Cleft lip and cleft palate

The face closes up early in pregnancy — the lip during the second month, the palate a little later. When that closing never finishes, a baby is born with a gap in the lip, in the roof of the mouth, or in both. It is one of the commonest things a child can be born with, and one of the very few that surgery can put right almost entirely: by school age all that is usually left is a thin line above the lip. Older nicknames for it still turn up in print, but they explain nothing and they sting, so they are best dropped. What follows is what you can see in the first hours, what should get a doctor out of bed, and how the road from delivery room to the teenage years is laid out.

What you can see at birth

A gap in the lip is obvious straight away. It may be on one side or on both, and it ranges from a shallow notch in the red border to a wide split running up to the base of the nose. The nostril on that side is usually pulled out of shape along with the lip, so the nose looks lopsided.

A gap in the palate is much harder to spot — you have to look inside an open mouth in good light. It may involve only the soft palate near the throat, or it may run forward as far as the gum. The lip is often perfectly formed, and the reverse is true too: a split lip does not mean the palate is involved.

There is a third version, the submucous cleft: the muscles of the palate are separated but the lining over them is intact, so the mouth looks ordinary from outside. The clues are a split uvula, a pale translucent stripe down the midline, and a notch you can feel at the back edge of the hard palate. This kind is often found only at two or three years old, when speech starts to sound nasal and drinks come back down the nose.

When it cannot wait until morning

The gap itself is not dangerous, but in some babies it comes with a small lower jaw and a tongue that falls backwards, and then the airway closes off. That is sorted out on day one, not at a booked appointment. Call emergency services or fetch the on-call team if your baby has:

• noisy or snoring breathing in, with the skin sucking in between the ribs and below the breastbone;
• a blue tinge round the mouth or on the tongue, or a grey, washed-out colour;
• breathing that is clearly easier on the front or on the side and clearly worse on the back;
• choking or going blue during a feed, with milk coming out of the nose;
• floppiness, a weak cry, or a baby who does not wake for feeds.

Weight deserves separate attention. A baby with a cleft palate can suck long and hard and still take in very little. Passing urine fewer than six times a day, no weight gain over a week, or feeds that drag past forty minutes are reasons to be seen the same day. After surgery, treat bleeding from the mouth, a wound that has opened, refusal to drink and a fever above 38 °C as urgent.

What makes the first months hard

Feeding is the main hurdle, and it comes from the palate rather than the lip. Sucking needs a sealed mouth and a vacuum; with a gap in the palate the air escapes upwards, the baby swallows air instead of milk, tires quickly and brings feeds back. A more upright hold, soft squeezable bottles with a valve made for this, expressed milk and frequent pauses to burp all help. Occasionally a feeding tube is needed until the operation.

Ears are the second front. A cleft palate disturbs the muscles that open the Eustachian tube, so fluid sits in the middle ear in nearly all of these children. It rarely hurts — the child simply hears less well, and so picks up speech more slowly. Hearing is checked regularly, and if the loss persists a tiny drainage tube is placed in the eardrum.

Teeth and speech are the third. Where the gap crosses the gum, the neighbouring teeth come through crooked, one may be missing and another may be extra, and decay sets in more easily, so brushing and dental visits start earlier than usual. With the palate closed in good time, speech usually develops normally, but some children keep a nasal tone. A speech and language therapist works on that, and a small number need a further operation on the palate.

How it is picked up

A cleft lip is usually seen at the second-trimester scan, between 18 and 21 weeks. The palate is almost impossible to assess on a scan, so an isolated cleft palate goes unnoticed before birth — that is the limit of the method, not an oversight by the sonographer.

If nothing was found in pregnancy, the diagnosis is made at the first newborn examination, where the palate is looked at and not just the lip. A submucous cleft is easy to miss even then, which is why a nasal-sounding three-year-old, drinks escaping through the nose, or repeated ear infections are good reasons to ask for the palate to be examined specifically.

Why the gap stayed open

In most families no cause can be named, and that is worth saying plainly: the lip and palate form at a stage when the pregnancy is often not yet known about, and it is almost never possible to trace what happened to anything the mother did or failed to do. Only a handful of circumstances are known to raise the odds a little:

• clefts in close relatives, though most often the case is the only one in the family;
• smoking and alcohol in the first trimester;
• marked excess weight and diabetes in the mother;
• too little folic acid before conception and in the earliest weeks;
• a few medicines taken early on, including some anticonvulsants and vitamin A derivatives.

Some clefts, especially isolated clefts of the palate, are part of a syndrome. The ones seen most often are 22q11.2 deletion (also called DiGeorge or velocardiofacial syndrome) and Pierre Robin sequence. That is why the first weeks involve more than a look in the mouth: the heart, the hearing and the growth curve are all checked, and genetic testing is arranged if there is a reason for it.

What happens next: a plan measured in years

A team looks after this — a maxillofacial surgeon, a paediatrician, an ear-nose-and-throat doctor, a speech therapist, an orthodontist and a dentist. There is no single operation that settles everything, but the treatment is not endless either: the main work falls in the first year.

• First weeks: help with feeding, weight checks, a hearing screen and a general examination.
• Around 3–6 months: repair of the lip, under general anaesthetic, one to two hours in the operating room and a day or two in hospital.
• Around 6–12 months: repair of the palate, closing the gap and rebuilding the muscle sling. The scar is inside the mouth. Getting it done before speech takes off matters more than doing it as early as possible.
• From eighteen months to two years: regular sessions with the speech therapist.
• Roughly 8–12 years: a bone graft to the gum so that the adult teeth can come through in place.
• The teenage years: braces, monitoring of jaw growth and, if wanted, refinement of the nose or the bite once growing has stopped.

One detail worth knowing in advance: in a child with a cleft palate, including a submucous one, the adenoids are removed only with great caution and not on the usual grounds. That tissue helps such a child separate mouth from nose, and taking it out can leave the speech sounding nasal. If an operation is suggested, say that your child has a cleft.

Will the next baby have it too?

When the cleft is isolated and is the only case in the family, the chance of it happening again is low — it is put at roughly 2–8%. A similar figure applies to the child of a parent who was born with a cleft. The numbers change when the cleft is part of a syndrome: an adult with a 22q11.2 deletion passes it on to each child with a one-in-two chance. That is why a family planning more children gains from a genetics appointment — a single visit replaces years of guesswork.

Online consultation

Everything that does not need hands works well remotely: how to tell whether the baby is getting enough, which bottle to try, what a gap seen on a scan actually means and what to ask the surgeon before the birth. It is also useful after discharge — for the timing of surgery, for what the ear tubes are for, for whether a nasal-sounding three-year-old is a worry, and for where to start if the cleft has only just been suspected. The breathing and weight-loss signs listed above cannot be judged through a screen: for those, go in person or call emergency services.

This material is for information only and does not replace medical advice.