Cleft lips

What is a cleft lip?

A cleft lip is a gap or split in the upper lip that's present from birth, and it often occurs alongside a cleft palate, a similar gap in the roof of the mouth. It happens when the tissues forming the upper lip fail to join properly during the first few weeks of pregnancy.

Symptoms of a cleft lip

A cleft lip is visible straight away, ranging from a small notch in the upper lip to a wide gap that reaches up to the nose. It can affect one side of the lip, or both (called a bilateral cleft), and can sometimes extend through the gum as well, affecting the teeth.

A cleft palate affects the roof of the mouth, and can range from a small opening at the back (affecting the soft palate) to a split reaching all the way to the front of the mouth (the hard palate). Sometimes it's hidden underneath the lining of the mouth and isn't obvious to see.

A cleft lip on its own doesn't usually cause feeding or speech problems. A cleft palate is more likely to, because a baby needs an intact palate to form a seal and suck properly, so difficulty feeding is often the first sign that the palate is affected. Left untreated, a cleft can also lead to:

• ear infections and a build-up of fluid in the ears (glue ear), which can affect hearing
• dental problems, as teeth may not develop correctly
• speech problems, such as unclear or nasal-sounding speech.

Causes and who is at risk

In most cases, it isn't clear exactly why a cleft happens, and it's very unlikely to be caused by anything a mother did or didn't do during pregnancy. Experts generally agree the causes are multi-factorial, meaning a combination of genetic and environmental factors probably play a part, including:

• a family history of clefts, though most cases happen out of the blue, with no family history at all
• smoking or drinking alcohol during pregnancy
• being obese during pregnancy
• not having enough folic acid during pregnancy
• having diabetes before pregnancy
• taking certain medicines in early pregnancy, such as some anti-seizure medicines
• certain infections, such as rubella (German measles).

Being a teenager or being over 35 during pregnancy is also linked to a higher risk. Cleft lip is somewhat more common in boys, while cleft palate on its own is somewhat more common in girls. Incidence also varies by ethnicity: it's thought to be highest in people of Asian descent (around 1 in 500 births), around 1 in 700 in Caucasian people, and lowest in people of African descent (around 1 in 1,200 births).

Occasionally, a cleft lip or palate occurs as part of a wider genetic condition, such as 22q11 deletion syndrome (also called DiGeorge syndrome) or Pierre Robin sequence.

Are there different types of cleft lip?

Clefts vary widely from person to person. They're generally described by:

• what's affected: the lip only, the palate only, or both together
• how far they extend: an incomplete cleft is a small notch, while a complete cleft reaches further, up to the nose for a cleft lip, or through to the front of the mouth for a cleft palate
• which side: a unilateral cleft affects one side, a bilateral cleft affects both sides
• visibility: a submucous cleft palate is hidden underneath the lining of the roof of the mouth, and may not be picked up straight away.

How is a cleft lip diagnosed?

A cleft lip is often picked up during the 20-week anomaly scan in pregnancy (usually done between 18 and 21 weeks), though not every cleft lip will be obvious on the scan. A cleft palate is much harder to detect on an ultrasound scan, even when a cleft lip is also present, so it's often not identified until after birth.

If a cleft wasn't picked up during pregnancy, it's usually spotted straight after birth, or during the newborn physical examination carried out in the first 72 hours. Once diagnosed, your baby will usually be seen by a specialist cleft nurse within 24 hours, and referred to a specialist multidisciplinary cleft team.

How is a cleft lip treated?

Non-surgical support

Alongside surgery, your child's cleft team will support you with the following:

• Feeding: This includes advice on positioning your baby at the breast, or feeding with a special type of bottle designed for babies with a cleft
• Hearing: This will be regularly monitored, since a cleft palate makes glue ear more likely. A hearing aid or small tubes called grommets may be used if hearing is significantly affected
• Speech and language therapy: This is to support your child's speech development throughout childhood
• Dental care and orthodontic treatment: This includes braces if their adult teeth don't come through properly.

Surgical treatment

Surgery is the main treatment, carried out by a specialist multidisciplinary team that includes a surgeon, cleft nurse, orthodontist, ENT surgeon, paediatric dentist, speech and language therapist, psychologist and geneticist.

• Cleft lip repair: This is usually done at around 3 months old. It's carried out under general anaesthetic and takes around one and a half hours. The surgeon rearranges the skin and muscle of the lip, using local tissue to close the gap
• Cleft palate repair: This usually happens between 6 months and 1 year old, also under general anaesthetic and taking around 1 and a half hours. The lining and muscles of the palate are rearranged to close the gap, usually without needing extra tissue
• If the cleft also affects the gum: A bone graft operation is usually carried out later, between the ages of 9 and 12.

Recovery is generally quick. Babies who've had a cleft lip repair can often go home within a day or two, while those who've had a cleft palate repair usually stay in hospital for several days. Stitches from a cleft lip repair are removed a few days later, while stitches from a cleft palate repair dissolve on their own.

Some children need further surgery later in childhood or adolescence, for example to improve a scar, the shape of the nose (rhinoplasty), or the jaw (maxillary osteotomy, usually after the age of 17, if it hasn't grown forward normally). Follow-up appointments usually continue until around the age of 20.

When should I see a doctor?

If you're pregnant and a 20-week scan suggests your baby may have a cleft lip, you'll be referred to a specialist cleft team before the birth, so you can ask questions and prepare. If a cleft isn't picked up until after birth, it's usually found during the newborn physical examination in the first 72 hours, and you'll be referred to a specialist team straight away.

Contact your health visitor, GP or cleft team promptly if your baby is struggling to feed, isn't gaining weight as expected, or you notice signs of an ear infection, such as a temperature, pulling at their ear, or being unusually unsettled. Feeding difficulties in particular need swift support, since they can affect a baby's weight gain if not managed early.