cleft lip

What are Cleft Lip (CL) and Cleft Palate (CP)?

CL and CP are two distinct facial defects that can occur individually or in combination. Cleft lip with or without cleft palate occurs in 1 out of every 600 live births in the United States.

What is the believed cause of CL/CP?

The cause of CL/CP is believed to be multifactorial, involving a combination of genetic and environmental influences.

How does Cleft Lip form and when does this occur?

Cleft lip, with or without cleft palate, results from the failure of the maxillary processes to fuse with the elevations on the frontal prominence. This failure occurs during the 6^{th} week of gestation.

How does Cleft Palate form and when does this occur?

Cleft palate occurs because the tongue fails to move downward at the correct time, which prevents the palatine processes from fusing. Fusion of the secondary palate typically occurs between 5 and 12 weeks of gestation.

What percentage of children with CL(with or without palate) and CP alone have an associated congenital anomaly?

Approximately 30\% of children with cleft lip (with or without palate) and 50\% of children with cleft palate alone will have an associated congenital anomaly.

What are some risk factors for CL/CP?

Risk factors include:

• Prior family history of CL or CP.

• Environmental factors: smoking during pregnancy, maternal use of alcohol, viral infections, and maternal use of medications such as anticonvulsants and steroids during pregnancy.

• Studies suggest that folate intake during pregnancy may reduce the incidence.

How does Cleft Lip typically manifest clinically?

A cleft involving the lip is readily apparent at birth. It can manifest as a simple dimple in the vermilion border of the lip or as a complete separation extending to the floor of the nose. It may be unilateral or bilateral, with varying degrees of nasal deformity.

How does Cleft Palate typically manifest clinically?

Cleft palate defects that occur without a cleft lip are less obvious and may not be detected at birth. Clefts of the hard palate create a continuous opening between the mouth and nasal cavity. These defects may involve just the soft palate or both the soft and hard palates, and can be unilateral or bilateral.

How is CL/CP typically diagnosed?

CL/CP are usually diagnosed at birth or during the newborn assessment. Prenatal diagnosis via transabdominal ultrasound is possible as early as 13 to 14 weeks’ gestation. Three-dimensional ultrasound or magnetic resonance imaging can offer a clearer picture and detect isolated cleft palate prenatally.

When does cleft lip repair typically occur and why?

Cleft lip repair generally occurs between 3 and 4 months of age. Earlier closure enables the infant to form a better seal around the nipple for feeding, and the sucking motion strengthens the muscles necessary for speech.

When does cleft palate repair typically occur and why?

Cleft palate repair is generally closed between 9 and 18 months of age. This timing protects the formation of tooth buds and allows the infant to develop more normal speech patterns.

What are important aspects of long-term follow-up for children with cleft palate repair?

Children require orthodontic care. Infants with CL/CP are prone to recurrent otitis media, which must be addressed to prevent hearing problems. Audiology screening is emphasized to evaluate for conductive hearing loss.

What are key nursing assessments for infants with CL/CP?

Key nursing assessments include:

1. Defect Description: Location and extent of the cleft lip and palate defect.

2. Associated Conditions: Complete physical assessment for additional defects.

3. Family and Developmental Assessment: Family reactions, child's developmental level, and peer interactions.

4. Potential Problems Before Surgery (Preoperative): Risk of respiratory distress associated with aspiration and poor weight gain associated with poor feeding.

5. Potential Problems After Surgery (Postoperative): Difficulty breathing, pain, feeding issues, and concerns related to development and parental stress.

What are key aspects of preoperative nursing care for CL/CP?

Preoperative care includes:

• Emotional Support and Bonding: Helping parents bond with the infant, explaining the defect, pointing out positive attributes, and referring to support resources.

• Feeding Management: Using special feeders, smaller/more frequent feedings, lactation consultants, nasogastric tube if needed, and teaching proper positioning to prevent aspiration.

• Surgical Preparation: Providing clear explanations, allowing questions, and encouraging holding/cuddling the infant.

What are key aspects of postoperative nursing care for CL/CP, particularly for suture line protection?

Postoperative care includes:

• General Care: Frequent vital signs, maintaining airway, measuring intake/output.

• Feeding Post-Surgery: Starting clear liquids via dropper/syringe/special feeder, positioning infant in sitting position, frequent burping. Cleansing suture line with water or normal saline after each feeding.

• Suture Line Protection:

  • Positioning the infant in a supine position.

  • Maintaining soft elbow immobilizers for two weeks.

  • Avoiding pacifiers.

  • Maintaining suture line or Steri-Strips, applying antibiotic ointment if ordered.

  • Medicating for pain to minimize crying.

  • After cleft palate surgery, avoiding metal utensils or straws.

What are key health promotion strategies for long-term management of CL/CP?

Key strategies, often involving a multidisciplinary team, include:

• Nutrition: Upright feeding, frequent burping, referral for special feeding devices.

• Oral Health: Routine dental/orthodontic evaluation and screening for caries.

• Disease Prevention: Recognizing signs of infection (especially recurrent otitis media) and emphasizing audiology screening.

• Injury Prevention: Avoiding sharp utensils, straws, or metal spoons post-palate repair.

• Psychosocial Support: Encouraging adherence to full treatment, addressing financial constraints, being alert for teasing/poor self-esteem, and promoting parent-infant bonding.

What are the expected outcomes of nursing care for children with CL/CP?

Expected outcomes focus on:

1. Adequate nutritional intake for growth and development.

2. Absence of signs of respiratory distress secondary to aspiration.

3. Parents demonstrating effective coping with the child’s congenital defect and necessary surgeries.