Monday, July 2, 2018

Cleft Lip and Palate



What is cleft lip and palate:

Cleft lip and palate are birth defects of the mouth and lip, also known as oral-facial clefts. A normal fetus has a split lip and palate, but early in pregnancy, the sides of the lip and the roof of the mouth should fuse. Failure of fusion results in cleft lip and/or cleft palate.
·         About 6,000 to 8,000 kids are born in the United States with oral-facial clefts every year
·         Cleft defects are more common in boys
·         Cleft defects are more common in Asians and certain groups of American Indians and are less common in African-Americans
·         Less than 13 percent of patients have other birth defects
What are the different types of cleft lip and palate?

Unilateral defects – the most common type of cleft, affecting one side of the lip, with or without a cleft palate
·         Incomplete unilateral cleft lip – a notching of the lip that does not extend into the nose
·         Incomplete unilateral cleft lip and palate – a notching of the lip that does not extend into the nose, accompanied by a cleft palate
·         Complete unilateral cleft lip and palate – an opening that extends from one side of the lip into the nose and palate.
Bilateral defects – affecting both sides of the lip and/or palate, forming two clefts
·         Incomplete bilateral cleft lip and palate – a notching of both sides of the lip that does not extend into the nose
·         Complete bilateral cleft lip and palate – an opening that extends from both sides of the lip into both nostrils.
Children can also have less noticeable form of cleft lip than those depicted above, known as a form-fruste cleft lip. A form-fruste cleft lip is a small, subtle indentation of the lip.
Causes:

The cause of cleft lip and palate is not completely understood, though we know that a combination of genetic and environmental factors contribute to cleft lip and palate. If parents without clefts have a baby with a cleft, the chance they will have another baby with a cleft ranges from 2 percent to 8 percent, which suggests a genetic factor. If clefts run in your family, genetic consultation is recommended.
In addition, there are several syndromes commonly associated with cleft lip and palate:
·         Van der Woude syndrome
·         Stickler syndrome
·         Pierre Robin sequence or syndrome
·         Hemifacial microsomia
·         Goldenhar syndrome
·         Downs syndrome
·         CHARGE syndrome
·         22q and velocardiofacial syndrome
·         DiGeorge syndrome
·         Oro-mandibular limb hypoplasia syndrome
·         Opitz syndrome

Treatment:

At The Children’s Hospital of Philadelphia, children with cleft lip and cleft palate receive coordinated care through our Cleft Lip and Palate Program. The multidisciplinary program brings together plastic surgeons, pediatricians, orthodontists and other specialists in order to provide the most comprehensive care for your child.
The surgical procedures required for each patient with cleft lip and palate will vary depending upon the type and severity of the deformity. Timing and treatment will be adjusted based on each patient’s overall medical needs, but treatment typically includes a combination of the following procedures and stages, performed within general time frames based on development.
·         Nasoalveolar molding (NAM) (1 week to 3 months of age, if needed)
·         Cleft lip repair (3 to 6 months)
·         Cleft palate repair (9 to 18 months)
·         Palatal expansion (5 to 7 years, if needed)
·         Alveolar bone graft and fistula repair (6 to 9 years)
·         Tip rhinoplasty (6 to 9 years)
·         Phase I orthodontics (6 to 9 years)
·         Phase II orthodontics (14 to 18 years)
·         Orthognathic surgery (jaw surgery) (14 to 18 years)
·         Final touchup surgery (adolescence or adulthood)





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