Cleft lip and cleft palate

Dr. Sylvester Ikhisemojie

Written by Dr. Sylvester Ikhisemojie

This is a developmental anomaly involving the face and nose which occurs very early during a pregnancy. It is one of the most common birth defects seen in new-borns and it refers to a deep defect occurring in a ridge between the nose and the mouth such that it is often deep enough to involve the floor of the nostril. This is known as a cleft lip. It may occur on one side of the face or the two sides. When the two sides are involved, it gives a baby the appearance of a big cat often referred in the past to as a "hare lip". That suggests a closer resemblance to a rabbit but it is a term that has now been discarded because it is considered offensive. 

Sometimes, this defect involves the palate in which the deep groove may also involve the floor of the nose. This is thus known as a cleft palate. Either of these problems may occur alone or together. Sometimes, it affects the centre of the face extending from one nose to the middle of the upper lip. It is a range of defects that a baby may be born with as a result of certain factors which make it impossible for the various plates of tissue that form the face to meet perfectly in their designated places. The result of that failure to integrate properly is what we are talking about this week. The cause is not known.

Cleft lip is described when the split in the lip does not affect the palate in the roof of the mouth. It may be complete or incomplete but the approach to its repair either way is the same. It can occur separately from a cleft palate because they develop separately. When the palate is cleft, the uvula in the centre of the throat may also be split in two. It assures a direct connection between the mouth and the nose.

Cleft lip is two times more common in males than in females. The cleft palate that occurs without cleft lip is two times more common in female children. The risk factors are: increasing maternal age, smoking in pregnancy, obesity, diabetes and certain anticonvulsants deployed in the treatment of seizures.

Other drugs that are known to predispose mothers to having a baby with cleft lip or palate or both are drugs used in acne treatment, containing Accutane and also, Methotrexate. The latter drug is used in the treatment of cancer, psoriasis and arthritis. A sibling who has a cleft lip or palate makes it far more likely for another child in the family to have it. In good centres with modern ultrasound machines, the defect can be detected during routine scanning in the ante-natal period and the mother adequately prepared psychologically for what is coming her way. In such patients, and in places where the expertise is available, the surgical repair can even be done soon after birth. In some others, treatment may be carried out all through the life of the patient, with some requiring dental work to correct their dentition. Achievement is made with one operation alone but this may also be done in a series with the result that one team of cranio-facial surgeons takes care of such patients literally from the cradle to the grave.

The problem may appear to be very intimidating but it is eminently treatable by surgical operations of various types. The type of corrective operation employed depends on the severity and complexity of the defect and whether it occurred alone or in association with some other defects.

Many surgeons have progressively lowered the period of surgical intervention gradually over the last two decades with the result that many of the cleft lip repairs are performed within the first 10 weeks of life. With adequate exposure during the operation and the availability of skilful hands, results are delightful in most cases. When a child has a cleft lip, that intervention solves the problem long before it is time for that child to talk. This helps prevent the development of speech difficulties.

Other problems associated with this condition are recurrent ear infections, feeding difficulties and chest infections. These are more commonly found in association with the cleft palate. Where the two of them occur together, as in combined cleft lip and palate, the lip is thus repaired first in the first two or three months of life, followed at a later date by repair of the palate. That is usually done at no later than 18 months in ideal situations.

The diagnosis of the condition is rather straight forward being that it is an obvious defect which is easy to elicit on physical examination of the mouth, nose and palate. An effort must be made to determine the full extent of the problem so that a full documentation of the problems can be made. In that way, it becomes possible to offer what is possible in respect of treatment and what the prospects for full recovery might be. The case of ear infections in particular, can be prevented from being complicated in a variety of ways.

An artificial patch can be placed over the defect in the palate so that the repeated soiling of the middle ear can be prevented. That allows the child feed better and also gains enough weight to be safe enough for the operation that is required. The ear infections can be attenuated by the placement of special tubes in the middle ear to aid drainage and help control such infections which can cause hearing loss when they are recurrent.

In Nigeria, late presentation is often the rule. Sometimes, babies with this sort of abnormality are even thrown away and left to die. Yet others are neglected at home in a benign sort of way and left to die nonetheless.

So infanticide, abandonment and social ostracisation of these babies are events occurring all the time even in our midst. However, many more people have begun to come forward within the last few years to place their problems at the feet of doctors, nurses, speech therapists and psychologists without shame in attempts to seek a solution.

Others have been rescued by various non-governmental organisations stepping forward to assure the parents that these problems are not necessarily a death sentence. The operation is performed by different specialist surgeons who could be Burns and Plastic Surgeons or Maxillofacial Surgeons or by Paediatric Surgeons.

The operation is a painstaking one with careful consideration for the final result. The lighting must be adequate in a modern operating room and the sutures used are of the finest, thinnest grade that the eyes could conveniently see. In addition, the anaesthetists in charge must have adequate training and qualification to handle the cases so that the outcome is a pleasant one. It is when all these considerations are met that it is considered as prudent for the operation to go ahead as planned. It is not an operation that should be attempted in every hospital unless the relevant infrastructure and manpower requirements are fully deployed.

The specialists who should thus treat these patients are as follows:

1) The plastic surgeon to perform the necessary operations on the lip and palate.

2) An oral/ maxillofacial surgeon to reposition segments of the upper jaw as required so that function and appearance can be improved.

3) An orthodontist to reposition and straighten the teeth.

4) A dentist to do the required routine care of the teeth and gums.

5) An ear, nose and throat surgeon to determine any hearing problems and plan for a way to treat what is diagnosed.

6) An audiologist to evaluate who is an expert in disorders of communication resulting from hearing disorders to help assess and monitor hearing.

7) A speech therapist who will work with the child to improve the speech.

8) A social worker to support the family and assess any problems in the home to do with adjustment.

9) A nurse coordinator to supervise the child's health and monitor the progress.

10) A paediatric surgeon where 1 and 2 above are not available in that hospital.

It must be stated that the above organogram is available in ideal centres in reputable hospitals in western nations. In Nigerian hospitals where this condition is treated, 6 or 7 of those factors will be available but the work will be done with little coordination amongst the various staffs. As a result, much less is achieved.

_____________________________

Ask the doctor

Dear doctor, I am 45 years old. Is goitre hereditary? I noticed it in my mother. I am praying not to have it even though I noticed from about the age of 20 years that I had it. I want to go for an operation although I am very scared. What are the chances? Should I go to a private or a public hospital? God bless you sir. 

Goitre is not hereditary but it may run in certain families because they grew up eating the same kind of food and share exposure to the same kind of environmental factors. The operation can be performed safely in either public or private hospitals because it is usually the case that whoever will do the operation in a public hospital would almost certainly be operating in a private hospital. The choice of where to do it has to be yours.

--------------------------------------------------

Dear doctor, at last, you wrote about my case and I know that by God's grace, a solution has come. I am suffering from chronic osteomyelitis. How can I see you?

You do not have to see me. You need to have an evaluation of the extent of your problem from an orthopaedic surgeon. Depending on where you live, you should see one in any of the large public hospitals in your city.

-------------------------------------------------

Dear doctor, the lower sides of my abdomen pain me a lot. Please what type of test can I run to know what is wrong?

You have not given us enough information to be able to help you but the first thing to do in the circumstances is to see a doctor for a good physical examination. He will then probably ask for some blood and urine tests as well as an abdominal ultrasound scan.

---------------------------------------------------

Dear Doctor, what's the real cause of the eyeball of some children to shift to one side? When they look at you, it would appear that they are looking at another person. What will one do to avoid having such a child? 

The cause of that problem is that the eye muscles are not balanced. As a result, the two eyes cannot be directed at an object at the same time. This situation is called a squint or crossed eyes or strabismus. It is not something to do with what the child has done or what any of the parents did. It can be treated. There is nothing you can do as a person to prevent your child from having it.

----------------------------------------------------

Dear doctor, I am neither an orthodox medical doctor nor a traditional healer but I'm touched by the person who wrote you to say the daughter has HbSS and has been having pains. The person can contact me so that I can see how I can assist them. 

Your number may have to be published in full so that the person can contact you directly. Thank you so much for your offer.

--------------------------------------------------------

Dear doctor, I have a unique problem as a 43-year-old mother of two teenagers. My periods refused to come for more than eight months now but while on a recent trip to the US, I developed a severe lower abdominal pain that did not reduce even with pain-killers. I contacted a doctor and he recommended some others drugs for me which did not help much. Then, after a week of suffering, my period just came out with clotted blood and the pain went off. What could have happened? 

You are still young at 43 to become menopausal. It is perhaps a result of a hormonal imbalance that this has happened. The periods for the most part of a woman's life are regular but then, it is not exactly like clockwork. You will have to observe and see whether this happens again or if your periods will now flow regularly for some time before it finally stops. If it delays again soon, you should see your doctor and have a hormone analysis done. That will make clear what exactly is wrong with you at this time. And if the problem has corrected itself, as is very possible, nothing may be found wrong with you on the other hand.

-----------------------------------------------------------

Dear doctor, Is it in every case that we have to see a specialist doctor? I am asking this because I am an avid reader of your column but you are always telling people to see this or that specialist. 

My dear, it is not in every case that such advice is given. There are occasions when I ask people to see a doctor, or see their doctor but when their complaints require specialist attention, I make sure to advise them to seek such help.