Cleft Lip and Palate

Cleft lip and cleft palate malformations arise when the developing face fails to fuse early in pregnancy. Cleft lip with or without cleft palate is one of the most common congenital abnormalities. The formation of the fetal face occurs in the first trimester of pregnancy. Cleft lip is a defect of normal continuity of the upper lip and jaw and can be complete or incomplete depending on severity and structures involved. In addition to the obvious external deformity, a multitude of functional problems exist including difficulty feeding, communication between the mouth and nose, and normal speech development.

A cleft palate involves a separation in the roof of the mouth. The defect may encompass both the hard (bone) and soft palate. The mobile soft palate is responsible for controlling airflow in the formation of speech. The escape of air through the palatal incompetence creates a nasal sound.

The incidence of cleft lip is approximately 1 in 1000 births. The incidence varies according to race with American Indians the highest of any known group and the black population possessing the lowest rate. Cleft palate occurs in roughly 1 in 2000 births and females are affected twice as often as males. In contrast, cleft lip occurs more frequently in males and is most often isolated on the left side. The incidence of clefting is increased from falling birth mortality, increasing maternal age, increasing therapeutic drug consumption and increasing associated abnormalities.

The cause of cleft lip and palate is generally regarded as multifactorial with both genetic and environmental influence. An increased chance of clefting exists in a newborn if a sibling, parent or relative has a cleft (2% to 5% with one affected family member verses 0.14% normally). Environmental factors are associated with influences on the fetus during pregnancy including medications, alcohol and tobacco. Cleft lip with or without cleft palate can be isolated or associated with other congential abnormalities.

Carolinas Center for Oral and Facial Surgery is associated with a cleft lip and palate team. The team, consisting of a plastic surgeon, speech pathologist, pediatric dentists, orthodontist, oral surgeons, has been treating the complex functional and cosmetic aspects associated with cleft lip and palate for nearly twenty years. Quarterly meetings are held to monitor facial growth and development as individuals’ transition through speech development and eruption of teeth.

Neonatal care focuses on parental counseling and nursing education as the absence of suckling makes feeding challenging. Hearing is also closely monitored as most children affected with cleft lip and palate have inner ear abnormalities.

Surgery is required at multiple stages for repair of the cleft lip and/or palate. The timing of surgical intervention is based on balancing growth and function. Early surgery can create scarring and slow growth. The ideal is to delay surgery to allow for normal growth until function is necessary.

Closure of the lip (cheilorrhaphy) is generally completed at 3 months of age. The goal is to restore continuity of the upper lip skin and muscle for function. The development of speech dictates the surgical repair of the cleft palate typically at 16 to 24 months of age. Additional surgery may be required on the roof of the mouth to help improve the mobility of the soft palate during speech and limit the escape of air into the nose creating nasal speech (staphylorrhaphy). Restoring the continuity of the tooth bearing upper jaw is accomplished at 8 to 11 years. The timing is based on the development and eruption of permanent teeth adjacent to the cleft. Teeth typically found in this region of the jaw are often absent in clefts. Closure of the commnication between the mouth and nose is also an important goal in correction of the bony cleft. Orthodontic guidance is used throughout the transitioning into the permanent teeth to aid in the orientation.

Multiple surgeries involving the lip, roof of the mouth and jaw restricts growth of the upper jaw creating deficiency in the midface. Surgery to advance the upper jaw is completed in the teenage years to correct the bite (see orthognathic surgery). Additional surgical revisions may be required on the lip, nose, etc. to finalize cosmetics and function.

LINKS:

American Cleft Palate - Craniofacial Association