Cleft Lip and Cleft Palate

Published on 21/03/2015 by admin

Filed under Pediatrics

Last modified 22/04/2025

Print this page

rate 1 star rate 2 star rate 3 star rate 4 star rate 5 star
Your rating: none, Average: 0 (0 votes)

This article have been viewed 1532 times

Chapter 14 Cleft Lip and Cleft Palate

PATHOPHYSIOLOGY

Cleft lip and cleft palate are the outcomes of the failure of the soft tissue and/or bony structure to fuse during embryonic development. Cleft lip is a separation of the two sides of the lip. It may affect both sides of the lip as well as the bone and soft tissue of the alveolus. Cleft palate is a midline opening of the palate that results from the failure of the two sides to fuse during embryonic development. The exact cause is unknown, but in most cases it is thought to be multifactorial (a combination of environmental and genetic factors). Clefting is usually an isolated event but may occur as part of a syndrome. A good physical examination is very important to identify any other malformations.

INCIDENCE

1. Cleft lip with or without cleft palate occurs in 1 in 700 live births.

2. Clefting is more common in Hispanic and Asian groups but may be seen in all ethnic groups.

CLINICAL MANIFESTATIONS

1. Visible unilateral or bilateral cleft lip (may be a complete cleft through the nares or an incomplete cleft of part of the lip)

2. Palpable and/or visible cleft palate

3. Cleft of the alveolus (gum line, may affect bone and soft tissue)

4. Nasal distortion

5. Feeding difficulties

COMPLICATIONS

1. Speech and language: speech difficulties may include hypernasality, hyponasality, and compensatory articulation.

2. Dental: malocclusion may occur, with abnormal tooth eruption pattern and abnormal development of the way the mandible and the maxilla meet; excessive dental decay is not unusual.

3. Auditory: chronic otitis media, secondary to eustachian tube dysfunction, may result in hearing loss and speech delay.

4. Psychosocial: altered self-esteem and body image may occur.

LABORATORY AND DIAGNOSTIC TESTS

1. Multidisciplinary team evaluation to counsel parents on clefting and to prepare them for the treatment plan including the surgical plan.

2. Evaluation by a geneticist including laboratory and diagnostic tests if other anomalies exist.

3. Audiologic evaluation to determine if hearing loss is present and if the use of pressure-equalizing tubes is warranted.

SURGICAL MANAGEMENT: CLEFT LIP AND CLEFT PALATE REPAIR

Cleft lip is repaired at 8 to 12 weeks of age if the baby has demonstrated good weight gain. The usual suggestion is that the baby weigh 10 pounds at the time of cleft lip repair. Cleft lip repair is usually done by a plastic surgeon specially trained in the repair of clefts. The plastic surgeon may choose among several methods for repair of the cleft lip, depending on his or her training and experience. Some surgeons choose the Millard repair, which results in a zigzag scar on the lip. Others choose to place the suture lines where the lines of the columella would have been if the clefting had not occurred. Still others choose among other types of repairs. The goal of all cleft lip surgery is the same, however—to achieve lip competence and to create the most natural-appearing lip.

Cleft palate repair is usually performed when the child is 9 to 12 months of age, weighing 18 to 20 pounds. Palatoplasty involves reconstruction of the palatal musculature with the goal of creating an intact palate in order to develop normal speech.

NURSING ASSESSMENT

1. Assess feeding to determine if the baby is receiving adequate caloric intake for growth. Work with the family to develop the best feeding method for that baby. The following are broad guidelines for determining infant calorie requirements:

a. Newborns require about 100 kcal/kg/day to grow. (Multiply the weight in kilograms by 100 to obtain the daily calorie requirements. For example, 2.87 kg × 100 kcal/kg/day = 287 kcal/day.) Standard infant formula and breast milk have 20 kcal/oz.
b. Daily intake (number of ounces needed in a 24-hr period) is determined by first calculating total kilocalories needed per day (as indicated earlier), then dividing by 20. For example, 287 kcal/day ÷ 20 kcal/oz = 14.35 oz/day.
c. Most infants feed every 3 or 4 hours, which amounts to six to eight feedings per day. To determine the volume of each feeding, calculate the number of ounces needed per day and divide by the number of feedings. For example, 14.35 oz divided by 6 (feeding every 4 hr) is 2.4 oz or about 2.5 oz every 4 hours. If the infant is fed more frequently—every 3 hours—divide the ounces per day by 8. In this example, feedings would be 1.8 oz, or about 2 oz, every 3 hours.

2. Assess parents’ interactions with infant for signs of healthy attachment.

3. Screen for maternal depression.

4. Assess respiratory status (respiratory effort, rate, oxygenation, tachycardia).

5. Assess for signs of infection.

NURSING DIAGNOSES

• Parenting, Risk for impaired

• Knowledge, Deficient (regarding clefting)

• Nutrition: less than body requirements, Imbalanced

• Gas exchange, Impaired

• Airway clearance, Ineffective

• Breathing pattern, Ineffective

• Pain, Acute (related to surgical procedure)

NURSING INTERVENTIONS

Preoperative Care

1. Facilitate parents’ positive adjustment to infant.

a. Assist parents in dealing with their feelings about having a child with a visible difference. Encourage expression of feelings.
b. Discuss treatment plan for child, answering parents’ questions.
c. Provide information that instills hope and positive feelings for infant (e.g., comment on infant’s positive features, note positive aspects of parent-child interactions).
d. Encourage parents to engage in caregiving activities.
e. Model acceptance of and delight in the baby, congratulate family on birth of their baby.
f. Arrange meeting with parents of other babies who have undergone surgical and medical treatment.

2. Explain concept of team care and refer to local cleft palate team. Local team listings may be found by contacting the American Cleft Palate–Craniofacial Association (ACPA/CPF), 104 South Estes Drive, Suite 204, Chapel Hill, NC 27514, 919–933–9044, www.info@cleftline.org.

3. Provide information and resources to families regarding cause and treatment of clefting.

a. Provide information on etiology of cleft lip and palate that explains when, during embryonic development, clefting occurred.
b. Explain timing of surgical intervention.
c. Educate parents on feeding techniques.

4. Maintain adequate nutritional intake to promote optimal growth.

a. Babies with isolated cleft lips can usually feed at the breast. It is important to encourage and provide support to a new mother in breast-feeding her baby with a cleft lip.
b. Babies with cleft palates have a difficult time breast-feeding. Most babies with cleft palates cannot generate enough suction at the breast to obtain the necessary nutrition. Mothers of babies with cleft palates are encouraged to pump their breast milk and feed it to their babies via a special nipple and bottle.
c. A variety of bottle-nipple combinations may be used. No single method is best. What is best is what works for the family and allows the infant to finish feeding within 20 to 30 minutes, so that the feeding experience is satisfying for the family.
i. Most babies with a cleft palate can be fed successfully using a soft nipple with an added crosscut (about ⅛ inch each way).
ii. Another feeding system is the Pigeon nipple-bottle system. The Pigeon system has a squeeze bottle and a soft nipple with a valve, which prevents the baby from ingesting excess air during the feeding.
iii. The Mead Johnson cleft lip/palate nurser can also be used. It is a compressible bottle.
d. Feeding instructions are as follows:
i. Place the infant in an upright position (almost sitting).
ii. Place the nipple in the baby’s mouth, trying to position the nipple where there is more palatal tissue.
iii. Support chin with your pinky finger to improve the oral seal.
iv. Assess the infant’s response to feeding.
v. If the baby arches and turns away, the flow of milk may be too rapid. The X cut may be too large. Use a nipple with a smaller X cut. If the baby does not get enough milk, you may need to enlarge the X cut or compress the bottle.
vi. If a squeeze bottle is used, parents are taught to compress the bottle in time with the infant’s chewing motions and allow for rest periods when the infant rests. The parents should be taught to read their infant’s cues.
vii. Burp the baby about halfway through the feeding. The feeding should not last more than 20 to 30 minutes; otherwise the calories taken in will be expended by the feeding effort.
viii. Monitor weight at least twice weekly until weight gain is at least an ounce per day.

5. Monitor respiratory status (respiratory effort, breath sounds, vital signs).

Preoperative Home care

1. There is no special instruction for preoperative care. Bathe infant as usual.

2. Refer to institutional regimen for preoperative procedure, including when to stop feeding before surgery.

Postoperative Care

Cleft Lip Repair

1. Promote adequate nutritional fluid intake.

a. Give breast milk or formula as tolerated (or per institution policy).

2. Promote healing and maintain integrity of child’s incision site.

a. Cleanse suture line gently if required by institutional protocol.
b. Many surgeons use Dermabond skin adhesive to close outer layer of lip (skin). Antibiotic ointment and hydrogen peroxide should not be used in such cases, since they break down the Dermabond.

Cleft Palate Repair

1. Offer clear liquid (when recovered from anesthesia) with same feeding device used before surgery (or per surgeon’s orders). Advance to blenderized or soft diet as tolerated.

2. Avoid placing objects in child’s mouth (suction catheters, tongue depressors, straws, ice chips).

3. Remove toys that are hard or pointed.

4. Monitor for signs of infection or bleeding.

5. Observe for respiratory difficulty.

Both Types of Repair

1. Monitor infant’s level of pain and need for nonpharmacologic interventions (e.g., holding, rocking) and/or pharmacologic interventions (see Appendix I). Ensure that you have a current infant pain scale, either the FLACC (face, legs, activity, cry, consolability) or NPASS (Neonatal Pain, Agitation, and Sedation Scale).

Discharge Planning and Home Care

1. Instruct parents about care of surgical site.

2. Instruct parents about feeding techniques.

3. Instruct parents about signs of infection.

4. Instruct parents on how to position child for feeding.

5. Encourage parents to discuss feelings and concerns about caring for baby at home, as well as about long-term care.

6. Reinforce to parents importance of long-term team care for early treatment of problems, which may include abnormalities in speech, language, hearing, dentition, and occlusion.

7. Inform parents of agencies and support groups for children with cleft lip and/or palate (refer to Appendix G).

CLIENT OUTCOMES

1. Infant’s or child’s incision will heal properly without complications.

2. Infant or child will have appropriate weight gain.

3. Feeding will be pleasurable experience for infant and parent.

4. Parents will demonstrate correct feeding techniques.

5. Parents will demonstrate attachment to their infant or child.

REFERENCES

Andrews-Casal M, et al. Cleft lip with or without cleft palate: Effect of family history on reproductive planning, surgical timing, and parental stress. Cleft Palate Craniofac J. 1998;35(1):52.

Behrman RE, Kiegman R, Jenson HB. Nelson textbook of pediatrics, ed 17, Philadelphia: WB Saunders, 2004.

Golden AS. Cleft lip and cleft palate. Hoekelman RA, et al. Primary pediatric care, ed 3, St. Louis: Mosby, 1997.

Grow JL, Lehman JA. A local perspective on the initial management of children with cleft lip and palate by primary care physicians. Cleft Palate Craniofac J. 2002;39(5):535.

Heidbuchel KL, et al. Dental maturity in children with a complete bilateral cleft lip and palate. Cleft Palate Craniofac J. 2002;39(5):509.

Hockenberry MJ, et al. Wong’s essentials of pediatric nursing, ed 7. St. Louis: Mosby, 2004.

Resnick JI, Zarem HA. Diseases and injuries of the oral region. Burg FD, et al. Gellis and Kagan’s current pediatric therapy, ed 16, Philadelphia: WB Saunders, 1999.

Savage HE. An early intervention guide to infants born with clefts. Infant-toddler intervention. Transdisciplinary J. 1997;7(4):271.

Smahel Z, et al. Changes in craniofacial development due to modifications of the treatment of unilateral cleft lip and palate. Cleft Palate Craniofac J. 1998;35(3):240.

Related posts:

Default ThumbnailAsthma Default ThumbnailEpiglottitis Default ThumbnailHepatitis Default ThumbnailIdiopathic Thrombocytopenic Purpura Default ThumbnailMyringotomy with/without PE Tubes Default ThumbnailRespiratory Tract Infections