Cleft Lip and Palate: Types, Causes, Risks & Treatment

What is Cleft Lip and Palate?

Orofacial clefts are one of the most common head and neck birth defects that affect roughly 1 in 700 children worldwide. Oral clefts appear as splits or openings in the upper lip, palate (roof of the mouth), or both. In addition, the splits cause gaps and defects in the affected areas. They are also usually associated with a nose deformity on the same side.

Half of all clefts involve both the lip and palate, while clefts only affecting the palate are less common.

Serious medical and dental conditions typically occur in children who have cleft lips and/or palates, including:

  • Dental abnormalities, such as missing or misaligned teeth.
  • Greater risk for cavities, due to weaker enamel and thicker saliva production.
  • Hearing problems.
  • Language and speech disorders.
  • Abnormalities affecting the middle ear.
  • Psychosocial issues (social and psychological aspects).

Cleft Lip

Cleft lips can either be complete or incomplete, which means the splits may or may not extend to the nose. They can also develop on one side (unilateral) or on both sides (bilateral) of the upper lip. There are four classifications of cleft lips:

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Incomplete Unilateral

Clefts that develop on one side of the upper lip and do not extend into the nose.

complete unilateral cleft lip
Complete Unilateral

Clefts that develop on one side of the upper lip and extend into the nose.

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Incomplete Bilateral

Clefts that appear on both sides of the upper lip, but do not extend into the nose.

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Complete Bilateral

Clefts that appear on both sides of the upper lip and extend into the nose.

Cleft Palate

Similar to cleft lips, cleft palates can either be complete or incomplete, which means the splits may involve the primary (front) palate, secondary (back) palate, or both. There are four classifications of cleft palates:

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Incomplete Cleft Palate

Clefts that develop in the soft palate (back of the mouth).

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Complete Cleft Palate

Clefts that develop in the soft and hard palate on one side of the mouth. The splits extend past the lips and into the nose.

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Unilateral Complete Cleft Palate and Lip

Clefts that develop in the soft and hard palate on one side of the mouth. The splits extend past the lips and into the nose.

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Bilateral Complete Cleft Palate and Lip

Clefts that develop in the soft and hard palate on both sides of the mouth. The splits extend past the lips and into the nose.

Causes

Clefts are birth defects, which means they form in the womb. Common reasons a child may develop the birth defect include:

  • Family history of cleft lip and palate (genetics).
  • Having diabetes during pregnancy is linked to birth defects, such as cleft lips.
  • Obesity during pregnancy can increase the risk of cleft lip and palate.
  • Exposure to unhealthy substances during pregnancy, such as cigarettes, alcohol, and some medications.

Abnormalities Associated with Cleft Lip and Palate

Common abnormalities and syndromes associated with cleft lip and palate include:

  • Van der Woude syndrome — affects face development.
  • CHARGE syndrome — causes coloboma, heart defects, atresia choanae, and ear abnormalities.
  • Apert syndrome — causes abnormal skull growth and affects the shape of the face and head.
  • Pierre Robin sequence — a birth defect that results in a smaller jaw, breathing difficulties, and a tongue that falls back in the throat.
  • Kabuki syndrome — a congenital disorder that causes abnormal facial features.
  • Klippel Feil syndrome — causes neck and spine abnormalities and results in a shorter neck.
  • Stickler syndrome - causes eye abnormalities, hearing loss, joint problems, and a flattened facial appearance.
  • Treacher-Collins syndrome — causes ears, eyes, cheekbones, and chin deformities.
  • DiGeorge syndrome — a chromosomal disorder that can cause poor heart development, issues with the immune system, and a cleft palate.
  • Beckwith-Wiedemann syndrome — children with this syndrome have an abnormally large tongue, which results in breathing, swallowing, and speaking difficulties.
  • Goldenhar syndrome — a birth defect that causes poor development of the nose, soft palate, lips, mouth, and ears.

Phases of Treatment

Children who are born with a cleft lip, palate, or both require multiple phases of treatment starting from birth and into early adulthood (18+ years old). Treatment typically includes adhesions, repairs, surgeries, bone grafts, and orthodontic treatment.

Cleft lip and palate repairs are performed by a plastic surgeon, pediatric otolaryngologist, or an oral and maxillofacial surgeon with extensive training in pediatric craniomaxillofacial surgery. In addition, there are a few phases of treatment throughout childhood, depending on the child’s needs:

Birth — Infant Orthopedic Treatment, Lip Repair, and Lip Adhesion

From birth, children with a cleft lip and palate require pre-surgical infant orthopedics (PSIO). In particular, this procedure reshapes nasal and alveolar segments before cleft lip surgery. A cleft lip is repaired between 3 and 6 months of age. A nasolabial (lip) adhesion may also be necessary before repairing the cleft lip to reduce tension and speed up the recovery process.

1 Year — Palate Repair

Cleft palates are repaired between 9 and 18 months of age, typically around age 1. Early palate repair allows for better speech development. Although, repairing the defect early creates more scar tissue and may lead to improper growth of the mouth, improper bite, and poor facial esthetics. Orthodontic treatment is typically necessary later on to correct these issues. If palate repair is completed after 18 months of age, speech may be negatively impacted.

2-3 Years — Velopharyngeal Surgery

Velopharyngeal insufficiency (VPI) occurs when the soft palate does not close tightly against the back of the mouth, which leads to abnormal speech and/or hypernasality. Velopharyngeal surgery is completed around 3 years of age if a child’s speech does not improve.

5-9 Years — Alveolar Bone Grafts and Maxillary Expansion

After a cleft lip and palate repair, a surgeon performs alveolar bone graft surgery a few years later. In short, the surgery adds bone to the gum ridge in children born with clefts. In addition, the bone graft must be completed as a child begins to lose their baby teeth and before permanent teeth grow in.

11 Years — Orthodontic Treatment (Phase I)

As baby teeth fall out and permanent teeth grow in (mixed dentition phase), the first round of orthodontic treatment takes place. Orthodontic treatment aligns the roof of the mouth, fixes crossbites, creates space for erupting permanent teeth, and treats impacted teeth.

13-15 Years — Orthodontic Treatment (Phase II)

By age 13, all permanent teeth have erupted. An additional phase of orthodontic treatment may also be necessary to align teeth and fix any dental abnormalities.

18+ Years — Orthognathic Surgery and Final Restorative Care

Orthognathic surgery is an operation that corrects the alignment of the jaws. It may be necessary after a child’s cleft lip and palate repair, bone graft surgery, and orthodontic treatment.

Resources

Ahlin, Jeffrey H. Maxillofacial Orthopedics: a Clin. Approach for the Growing Child. Quintessence Publ., 2003.

“Cleft Lip and Palate.” CHLA, 9 July 2019, www.chla.org/cleft-lip-and-palate.

Nowak, Arthur J. Pediatric Dentistry: Infancy through Adolescence. Elsevier, 2019.

Updated on: May 28, 2020
Author
Alyssa Hill
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Medically Reviewed: October 11, 2019
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Lara Coseo
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