Statistically, 1 in 1.000 children is born with cleft lip and cleft palate - birth defects that occur when a baby’s lip or mouth do not form properly during pregnancy. A cleft lip happens if the tissue that makes up the lip does not join completely before birth, and this results in an opening in the upper lip. Children with a cleft lip also can have a cleft palate. A cleft palate happens if the tissue that makes up the roof of the mouth does not join together completely during pregnancy. Prof. Dr. Hakan Ağır - an ACIBADEM expert and a world-renowned plastic and reconstructive surgeon - famous especially for the hundreds of cases of cleft surgeries he has performed during his career - emphasizes the importance of an early diagnosis.
“Most of these facial deformities could be diagnosed at the end of the first trimester or in the first half of the second trimester. The only diagnostic tool in pregnancy, at the moment, is ultrasonography for both cleft lift and cleft palace. But even in the developed countries, the chances to diagnose a cleft-lip or any facial deformity are about 60-65%. It is still not even close to 90% even in well developed countries at very good medical centers. That’s because the baby in the pregnancy period may hide his or her face and can simply not show it at all. To see the cleft palate is even more difficult. The lip is easy to see because it's outside, but the cleft palate, isolated cleft palate, has a detection rate in pregnancy of 30%, it's very difficult to detect it on ultrasonography, so most of the parents learn about this problem of their baby only after birth. But if you diagnose it, you must tell the patient about this diagnosis, even if it’s just called a suspicion. The shock, the trauma they experience, is much worse once the baby is born and they thought there was nothing wrong. This way, the impact is not so devastating and they have the chance to talk to cleft surgeons and find out more about what’s about to follow”, Prof. Dr. Hakan Ağır gives some pieces of advice.
Children diagnosed with cleft lift and cleft palate will go, on average, through 5-6 surgeries until they reach 18, maybe even 20 years old.
“We first operate on these patients when they are about 3 months old. The first operation is the lip repair. And maybe we add the anterior palate repair. The second operation, if the child has the cleft palate, as well, combined with the lip, then the palate repair will be around roughly before the age of one. In the world there are different protocols, different centers, different surgeons preferring different times to operate on the cleft palate. There is not a general consensus on the timing of the palate repair. But all the reports, all the research and studies prove that the surgery for the palate repair must be done before the age of 1 because then the speech outcome is near to perfection. 80 to 85% of these kids - if the surgery is done very well, in good hands, in skilled hands - will not have a speech problem”, declares Professor Ağır.
It’s very important that the doctor who takes on their case is highly skilled in this sort of diagnosis and surgical treatment, says Prof. Dr. Hakan Ağır, an expert who has seen many patients after they had surgeries badly performed by other surgeons. “If you are not doing these surgeries quite often you shouldn't be touching the patient”, he underlines. The risks are very big.
“On one hand we have the cosmetical aspect - the child will have a bad look, an ugly look, bad scars, bad aspect of the nose and of the upper lip. This is a stigma, we call it, a bad stigma. And then, on the other hand, we may have worse problems – a lifetime affected speech. The speech outcome after a badly performed surgery is very low, the intelligibility of the speech of the kid is significantly low compared to the results obtained by a skilled surgeon. There's a 30-40% difference, it's a huge difference. The cosmetical aspect, in a way, you can correct it, but for the speech… It’s our way of communication, the child will be affected for life”, warns the ACIBADEM expert.
“She should have a post-graduate training of at least one, preferably two years, and must be working in a unit which does quite often these surgeries and follows these kids. Americans say that as a standard a cleft surgeon must see 20 cases per year, at least. 20 new cases. If you do this surgery once a year or twice a year, you’d better stay out of it. For example, in the United Kingdom, after many surveys and studies, their national health system, NHS, made a big protocol and examined all the results, and now allows only 11 centers to do this kind of surgeries. No one else can, they don’t have an accreditation for it. This is a specific area, this is a team work, this is not the plastic surgeon’s job only, you must have a very good orthodontist, you must have a good speech therapist, a pediatrician, a good pediatric anesthetist, a good hospital, a great genetics department, they all play their roles during the protocol. Here, at ACIBADEM, I have a great team”, says Prof. Dr. Hakan Ağır.
The correction of a badly performed surgery is very difficult, Professor Ağır explains. Because the first surgeon touched and destroyed the original tissues, the so-called virgin tissues.
“And these tissues were our only chance to get a good result. It's just like, you know, fastening up the first button of your shirt. If it goes wrong, then it goes wrong all the way. And to correct it, you'll need multiple operations, even in the good hands of a surgeon. I do this, I try to correct the badly performed operations and I can hardly get the same results when they are operated originally because there are scar tissues, fibroses, scatris we call it. For example, if the clef palate is operated in a bad way, there are big holes, there will be falling apart, breaking down of the fistulas, as we call it - fistula, a hole in the palate that will require another operation. And another operation will be difficult and the risk of having another hole after that operation is very high, like 50-60%. You lose your chance, you use the original tissues that you can close the palate with. So, it's very important, there operations are very very important: the first two surgeries, the ones done in the first year of life. Because, when the surgery is done by the less skilled people, these kids need having 3 or 4 or 5 operations more”, adds Prof. Dr. Hakan Ağır.
As for the causes known so far for the cleft lip and cleft palate, we are talking about smoking, heavy drinking, drug use – even before pregnancy, the giving up does not help a lot. The medication use during pregnancy plays a role – the use of anti-epileptics, anti-convulsives, steroids may increase the risk. Not antibiotics or painkillers. Another important risk factor is the mother’s weight: being obese, overweight, or gaining too much weight during the pregnancy, receving a diagnosis of diabetes or gestational diabetes, all these situations also increase the risk.
“Severe depression is another aspect that we are researching. In my research - here at ACIBADEM I've done one - I have realized that 5-10% of the mothers that gave birth to children affected by this diagnosis were in a stressful environment during the pregnancy. Either they had to work very hard, or they had a tense marriage relationship, difficult family relationships, some depressions, some separations, and deceased in the family. You know, in our society these things are lived through very intensely. If that happens around the first two months or the three months of the pregnancy, that may affect the child's development. This is scientifically proven. Another thing that I have researched in my study group is the role of maternal age. If the woman is above 40, then the risk goes up. This is what we know. But this is not true - in my study, at least - for the cleft lip and palate. This may be true for some other cardiac anomalies or for something else, but this is not true, I couldn't prove it”, concludes Professor Ağır.