When a cleft lip (CL) occurs, the lip is not contiguous, and when a cleft palate (CP) occurs, there is communication between the oral and nasal cavities. Clefts can range in severity from a simple notch in the upper lip to a complete opening in the lip extending into the floor of the nasal cavity and involving the alveolus to the incisive foramen. Similarly, CP may involve just the soft palate or extend partially or completely through the hard and soft palates. In CP, the alveolus remains intact. A CP may be submucous and not immediately detected if there are subtle or no corresponding clinical signs or symptoms.
The worldwide prevalence of CL and/or CP (CL/P) ranges from 0.8 to 2.7 cases per 1000 live births. There are differences in incidence rates across racial groups, with the lowest reported incidence among African-American populations (approximately 0.5 per 1000) and white populations (approximately 1 per 1000 births) and higher incidence among Native American (approximately 3.5 per 1000) and Asian (approximately 1.7 per 1000) populations.
Although reports vary considerably, it is estimated that out of the total number of infants with CL/P, approximately 50% have combined cleft lip and palate (CLP), whereas 30% have isolated CP, and 20% have isolated CL; CL extending to include the alveolus occurs in approximately 5% of cases. Clefts are usually unilateral ( Figure J-4 ); however, in approximately 10% of cases, clefts are bilateral.
Breastfeeding and CL/P
In these guidelines, breastfeeding refers to direct placement of the baby to the breast for feeding, and breastmilk feeding refers to delivery of breastmilk to the baby via bottle, cup, spoon, or any other means except the breast. Babies use both suction and compression to breastfeed successfully. The ability to generate suction is necessary for attachment to the breast, maintenance of a stable feeding position, and, together with the letdown reflex, milk extraction. Normally, when a baby is feeding, his or her lips flange firmly against the areola, sealing the oral cavity anteriorly. The soft palate rises up and back to contact the pharyngeal walls and seal the oral cavity posteriorly. As the tongue and jaw drop during sucking, the oral cavity increases in size, and suction is generated, drawing milk from the breast. Compression occurs when the baby presses the breast between the tongue and jaw. Suction and compression help milk transfer delivery during breastfeeding.
There is a relationship between the amount of oral pressure generated during feeding and the size/type of cleft and maturity of the baby. For this reason, babies with CL are more likely to breastfeed than those with CP and CLP. Some babies with small clefts of the soft palate generate suction, but others with larger clefts of the soft and/or hard palate may not generate suction. Newborns and premature babies generate lower suction pressures compared with older babies. Babies with CP or CLP have difficulty creating suction because the oral cavity cannot be adequately separated from the nasal cavity during feeding. For these infants, negative consequences may include fatigue during breastfeeding, prolonged feeding times, and impaired growth and nutrition.
The literature describing breastfeeding outcomes is limited, and the evidence is anecdotal and contradictory, making the recommendations that follow challenging.
Quality of evidence (levels of evidence I, II-1, II-2, II-3, and III) for each recommendation, as defined in the U.S. Preventive Services Task Force Appendix A Task Force Ratings, is noted in parentheses.
Summary of Recommendations for Clinical Practice
Based on the reviewed evidence, the following recommendations are made:
Mothers should be encouraged to provide the protective benefits of breastmilk. Evidence suggests that breastfeeding protects against otitis media, which is highly prevalent in this population (II-2). Breastmilk feeding (via cup, spoon, bottle, etc.) should be promoted in preference to artificial milk feeding. Additionally, there is speculative information regarding possible benefits of breastfeeding versus bottle feeding on the development of the oral cavity.
At the same time, mothers should be counseled about likely breastfeeding success. Where direct breastfeeding is unlikely to be the sole feeding method, the need for breastmilk feeding should be encouraged, and, when appropriate, possible delayed transitioning to breastfeeding should be discussed.
Babies with CL/P should be evaluated for breastfeeding on an individual basis. In particular, it is important to take into account the size and location of the baby’s CL/P as well as the mother’s wishes and previous experience with breastfeeding. There is moderate evidence to suggest that infants with CL are able to generate suction (III), and descriptive reports suggest that these infants are often able to breastfeed successfully (III). There is moderate evidence that infants with CP or CLP have difficulty generating suction (I) and have inefficient sucking patterns (I) compared with normal infants. The success rates for breastfeeding infants with CP or CLP are observed to be lower than for infants with CL or no cleft (III) ( Appendix J , Protocol 3).
As in normal breastfeeding, knowledgeable support is important. Mothers who wish to breastfeed should be given immediate access to a lactation specialist to assist with positioning, management of milk supply, and expressing milk for supplemental feeds. Several studies have suggested that there is a need for and benefit from having access to a health professional who specializes in CL/P, such as a clinical nurse specialist, during the newborn/infant periods for specialized advice on feeding a baby with CL/P as well as referrals to appropriate services. Surveys of parents with a child with CL, CLP, or CP indicated a desire for more instruction on feeding challenges as early as possible (III).
Families may benefit from peer support around breastmilk feeding or breastfeeding found through associations like Wild Smile in addition to routine referral to breastfeeding support groups.
Monitoring of a baby’s hydration and weight gain is important while a feeding method is being established. If inadequate, supplemental feeding should be implemented or increased. (See “ABM clinical protocol #3: Hospital guidelines for the use of supplementary feedings in the healthy term breastfed neonate, revised 2009.” ) Infants with CL/P may require supplemental feeds for adequate growth and nutrition (III). There is one study that demonstrated that additional maternal support by a clinical nurse specialist can both improve weight gain outcomes and also facilitate referral to appropriate services (III).
Modification to breastfeeding positions may increase the efficiency and effectiveness of breastfeeding. Positioning recommendations that have been made on the basis of weak evidence (clinical experience or expert opinion) and that should be evaluated for success are:
For infants with CL:
The infant should be held so that the CL is oriented toward the top of the breast (for example, an infant with a [right] CL may feed more efficiently in a cross-cradle position at the right breast and a football/twin style position at the left breast) (III).
The mother may occlude the CL with her thumb or finger and/or support the infant’s cheeks to decrease the width of the cleft and increase closure around the nipple (III).
For bilateral CL, a “face on” straddle position may be more effective than other breastfeeding positions (III).
For infants with CP or CLP:
Positioning should be semiupright to reduce nasal regurgitation and reflux of breastmilk into the eustachian tubes (III).
A football hold/twin position (the body of the infant positioned alongside the mother, rather than across the mother’s lap, and with the infant’s shoulders higher than his or her body) may be more effective than a cross-cradle position (III).
For infants with CP it may also be useful to position the breast toward the “greater segment”—the side of the palate that has the most intact bone. This may facilitate better compression and stop the nipple being pushed into the cleft site (III).
Some experts suggest supporting the infant’s chin to stabilize the jaw during sucking and/or supporting the breast so that it remains in the infant’s mouth (III).
If the cleft is large, some experts suggest that the breast be tipped downward to stop the nipple being pushed into the cleft (III).
Mothers may need to manually express breastmilk into the baby’s mouth to compensate for absent suction and compression and to stimulate the letdown reflex (III).
If a prosthesis is used for orthopedic alignment prior to surgery, caution should be used in advising parents to use such devices to facilitate breastfeeding, as there is strong evidence that they do not significantly increase feeding efficiency or effectiveness (III).
Evidence suggests that breastfeeding can commence/recommence immediately following CL repair and that breastfeeding may be slightly more advantageous than spoon feeding (I). Breastfeeding can commence/recommence 1 day after CP repair without complication to the wound. In a survey of CP surgeons regarding postoperative care after palatoplasty, two thirds of surgeons allowed mothers to breastfeed immediately after surgery (III).
Assessment of the potential for breastfeeding of infants with CL/P as part of a syndrome/sequence should be made on a case-by-case basis, taking into account the additional features of the syndrome that may have an impact on breastfeeding success.