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  • Shaken Baby Syndrome

    Tuesday, 21 July 2015 16:28
  • Amniotic fluid problems

    Thursday, 14 May 2015 12:54
  • Choosing a pre-school

    Friday, 10 April 2015 17:50
  • Newborn reflexes

    Tuesday, 03 March 2015 15:49
  • Mastitis

    Tuesday, 03 March 2015 15:41
  • Pelvic floor exercises

    Wednesday, 11 February 2015 17:20
  • Colic

    Wednesday, 11 February 2015 17:11
  • Antenatal Classes

    Monday, 03 June 2013 09:34
  • Strap-in-the-Future

    Thursday, 30 June 2011 13:52

Cleft Lip and Palate

Cleft_Lip_PalateCleft lip (split of the upper lip) and cleft palate (split/opening in the roof of the mouth) are due to a congenital deformity which affects the facial development of a baby during gestation. These conditions may occur singly or together and are called oral clefts. Oral clefts happen when the upper lip or roof of the mouth do not fuse completely in the foetus.

The problem ranges in severity, from a small notch in the lip to a grove that runs into the roof of the mouth and nose. A cleft lip can happen on one or both sides of a baby's mouth. Similarly, a cleft palate can be unilateral or bilateral. Cleft palates can affect the soft palate (the soft tissue at the back of the roof of the mouth) or the hard palate (the bony front part of the roof of the mouth).

Oral clefts can affect the way a baby's or child's face looks. They can also lead to speech, dental and feeding problems, as well as ear infections and hearing loss. Some clefts can be repaired in one operation; others may require further procedures.

Causes:

In very early pregnancy, all babies have an opening in the lip and palate. A baby's lips are formed at approximately 6 weeks of pregnancy and their palate is generally formed by 10 weeks. As the baby grows, these openings gradually merge together and by birth, the skin and muscle between the nose and upper lip, the skin covering the gum ridge, and the bone and muscle in the upper jaw are completely joined. For some reason, in children with oral-facial clefts, these openings fail to close.

In many cases the exact cause of cleft lip and/or cleft palate are unknown. Sometimes these conditions run in families, with the problem being passed down from one or both parents. In most cases, clefting appears to be the result of both genetic and environmental factors presenting during early pregnancy. Drugs, viruses and other toxins can all cause birth defects, including clefts. It is also common for oral-facial clefts to occur together with other syndromes or birth defects.

Diagnosis:

Cleft lips are sometimes diagnosed during an ultra sound examination and can be seen as early as 18 weeks into pregnancy. Cleft palates are more difficult to detect before birth because they are situated inside the mouth.

Cleft lips and palates are generally diagnosed on the physical examination of the mouth, nose and palate at birth. Sometimes oral clefts are associated with other medical conditions that may require additional treatment. Your doctor should be able to tell you if your baby's cleft is a sign of a larger medical condition.

Symptoms:

The primary symptom of oral-facial clefts is a visible opening in the lip or palate. Other symptoms include: changes in nose shape and poorly aligned teeth. Missing or improperly positioned teeth may affect your child's appearance and chewing ability. Other problems that may present because of a cleft lip or palate are: feeding problems, failure to gain weight, poor growth, flow of milk through the nasal passage, frequent colds, and sore throats; tonsil and adenoid problems; repeated ear infections and speech difficulties.

Treatment:

A newborn with an oral cleft may be referred to a team of medical specialists soon after birth. Treatments for children with orofacial clefts vary depending on the severity of the problem, the presence of other syndromes or birth defects, or both; as well as the child's age and needs. Early intervention is essential to alleviate feeding problems or delays in speech development.

A cleft lip is usually surgically repaired by the time a baby is 3 months old. This involves a lip closure, to bring the separated lip muscle parts into place. In more severe cases, where the cleft extends into the base of the nose, a lip adhesion and definitive lip repair may be required to improve lip and nose contour. Cleft palates are generally repaired between 6 and 15 months of age. Palatal repair surgery varies, depending on the type, severity and area of the palate affected. Cleft palate surgery is designed to close the hard palate, maximize jaw growth and development, and to produce normal speech. Palatal surgery focuses on increasing the soft palate's length and closing the palatal cleft space. While, soft palate (velum) surgery aims to close and/or improve muscle control.

Additional surgery may be needed later in life, for bite alignment if the jaw is not properly aligned. Some children may require facial or nasal surgery to improve their facial appearance and function. Dental surgery or orthodontic treatment may be needed when a child is older, to treat dental problems that may be associated with oral clefts. For example, some children with orofacial clefts have missing teeth, especially when the cleft lip extends to the upper gum area. The teeth may need to be corrected as they come in. Children whose cleft involves the gum line may require a procedure known as an alveolar bone graft. This operation is usually done between 8 and 10 years of age, to allow the permanent teeth to grow properly. Bone is generally taken from the hip and is used to create a solid ridge of bone in the gum to properly anchor the teeth. Orthodontic treatment may follow the bone graft and some children may need a dental implant in early adulthood when growth is complete.

Babies, who are severely affected and find it difficult to feed, may be fitted with a dental plate initially. This is placed in the roof of the mouth, to make eating and drinking easier until surgery can be done. There are also several special types of bottles, known as "cleft feeders", for infants with cleft lip or palate. These devices allow you to feed your baby the same type of formula as a child without a cleft. If your child is functionally unable to nurse, you may wish to use a breast pump to collect your milk and feed it to your baby with a cleft feeder, if this is your preference.

Children with a cleft lip and/or palate are prone to glue ear, caused by the persistent build up of fluid in the middle ear. Medications may be used to treat infections and prevent fluid build-up. Sometimes surgery (called myringostomy) is necessary to drain excessive fluid and prevent future infections (fluid attracts bacteria that can result in an infection called otitis media). Some children also require tonsil and/ or adenoid surgery if they are experiencing severe and frequent problems.

Hearing tests should be done regularly to check for hearing impairments. Fluid collection in the middle ear space can prevent sounds from reaching the inner ear, resulting in hearing loss. Hearing problems can delay speech and some children require speech therapy when they begin talking. Speech therapy is also helpful for children, whose speech is affected by muscle problems in the palate because their clefting prevents them from making certain sounds. Following their repair surgery, most children quickly acquire these speech and language skills at a normal pace. However, a small percentage of children (5-15%) may be unable to seal off the nose during speech, even after their cleft has been repaired. This results in "nasal" sounding speech and may require corrective surgery. The procedure is called pharyngeal flap and is usually performed at age 4 or 5. It involves lengthening the palate or attaching tissue from the back of the throat to the palate. Surgery will only be performed following a speech video X-ray study and/ or an examination of the palate with a small fiber optic scope.

It is recommended that your child's speech be checked every 6 months until the age of 3, and on a yearly basis thereafter.

Risk factors:

Factors in the infant that may increase the risk of oral-facial clefts include: having other birth defects; being male; or having a sibling, parent or other close relative born with an oral-facial cleft.

There is a higher risk of cleft lip or palate if a mother takes certain drugs during pregnancy (such as anticonvulsant medications or drugs used for dermatological conditions, such as acne). Heavy drinking during pregnancy has been associated with a higher incidence of orofacial clefts (especially in the development of a cleft lip). Smoking causes 1 in 5 oral clefts. Having certain infections during pregnancy, being diabetic and not getting enough folic acid before pregnancy may increase the chances of your baby being born with certain birth defects, including oral clefts.

Prevention:

Pregnant women and women who are likely to become pregnant can reduce the risk of their unborn child developing oral clefts by:

Taking 400 micrograms of folic acid daily
Eeating foods that are high in folic acid, such as green, leafy vegetables; fruits and orange juice; dried beans and peas; and pasta, rice, bread, flour and cereals.
Not smoking or drinking during pregnancy
Getting early and regular prenatal care
Informing their doctor of any medications they are taking, to ensure they are safe to use during pregnancy
Consulting a genetic counselor to define the actual risk, which can vary greatly among families


Prognosis:

Both conditions are often very upsetting for parents but can be successfully repaired using plastic surgery. Surgery generally produces excellent results and most babies heal without any problems. It is normal for the lips, nose and face to be swollen for several days post-operatively. The scar may be red for several weeks. It will take 6-12 months for the scar to soften and fade. Surgical repair not only improves the appearance of a child's face, but may also have a positive impact on their breathing, hearing, speech and language. How your child heals depends on the severity of the oral cleft. Some children require further surgery to fix the scar from the initial surgical repair. Continued follow-up may be needed with speech therapists, an otolaryngologist (ear, nose and throat specialist) and orthodontist to manage any speech, dental or hearing problems associated with the condition. With treatment, most children with orofacial clefts do well and lead a healthy life. Although the scar will never completely disappear, it will become more difficult to see over time. With surgical intervention, your child's lip and nose will be nearly normal in appearance and function.

References:

A.D.A.M., Inc. The New York Times: Health Guide. Cleft Lip and Palate. The New York Times. http://health.nytimes.com/health/guides/disease/cleft-lip-and-palate/overview.html. Reviewed January 5, 2011. Accessed February 15, 2013.

Alan R. Oral-facial clefts. NYU Lagone Medical Centre. http://pediatrics.med.nyu.edu/conditions-we-treat/conditions/oral-facial-clefts. Reviewed September 2012. Accessed February 14, 2013.

FACES. Craniofacial Disorders: Cleft lip and Cleft palate. The national Craniofacial Foundation. http://www.faces-cranio.org/. Reviewed February 10, 2011. Accessed February 18, 2013.

March of Dimes Foundation. Birth defects: Cleft lip and cleft palate. March of Dimes. http://www.marchofdimes.com/baby/birthdefects.html. Accessed February 13, 2013.