Cleft Lip is caused because of failure of fusion of normal upper lip elements. This leads to a break in the upper lip. A cleft lip opening can present in any of the following ways:
- Small slit
- A large defect that extends into the mouth
- A defect that extends into the palate called cleft palate
I have seen many cases of cleft lip and have provided anesthesia for these babies. This defect is so common that it is worth jumping in to find out more:
By Dr. Rajeev Iyer, MBBS, MD, FASA, Board Certified Pediatric Anesthesiologist
What is cleft lip?
A cleft is a general defect in the body when the normal elements fail to form. A cleft lip is a defect in the upper lip. The cleft lip can be broadly classified as the following:
- Non-Syndromic – where there is isolated cleft lip
- Syndromic – where the cleft lip is associated with a syndrome
A syndrome is when a particular baby has a constellation of symptoms across many organ systems.
A review article says that most cleft lips are nonsyndromic (approximately 70%) and 30% are syndromic. Here are some examples of syndromes commonly associated with cleft lip:
- Apert syndrome
- Stickler syndrome
- Treacher-Collins syndrome
- Van der Woude syndrome
- Waardenburg syndrome
Why are babies born with cleft lip?
During pregnancy, the fetus is developing in the mother’s womb. The elements that form the face are formed between the third and eighth weeks of pregnancy. I call this nature’s miracle. Most times we know what happens and have no idea why this happens.
Getting back to the formation of the face, there are many different elements that have to fuse together for the face to form normally. I will name a few here:
- Fronto-nasal prominence
- Maxillary prominence
- Mandibular prominence
- Nasal placode
- Medial nasal prominence
It is easier to visualize this in a figure. I found this on Pinterest explaining the phases of face growth.
How common is cleft lip?
This article from India comments that worldwide, a baby with a cleft is born every 2 minutes. The incidence of the cleft lip can vary depending on what part of the world one comes from. I searched the literature and found the incidence of the cleft lip to be different in many countries. I have provided them in the table below.
|Country||Clefts (Cleft Lip with or without Cleft Palate)|
|South Korea||5.57 - 18.1|
How do you prevent cleft lip in babies during pregnancy?
There are many risk factors for a cleft lip that are described in the literature. I will tell you what those risk factors are and then it will make sense of how to prevent those risk factors. Based on that appropriate strategies can be employed to prevent clefts in babies during pregnancy.
I found this study that lists various risk factors for a cleft lip. I will summarize them here and mention how you can prevent them next to it in the table below.
|Risk Factors||Preventive Ideas|
|Smoking during pregnancy||Needless to say, stop smoking today - Pregnant or not. There are many resources available to seek help|
|Secondhand smoke exposure during pregnancy||Stay away from smokers as even secondhand smoke can be a problem. If staying away not feasible, educate them|
|Drinking alcohol during pregnancy||Needless to say stop this today.|
|Lack of folic acid & vitamin deficiency||Increase folic acid and vitamin intake. Discuss with your OBGyn|
|Certain medicines during pregnancy||Discuss with your OBGyn and the doctor who has prescribed these medicines|
|Diabetes during pregnancy||Discuss with OBGyn|
|Obesity||Maintain healthy body weight|
|Older maternal age||Some studies say older maternal age increases risk but not all studies. Well, it's preferable to get pregnant before the age of 40 years but may not always be feasible|
|Stress||Multifactorial way to assess and manage|
|Low zinc levels||Increase zinc intake. Discuss with your OBGyn|
|Dietary factors||Eat less pizza and less meat (personal opinion - don't eat them). Increase fruit intake|
|Coffee & Tea||Unclear. Follow general guidelines on restricting caffeine during pregnancy
|Coca-Cola and Soft Drinks||Personal opinion - Don't drink them.|
|Geographic variation||There is unfortunately nothing much you can do about it. This is where you are born. I am not sure if you migrate to a different country if the incidence changes.
|Genetic risk factors||Discuss with OBGyn|
Does cleft lip come from mother or father?
A study evaluated the relation between parents having cleft and the possibility of a cleft in the baby. This study had 540 children with cleft lip or palate or both and of those 127 children had parents with clefts. The type of cleft in the child depends on two things
- Type of cleft in the parent
- Sex of the child
According to this study, if its a baby boy, he is at increased risk of cleft if:
- Mother has a cleft lip
- Mother has cleft lip and palate
- Father has a cleft lip
If it’s a baby girl, she is at increased risk if
- Mother has a cleft palate
- Father has a cleft palate
If I have to simplify this, a cleft lip can come from either mother or father if any of them are carrying the genetic defects that predispose to the cleft.
What foods cause cleft lip?
I have mentioned the list of foods that have been found to increase the risk of cleft lip according to a study from the Netherlands.
- Pizza diet
- High meat consumption
- Low fruit intake
- Foods low in folate, zinc, and other vitamins.
The influence of diet is likely multifactorial. It is possible that those people eating the above diet may also be consuming alcohol and smoking or have other risk factors.
According to this same study, these foods did not increase the risk of a cleft lip:
- High fish intake
Now you know what to eat.
Can a cleft lip be detected in an ultrasound?
A cleft lip can typically be seen on the ultrasound during the 20th-week scan. The sonographer should be on the lookout for this as this may not always be obvious. The sonographer should also have a good understanding of face development and embryology.
Can a cleft lip heal in the womb?
There is a condition reported in the American Journal of Medical Genetics called congenital “healed” cleft lip (CHCL). This is an unusual anomaly with a scar on the upper lip and appears that a cleft lip has been healed in the womb during pregnancy. This is perhaps a variant of cleft lip. I did not find any report of a cleft lip spontaneously healing in the womb during pregnancy.
Is cleft lip considered a disability?
According to The Cleft Lip and Palate Association (CLAPA), cleft lip is not considered a disability. The CLAPA is a small charity that supports people with clefts and their families in London. However, people with clefts can have associated learning disabilities that might put them behind in school. These people can have speaking difficulties, hearing difficulties, and low self-esteem.
Is a cleft lip from alcohol?
A cleft lip can result from alcohol drinking. When I explored specifically the relationship between alcohol and cleft lip, I found this study published in the Europen Journal of Epidemiology.
This study said that mothers who binge drank alcohol had an association with an increased risk of cleft lip. To get into more specifics, binge drinking was an average of 5+ drinks. Women who did this amount of binge drinking 3 or more times during the first trimester (first 3 months of pregnancy) had a two-fold increased risk of cleft lip.
The study interestingly said that women who drank less than this at non-binge levels did not have an increased risk of cleft lip.
Hold on – before you get too excited, drinking alcohol during pregnancy is a terrible idea. It’s not only about clefts, but any amount of alcohol is bad during pregnancy for other reasons. Alcohol drinking during pregnancy can lead to a spectrum of problems called fetal alcohol syndrome.
Is there a link between cleft lip and autism?
Children with cleft lip and palate had an increased risk of autism spectrum disorder. According to a study from Sweden published in the Journal of American Academy of Child and Adolescent Psychiatry, children with cleft lips had a significantly higher risk of the following:
- Any psychiatric disorder
- Intellectual disability
- Language disorders
Is cleft lip related to mental retardation?
I have to refer to another study here. This study finds that in people with clefts associated with syndromes, cognitive defects (low IQ, language deficit) are frequent and they can have mental retardation. On the other hand, if it’s an isolated cleft lip without syndromes, the cognitive defects are less severe and the children may otherwise be normal. However, they can still have other consequences that can affect normal daily living.
How many surgeries does it take to fix a cleft lip?
A cleft lip is generally fixed when the baby is between 3 to 6 months old. This may also be done later. In my experience, this is fixed with one surgery done under general anesthesia. The surgery generally takes about 2 hours including anesthesia time. The baby is kept in the hospital for a day or two after the surgery.
What anesthesia is used for cleft lip?
The cleft lip surgery is performed under general anesthesia. In my experience, babies have inhalational induction with halothane (not used in most parts of the World) or sevoflurane with an anesthesia face mask. There are some instances where intravenous induction may be used. After the baby is off to sleep as noted by loss of eyelash reflex and muscle tone, an intravenous line is placed. Some may choose to get the baby off to sleep with intravenous anesthesia. The time taken to go to sleep depends on a few factors. Following this the following medicines are generally administered:
- Additional anesthesia medication – usually propofol. Sometimes this is skipped
- Pain medicine – typically a small dose of fentanyl
- Muscle relaxant – Vecuronium or rocuronium or cisatracurium
How is airway managed for cleft lip?
Following the administration of the above medicines and a few minutes for the medicines to kick in, the airway is managed with a breathing tube. A special breathing tube is called an RAE tube. This is a special tube that is performed and stands for Ring, Adair, and Elwyn for the authors who published the paper on the RAE tube. The size of the tube is determined based on two methods.
- An age-based standardized formula
- Size of the little or the index finger (Diameter of distal phalanx)
I personally use the age-based standardized formula. The formula is
This gives the size of an uncuffed endotracheal tube. I generally have found it helpful to use the same formula for a cuffed tube and go down half a size if I have to.
The breathing tube is placed using an instrument called a laryngoscope – either direct or video.
Here is a video where I have explained the anesthesia and the airway management in action. Check this out:
Is cleft lip surgery painful?
A cleft lip is a surgery and it tends to be painful. However, the pain will be significantly reduced by the following methods:
Local anesthetic infiltration by the surgeon – this is administered prior to the start of surgery and helps reduce the use of intravenous opioids.
Nerve blocks – A nerve block can be done by injecting local anesthesia in the infraorbital foramen to block the infraorbital nerve. I personally used to do this myself when I used to practice in India
Opioid analgesic – in my experience, this is usually a small dose of fentanyl. I would not be surprised if some anesthesiologists chose to administer morphine. All efforts are made to minimize or avoid the use of intravenous opioids.
Here is a video I have made on the available options for pain killer medicines to take at home. Check this out:
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