Approach to the Problem
Oral clefts are among the most common birth defects in children and the most common craniofacial abnormality. A failure of the medial and lateral nasal and maxillary processes to fuse results in a cleft lip, whereas a failure of the palatal shelves to join results in a cleft palate. A combination of genetic and environmental factors plays a role in the etiology of oral clefts. Males are twice as likely as females to present with both a cleft lip and a cleft palate. Females commonly have an isolated cleft palate. Asian and Native American populations have the highest frequency of oral clefts, whereas African Americans have the lowest frequency. Oral clefts can cause feeding problems in the newborn child that may be overcome by the use of special nipples and bottles. The first stage of surgical repair for cleft lip should take place by 3 months of age, and surgical repair for cleft palate should occur by 12 months of age. Ankyloglossia, caused by an abnormally short or thickened frenulum, is an oral cleft variant common in infants and children.
Key Points in the History
• Ankyloglossia can cause breastfeeding difficulty in infants and speech articulation disorders in older children.
• There may be a family history of oral clefts, especially if associated with syndromes that are genetically inherited such as van der Woude syndrome and velocardiofacial syndrome. Up to 40% of children may have a cleft lip or palate that is not associated with a genetic syndrome.
• Maternal factors such as folic acid deficiency and exposure to cigarette smoke, alcohol, corticosteroids, and/or anticonvulsants (phenytoin, valproic acid) may result in cleft lip or palate.
• An infant with cleft lip and palate may cough, gag, and/or show increased work of breathing during feeds.
• Difficulty feeding with a cleft lip or palate results from a decreased ability to form a tight seal around a standard nipple. The poor seal results in insufficient force to generate a strong suck reflex.
• Hearing loss and middle ear disease is highly prevalent in children with cleft palate due to chronic otitis media with effusion (OME) from eustachian tube dysfunction. However, children with isolated cleft lip do not have chronic OME.
• Velopharyngeal dysfunction in children with oral clefts may result in increased nasal resonance, language delay, and speech articulation disorders.
Key Points in the Physical Examination
• Ankyloglossia can lead to a variable degree of limited tongue elevation and mobility.
• Cleft lips are divided into three types: microform, incomplete, and complete.
• A microform cleft lip consists of a simple notch in the vermilion border of the lip.