’The number of craniofacial anomalies including cleft lip and palate is a little higher in India’
Prof Dr Krishna Shama Rao has been actively practicing craniofacial surgery since 1991, establishing the first dedicated cleft and craniofacial centre in Mangalore and later on through Maaya Foundation setting up 11 centres across India, where free surgery is provided to the patients involving both cranial and facial deformities. Since 1991, he has completed over 16,500 such surgeries, both in India and abroad. In an interaction with Lakshmipriya Nair, he explains further about craniofacial deformities, the reasons they are caused and how craniofacial surgery is beneficial and more
Tell us more about craniofacial surgery and the problems that it addresses?
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| Prof Dr Krishna Shama Rao |
Craniofacial surgery is a very niche superspeciality which deals especially with deformities involving the cranium (skull) and the face.
In some instances, either only the skull is deformed or only the face is deformed in isolation, whereas, in a larger number of patients, the entire skull and face (craniofacial region) is affected simultaneously.
When present at birth, such deformities are called congenital craniofacial anomalies, and almost 50,000 such children are born every year in India with cranial and facial deformities.
As special training, skills and qualifications are required for the surgeon to address the problem, not only from the medical and surgical point of view, but also with a very strong grounding in the fields of dentistry, especially maxillofacial surgery. There are less than 10 active full time craniofacial surgeons across Asia and less than a 100 across the globe.
How has it evolved with time?
Pioneered by stalwarts, like Dr Paul Tessier and Dr Hugo Obwegesser, amongst others around 1968, the techniques of surgery have become both easier in terms of planning as well as execution, due to tremendous advances in imaging like CT, MRI, 3D reconstructions and also making external steriolithographic models of the skull and the face to the exact millimeter in order to simulate the surgery prior to the actual procedure. The use of endoscopes, resorbable plating systems, 3D planning software and of course high-end operation theatres and ICU’s have made the surgery very safe and most such patients are usually operated upon at the age of six to eight months itself.
In fact some deformities where one needs to lengthen the bone using specialised distraction devices, surgery can be performed the day the baby is born in order to help save its life at the earliest
What are the challenges that you deal with in your chosen line?
The biggest challenge is the absence of awareness amongst the public as well as a lot of health professionals that such surgeries can be performed safely with excellent outcomes in India in a dedicated craniofacial centre. The surgeries are expensive as they take several hours and lots of materials and even a free surgery funded by Maaya Foundation would cost up to Rs 75,000, at a no profit, no loss basis.
The second challenge is the absence of enough trained surgeons and dedicated centres. With this in mind, Maaya Foundation hopes to initiate a separate training programme in craniofacial surgery by April 2013.
Clefts are congenital defects, so are there any factors which are responsible for it? Is it hereditary?
All congenital defects are not necessarily inherited. Cleft lip and palate has at least five genes implicated as one of the causative reasons, but, due to the low penetration of the gene, at present, one cannot label cleft lip and palate as a specific genetic defect. Hence, it is called multi factorial. However, some of the more serious craniofacial malformations like Crouzon’s, Apert’s Syndromes, have specific genes which have already been identified as mutated. It is possible in the future, that in those cases, where a specific gene is identified, gene therapy might prevent the disease completely. This is yet to happen.
How do Indians fare as far as these anomalies are concerned? Are we more susceptible to them? If yes, why?
The number of craniofacial anomalies including cleft lip and palate is a little higher in India (one in 700) as compared to the western countries where it is one in 1,000 live births. Due to the large population of India, the number of such patients is obviously high.
What are the problems caused by these deformities?
Common conditions like cleft lip and palate lead to obvious social stigma, poor self esteem, speech defects, hearing defects, etc. The more complicated craniofacial anomalies might also compress the brain leading to lower intelligence and scholastic performance, as well as compression of the eye ball leading to partial or complete blindness. In some conditions, like hemifacial micresomia, not only is the external ear missing, but the entire hearing system may be abnormal leading to deafness on one side.
What are the measures that need to be taken to avoid the occurrence of these deformities?
Currently, there are no guaranteed standard measures that can be taken to avoid such deformities. However, being in good health, and avoiding drugs and medication, during the first eight weeks of pregnancy, as well as avoiding exposure to radiation, would certainly help in reducing the chances of such a deformity. Consanguineous marriages also increase the risk of a malformed baby.
Tell us about the work done by Maaya Foundation.
Maaya foundation was started in 2006 as the first all Indian NGO dedicated purely to free treatment of children born not only with simple cleft lip and palate anomalies, but, also with the most complex craniofacial deformities. Spread over 11 centres across India, over 6500 free surgeries have been conducted since 2007. It is the vision of Maaya Foundation that each state have a comprehensive centre, which not only treats the children free of cost, but eventually, it is also self sustaining to remain active serving the society for generations to come.
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