- Hypernasal speech
- Articulation disorders
- Language delays
- Dental and orthodontic:
- Dental anomalies (missing, supernumerary, or malformed teeth)
- Malocclusion
- Maxillary growth restriction
- Increased caries risk
- Psychosocial:
- Self-esteem issues
- Social integration challenges
- Bullying/teasing
- Body image concerns
Long-term Follow-up
Children with cleft lip and palate require ongoing multidisciplinary follow-up throughout childhood and adolescence.
Regular Assessments
- Growth and development monitoring
- Speech and language evaluation (typically annually)
- Audiological assessment (every 6-12 months)
- Dental and orthodontic evaluation (every 6 months)
- Psychological assessment as needed
- Team evaluation (typically annually)
Additional Interventions
- Speech therapy
- Myofunctional therapy
- Orthodontic treatment
- Secondary surgeries as needed:
- Fistula repair
- Secondary rhinoplasty
- Pharyngeal flap or sphincteroplasty for VPI
- Orthognathic surgery (jaw alignment)
- Lip or nasal revision
- Psychological support
Transitional Care
As children with cleft lip and palate reach adolescence and adulthood, care transitions from pediatric to adult services. Nurses play a crucial role in facilitating this transition, ensuring continuity of care, and supporting patients in becoming self-advocates for their healthcare needs.
Family Support and Education
Supporting Parents
- Provide clear, accurate information about cleft lip and palate
- Address common misconceptions and guilt feelings
- Demonstrate feeding and care techniques
- Validate emotional responses
- Encourage bonding with infant
- Prepare for reactions from others
- Discuss realistic expectations for treatment
- Connect with parent support groups
- Provide written materials and reliable online resources
Supporting the Child
- Age-appropriate explanations about condition and treatment
- Preparation for surgical procedures
- Strategies for addressing questions from peers
- Building self-esteem and positive self-image
- Encouraging normal activities and social integration
- Connecting with peers who have similar conditions
- Developing coping skills
- Involving child in treatment decisions when age-appropriate
Community Resources
Support Organizations
- Cleft Palate Foundation
- American Cleft Palate-Craniofacial Association
- Operation Smile
- Smile Train
- Local support groups
Early Intervention Services
- Speech therapy
- Developmental therapy
- Educational support
- Home-based services
- Transition to school programs
Financial Resources
- Insurance navigation
- Medicaid/CHIP
- Supplemental Security Income (SSI)
- Charitable organizations
- Hospital financial assistance
Family-Centered Care Approach
Family-centered care is essential in managing cleft lip and palate. This approach recognizes the family as the child’s primary source of support and respects them as full partners in decision-making. Nurses should:
- Recognize family strengths and individuality
- Share information honestly and completely
- Encourage family-to-family support
- Respect cultural diversity
- Recognize and build on family coping skills
- Support family in their caregiving role
- Design services that are flexible and accessible
Summary
Cleft lip and cleft palate are common congenital anomalies resulting from failure of fusion during embryonic development. These conditions present various challenges including feeding difficulties, speech problems, dental issues, and psychosocial concerns. Management requires a multidisciplinary approach involving surgical repair, specialized feeding techniques, and ongoing follow-up care.
Nurses play a crucial role in the care of infants with cleft lip and palate through:
- Feeding support and nutritional management
- Pre- and post-operative care
- Family education and emotional support
- Care coordination
- Monitoring for complications
- Facilitating long-term follow-up
With comprehensive treatment and support, children with cleft lip and palate can achieve excellent functional and aesthetic outcomes, and lead normal, healthy lives.
Final Mnemonic: “SMILE”
Key concepts in cleft lip and palate nursing care:
- S – Surgical management and specialized care
- M – Multidisciplinary team approach
- I – Infant feeding techniques and nutrition
- L – Long-term follow-up and management
- E – Education and emotional support for families
References
- American Cleft Palate-Craniofacial Association. (2018). Parameters for evaluation and treatment of patients with cleft lip/palate or other craniofacial anomalies. The Cleft Palate-Craniofacial Journal, 55(1), 137-156.
- Cleft Palate Foundation. (2022). Feeding your baby with a cleft. Retrieved from https://www.cleftline.org
- Losee, J. E., & Kirschner, R. E. (2015). Comprehensive cleft care. CRC Press.
- Marazita, M. L., & Mooney, M. P. (2018). Current concepts in the embryology and genetics of cleft lip and cleft palate. Clinics in Plastic Surgery, 45(2), 111-129.
- Mayo Clinic. (2024). Cleft lip and cleft palate. Retrieved from https://www.mayoclinic.org/diseases-conditions/cleft-palate/symptoms-causes/syc-20370985
- National Institute of Dental and Craniofacial Research. (2023). Cleft lip and palate. Retrieved from https://www.nidcr.nih.gov/health-info/cleft-lip-palate
- Nurseslabs. (2024). Cleft lip and cleft palate nursing care management. Retrieved from https://nurseslabs.com/cleft-lip-cleft-palate/
- Pediatric Nursing: Caring for Children and Their Families (5th ed.). (2023). Cengage Learning.
- Seattle Children’s Hospital. (2022). Cleft feeding instructions. Retrieved from https://www.seattlechildrens.org/clinics/craniofacial/patient-family-resources/cleft-feeding-instructions/
- Wong’s nursing care of infants and children (11th ed.). (2023). Elsevier.