Cleft Lip and Palate

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Cleft lip and/or palate\: gap in lip/palate due to failure of normal development; ranges from unilateral cleft lip or palate to
bilateral cleft lip and palate.
Prevalence\: 1 in 700 UK babies; ~1200 born yearly with cleft; 31% cleft lip and palate, 45% isolated cleft palate, 24% isolated
cleft lip, 8% bilateral cleft lip and palate.
Development\: cleft lip forms due to failure of nasal/maxillary prominences fusion (weeks 5-6); cleft palate forms due to
failure of primary (hard palate) or secondary (hard/soft palate) palate fusion (weeks 5-12).
Risk factors\: family history, smoking/alcohol during pregnancy, obesity, low folic acid, epilepsy medications (topiramate,
sodium valproate); ~15% due to genetic syndromes (e.g. DiGeorge, Pierre-Robin sequence, Treacher-Collins).
Diagnosis\: anomaly ultrasound (weeks 18-21) or newborn physical examination (within 72 hours of birth); referral to
specialist cleft team and seen by cleft nurse within 24 hours.
Complications\: airway problems (e.g. Pierre-Robin Sequence), feeding di
psychosocial impact, dental issues, speech problems.
Initial management\: multidisciplinary approach (airway, feeding, hearing tests, psychological support, dental advice,
counselling, genetic counselling); cleft nurse visit within 24-48 hours.
Cleft lip repair\: surgery at ≥3 months old, 1-2 hours under GA, overnight hospital stay; complications\: anaesthetic risks,
bleeding, infection, airway obstruction, scarring, wound issues.
Cleft palate repair\: palatoplasty at 6-12 months old to aid speech/language development; aim to correct defect and
reattach soft palate muscles for normal velopharyngeal function.
Article 🔍
A comprehensive topic overview

Introduction

A cleft forms when the failure of normal lip and/or palate development leaves a gap or channel in the lip and/or palate.
The severity can range from an isolated unilateral cleft lip or palate to having a bilateral cleft lip and palate.
1
A cleft lip and/or palate a
means that around 1200 babies are born every year with a cleft in the UK.
Of these babies, 31% will have a cleft lip and palate, 45% will have an isolated cleft palate and 24% will have an isolated
cleft lip. Only 8% of babies born with a cleft in the UK have a bilateral cleft lip and palate.
1
This article will provide information on cleft lip and palate, how they form, as well as the risk factors and common genetic
syndromes associated with a cleft. It will also provide an overview of treatment and what the typical long-term
management pathway for a patient with a cleft may look like.

Aetiology

A patient may have an isolated cleft lip or palate, or a combination of both.Figure 1. Diagram showing how a
unilateral and bilateral cleft lip and palate
may look.
3
A cleft lip forms when the nasal prominences and maxillary prominences fail to properly fuse and create the lip during
weeks 5 to 6 of embryological development. This creates a unilateral or bilateral cleft lip depending on if the failure of
fusion occurs on one side or both.
2
The severity of the cleft can range from a little notch in the lip to complete separation of the lip and gum running up to
the nose.
1
The primary and secondary palates then develop during weeks 5 to 12\:
2,3
The primary palate consists of a triangular area of the hard palate anterior to the incisive foramen and a portion of the
alveolar ridge. It is created by the medial nasal prominences fusing at the midline.
The secondary palate consists of the remaining hard palate and the soft palate. It is created by the lateral palatine
processes fusing at the midline from anterior to posterior.
Figure 2. A diagram showing an inferior view of the bony palate.
4Figure 3. A diagram showing the anatomy and landmarks of the hard and soft palate.
5
A cleft palate forms when either of these processes fails and can result in a unilateral or bilateral cleft. A partial palatal
cleft only a
6
Cleft palates may also be submucous meaning that the overlying mucosa is intact while the underlying palate muscles
have failed to fuse at the midline.
3 7

Risk factors

The cause of clefts among most babies is unknown. It is widely agreed that clefts usually arise from a combination of
genetic and environmental factors during pregnancy. They may also arise due to a genetic syndrome.
1
Risk factors which increase the risk of a baby being born with a cleft include\:
8
Family history of cleft
Smoking or alcohol use during pregnancy
Obesity during pregnancy
Low folic acid during pregnancy
Taking epilepsy medications such as topiramate or sodium valproate during the
9
Around 15% of clefts arise secondary to a genetic syndrome, where a genetic mutation results in a wide range of birth
defects. 10
Some of these conditions include 22q11.2 microdeletion syndrome (DiGeorge), Pierre-Robin sequence and
Treacher-Collins syndrome.
7,8

Clinical features

A cleft lip is usually diagnosed during the anomaly ultrasound scan during weeks 18-21 of pregnancy.
If it is not diagnosed then, it should be picked up immediately after birth or during the newborn physical examination
(NIPE) within 72 hours of birth. This examination includes direct visualisation of the hard and soft palate using a torch and a
tongue depressor.
11
When a cleft lip or palate is diagnosed, the family is immediately referred to a specialist multidisciplinary cleft team and
seen by a cleft specialist nurse within 24 hours.
8
An untreated cleft lip or palate can cause several problems\:
8Airway problems\: especially in children with Pierre-Robin Sequence, which presents with a triad of a small jaw
(micrognathia), a
associated with a cleft palate in 90% of cases. Such babies may need to be positioned on their side or front to allow the
tongue to fall forwards and prevent it from obstructing the airway. Further treatment options include the use of a
nasopharyngeal airway, a tongue-lip adhesion surgery, or a tracheostomy.
Feeding problems\: a baby with a cleft lip or palate may struggle to create a good seal around the nipple or teat of a
normal baby bottle. This may result in fatigue, poor weight gain, excessive air intake, choking and nasal regurgitation. A
special feeding bottle and advice from the cleft specialist nurse are essential.
Glue ear and associated conductive hearing loss\: as many as 90% of patients with an unrepaired cleft palate have glue
ear secondary to eustachian tube dysfunction.
7
Psychosocial impact\: a cleft may lead to low self-con
bullying or discrimination.
Dental problems\: an untreated cleft can mean a child’s teeth do not develop correctly resulting in misaligned teeth and
future dental problems.
Speech problems\: a cleft palate can lead to a hypernasal voice and unclear speech as the child grows up.

Management

Initial management
A specialist cleft nurse will visit within 24-48 hours of birth to provide information and support. The
diagnosis involve giving the family support and information on the diagnosis and ensuring that the baby grows adequately
to allow future surgery.
Initial management requires a multidisciplinary approach\:
1
Airway assessment and management
Specialist feeding assessment\: advice and training may be given on the use of special feeding bottles and teats if the
baby is having trouble feeding. Some babies require the placement of a nasogastric tube and anti-re
Newborn hearing test\: children with a cleft palate will be regularly reviewed by specialist cleft ENT and audiology
teams.
Psychological support\: from a counsellor, specialist nurse or psychologist.
Dental health education and advice
Counselling the parents on future surgical options
Monitoring for signs and symptoms of genetic syndromes associated with a cleft
Genetic counselling\: this may provide the parents with information about what has caused the cleft and the likelihood of
it happening again with future children.
Cleft lip repair
Initial cleft lip surgery is usually performed when the child is at least three months old.
This typically takes 1 to 2 hours to complete under general anaesthetic. Patients are typically required to stay in hospital
overnight and can usually return home once they are drinking adequately and are comfortable.
1
Potential complications of cleft lip repair may include\:
12
Anaesthetic risks
Post-operative bleeding
Infection
Airway obstruction
Damage to deeper structures
Hypertrophic or keloid scar
Wound dehiscence
Notching of the lip vermilion (the red part of the lip)The Millard technique
One commonly used surgical technique to repair a unilateral cleft lip is the rotation-advancement technique, which
is also known as the Millard technique.
This technique involves making several incisions in the lip to create a rotational
cleft and an advancement
sutured to correct the cleft in the muscle, subcutaneous tissue and skin.
13
Figure 4. Diagram showing the incision lines usually made during a Millard technique cheiloplasty.
14
Figure 5. Diagram showing the
15
Cleft palate repair
Palatoplasty to repair a cleft palate usually takes place when the child is 6-12 months old so the child’s cleft palate will not
prevent adequate speech and language development.
1
The aim is to correct the palatal defect and reattach the soft palate muscles to create a normally functioning
velopharyngeal mechanism.
2The velopharyngeal mechanism
The velopharyngeal mechanism functions to separate the oral and nasal cavities during swallowing and speech.
The velopharyngeal port is the passage between the nasopharynx and oropharynx. The velopharyngeal mechanism
consists of a muscular valve that extends from the back of the hard palate to the posterior pharyngeal wall and
includes the soft palate (the velum) and the sides of the throat (lateral pharyngeal walls).
Several di
a tight seal between the velum and pharyngeal walls, thus preventing communication between the oral and nasal
cavities.
16
Figure 6. A diagram of the anatomy
associated with the velopharyngeal
mechanism. The area highlighted blue is
where the soft palate is elevated during
the velopharyngeal mechanism to close
o
17
There are
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Tensor veli palatini
Levator veli palatini
Palatoglossus
Palatopharyngeus
Musculus uvulae
The most important muscle to reconstruct is the levator veli palatini as it elevates the soft palate during swallowing and
speech. It is therefore essential for the velopharyngeal mechanism. Failure to reattach this muscle properly can lead to
velopharyngeal insu
2
Potential complications of cleft palate repair may include\:
19
Anaesthetic risks
Post-operative bleeding
Infection
Airway obstruction
Damage to deeper structures
Wound dehiscence
Total or partial
Oronasal
Hanging palate
Poor speech outcomes and the need for further speech surgery
Ongoing management
After the initial repair of their cleft lip and/or palate, patients will require regular follow-up until they are around 20 years
of age. This may include\:
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Regular audiology assessment of children with cleft palate until around 5 years of age
Speech and language therapy
Ongoing psychological supportPaediatric dentistry advice and treatment
Orthodontic assessment and treatment if required at around 10 years old
Monitoring of previous surgical sites for complications and assessing whether further surgery is necessary
Further surgery
Further surgery may also be required, which may include\:
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Palatal
Speech surgery (e.g. palatal lengthening)
Alveolar bone graft
Lip and/or nasal revision surgery (cleft rhinoplasty)
Orthognathic surgery
Restorative dental surgery
Palatal
Following a palatoplasty, the tissues may heal in such a way that an oronasal
cause nasal regurgitation during swallowing and speech problems, and so is often closed in a palate revision surgery
(secondary palatoplasty).
22
Speech surgery
Speech surgery may be o
insu
Such speech operations include palatal lengthening with a double-opposing Z-palatoplasty (Furlow’s) or a posterior
pharyngeal
palate, can be used to block some of the air leaking from the nose.
22
Alveolar bone graft (ABG)
An alveolar bone graft is performed when there is insu
teeth to erupt properly.
Bone marrow (cancellous bone) is taken from the iliac crest and grafted into the cleft defect to improve the bone support
for the adult teeth and allow the orthodontist to align the teeth in the cleft area.
This procedure is usually completed at around 9-11 years old, once all the baby teeth have been lost, but before the child’s
lateral incisors or upper canines have erupted through the gum.
22
Cleft rhinoplasty
Revision surgery can be completed at any age to improve the contour or shape of the lip and/or nose. However, it is often
suggested to delay revision surgery until the patient has
need for extra revision surgeries.
22
Orthognathic surgery
Orthognathic surgery involves re-aligning the upper and lower jaws to improve the function of the patient’s bite and side
pro
performed by a maxillofacial surgeon in conjunction with an orthodontist.
21
Jaw surgery is completed after growth stops, which is usually around the age of 14-16 years in girls and 17-21 years in
boys.
23
Restorative dental surgery
A restorative dentist may replace missing teeth with
to reshape the teeth to improve how they look. They can also treat any complex gum or tooth root issues.
This treatment can often not be completed until all the patient’s orthodontic treatment has been completed and all their
adult teeth have erupted.
24

References

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Related notes

r e p a i r \: a m u l t i v a r i a t e s t a t i s t i c a l a n a l y s i s o f 7 0 9 p a t i e n t s .Published in September 2014. Available from [LINK].
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Autism Spectrum Disorder (ASD)
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Contents

Mr David Sainsbury
Introduction
Consultant Cleft and Plastic Surgeon
Aetiology
Risk factors
Clinical features
ManagementSource\: geekymedics.com