Clef Lip or Palate - An Anatomical Abnormality
Prevalence: Cleft lip or palate appears in about 1 in 700 Caucasian babies, more often among Asians and certain groups of Native Americans, and less frequently among African-Americans.
Causes: The exact cause is hard to determine, but it's likely that genetic and environmental factors interact to prevent either the hard palate (the roof of the mouth), the soft palate (the tissue at the back of the mouth), or the upper lip, all of which normally are split early in fetal development, from closing.
Symptoms: The cleft can be mild (a notch on the upper lip) or severe (involving the lip, the floor of the nostril, and the dental arch). A child with a cleft palate usually needs a speech pathologist. Language development can be affected not only by the structure of the lip and palate but also by the side effects of middle-ear infections, which are common in babies and toddlers with this defect (probably because their ears don't drain properly).
Babies with a cleft palate may also need help with feeding. (Those with a cleft lip generally don't have problems in this area.) Because they have trouble sucking, they must be fed in a sitting position with a special bottle. Depending on the severity of the condition, mothers who breastfeed may have to express milk and bottle-feed baby until the cleft is repaired.
Treatment: Surgical repair for a cleft lip should be done by about 3 months of age. Surgery to repair a cleft palate, which restores the partition between the nose and the mouth, is usually done later -- between 6 and 12 months of age -- to allow for some normal growth of the child's face. At the Little Baby Face Foundation, we often repair clefts in older children.
Though follow-up treatment is sometimes necessary, repair of a cleft lip or palate almost always leaves the child with only minimal scarring and a face that looks like that of most other children.
Microtia and Atresia
• Microtia is a term used to describe malformation of the pinna (external part of the ear).
• Microtia varies in severity from quite minor changes (such as the ear being smaller than expected) to 'classic microtia' where the pinna is missing and is associated with absence of the external auditory meatus (ear canal). Absence of the ear canal is known as atresia. Microtia is frequently accompanied by atresia because the baby's outer ear and the ear canal develop together during pregnancy. In some cases, the ear canal can look normal from out side but ends at a 'blind alley' inside.
• Microtia happens more often in boys than in girls. It usually affects one side: this is known as unilateral microtia. Unilateral microtia more often affects the right ear than the left.
• Approximately one in ten children with microtia are affected both sides when it is known as bilateral microtia.
• Microtia is known to affect about one baby in every 8,000-10,000 births.
• In families where there is already one person with microtia or atresia there is some evidence that suggests the chance of future children having the same condition is about 1 in 20.
• The majority of children with microtia have no other medical problems. However, microtia also happens as one sign of a syndrome. 'Syndrome' means a collection of signs or symptoms that commonly occur together and that doctors recognize as being related. Microtia is known to occur as part of several different syndromes.
What is the cause of microtia and atresia?
Microtia or atresia occurs when the outer part of the ear fails to develop normally during the early stages of pregnancy. The exact reasons for isolated (or non-syndromic) microtia or atresia remain unclear although it is known that it is usually a random, one-off event. It is important for parents to understand it was not caused by anything the mother did wrong before or during the pregnancy. Medical research has suggested that occasionally certain prescribed medications taken during pregnancy, or genetic and/or environmental factors may be involved. Associated factors will be explored at the initial meeting with the multidisciplinary team.
How do we rebuild an ear?
Reconstruction with autogenous cartilage
Autogenous reconstructive surgery involves having an ear built from the child's own rib cartilage and body tissues. Because the ear is sculptured from the child's own tissues it is alive and grows with the child. It is believed that ears constructed in this way are likely to last a lifetime. This type of surgery is not normally done until about the age of six -eight years to allow time for sufficient rib cartilage to have developed, but individual surgeons have different preferences as to the timing of this surgery.
Additionally, leaving surgery until children are a little older can mean that they can be involved in discussion and consent to the procedure and that surgical aftercare is easier.
Sculpting the rib cartilage is an art and technically challenging to do within the operation time and ensuring it looks the same as the opposite ear. This type of reconstruction may take two or more operations to perform and is done by a specialist surgeon within the multidisciplinary microtia team. Children will have a chest scar following the removal of the rib cartilage.
Syndromes and other medical conditions that are associated with microtia
The majority of children with microtia have no other medical problems. However, microtia also happens as one sign of a syndrome. 'Syndrome' means a collection of signs or symptoms that commonly occur together and that doctors recognize as being related. Listed below are some of the syndromes known to include microtia. They are listed with the most common first.
• Treacher Collins
Treacher Collins syndrome is a genetic condition characterized by underdeveloped facial bones. Most children with Treacher Collins syndrome have malformations of or absent external and middle ears. Conductive deafness is caused by anomalies of the outer or middle ear.
• Hemifacial microsomia
Hemifacial microsomia affects the development of the lower half of the face, most commonly the ears, the mouth and the lower jaw. Most children with hemifacial microsomia have malformations of the external or middle ear. Conductive deafness is caused by anomalies of the outer or middle ear. Occasionally children may have a sensorineural deafness.
• Goldenhar
Goldenhar syndrome is characterized by incomplete development of the ear, nose, soft palate, lip, and mandible (jaw). Other parts of the body affected may include the heart, kidneys and lungs. Either the organ is not present on one side or will be underdeveloped. Usually just one side of the body is affected but occasionally both sides are affected. Conductive deafness is caused by anomalies of the outer or middle ear.
Disclaimer: The information contained on this website is not intended as a substitute for independent professional advice.