(January 15, 2009 - Insidermedicine) Dr. Leila Mankarious MD, a specialist in airway disorders, pediatric Cochlear implantation and cleft lip and palate, discusses what she would do if she had a newborn who had difficulty feeding. Dr. Mankarious is an Assistant Professor in the Department of Otology and Laryngology at Harvard Medical School as well as a Pediatric Otolaryngologist at Massachusetts Eye and Ear Infirmary.
On a recent trip to Boston we caught up with Dr. Leila Mankarious who is an Assistant Professor in the Department of Otology and Laryngology at Harvard Medical School, a Pediatric Otolaryngologist at Massachusetts Eye and Ear Infirmary, Dr. Mankarious specializes in airway disorders, pediatric Cochlear implantation and cleft lip and palate.
How might a newborn present with a cleft palate?
Dr. Mankarious: So typically patients present at birth with feeding problems, that’s their primary issue initially. They often need a special feeding bottle because patients with cleft palate cannot generate an appropriate suck, there’s a big communication between the mouth and nose, so as they try to suck, the air just comes in through their nose and they can’t seal the two off. So there’s a specific bottle that will actually deliver food for children rather then them having to develop a suck. As long as the patient can be feed adequately and have no other airway problems: coughing, difficulty breathing, the patient can really be allowed to grow and the need and urgency of seeing a specialist is not mandatory. You really need to get over the hurdle of feeding, once you get over the hurdle of feeding then the rest can come at its leisure, there’s no emergency.
What is the function of the palate?
Dr. Mankarious: The palate’s main function is twofold, one it is a structural barrier between the nose and the mouth and secondly the back half of it performs defined motor movements in order to develop and articulate clearly. You can imagine if my palate didn’t work well and I had a big communication between my nose and my mouth sounds, that should normally come out purely through the mouth would be leaking out of my nose. For example, Fish would sound as “feesh” and puppy comes out as “puffy”, so that you get inappropriate ear leak through your nose. In addition, it holds food purely in the mouth, so that if I swallowed from a bottle or a cup and I had an incompetent palate, it would come out through my nose. So the palate really is a structural barrier but then it also performs these fine motor skills that will clearly articulate sounds that we need to say. That we need to articulate to be clear to our listeners.
What constitutes the assessment of a cleft palate?
Dr. Mankarious: The cleft palate can be small, as small as a bi-fib uvula, which means just the uvula never fused, its now split into two segments or it can be very large extending all the way from the uvula out to involve the lip, the alveolar ridge that houses the teeth. So depending on the extensiveness of the cleft palate, dictates what kind of testing needs to be done, typically it’s just an exam to start and usually just by looking at it you can get a sense as to whether or not its extensive or it’s a minor cleft also you’re looking to see if the muscles have fused in the midline and if those muscles are actually functional, do they close off the nose from the mouth or are they just flopping in the wind if you will , flopping in the breeze if you will and not making contact with each other and not able to perform a successful lift and closure of the nose from the mouth so those are really the things you’re looking for initially. There’s no real testing in terms of x-ray that needs to be done in the beginning, those are usually later stages tests that need to be done when speech becomes more of an issue and initially it’s not so much of an issue, just feeding and growing is the main issue. So initially no testing, later we do extensive testing and looking at the fine movements of the palate and if it’s actually doing what it needs to do to perform adequate speech and language skills.
Are there genetic tests that are generally recommended?
Dr. Mankarious: Initially when a baby is born, we usually have patients see geneticists. There’s a number of syndromes of which cleft palate is one component of other systems that are affected, for example Velo-cardio- facial syndrome is a relatively common syndrome where the palate and the heart are affected together, those genes co-segregate which means that if they have an abnormality they often are affected together, so the palate can be affected alongside of the heart. There’s a few syndromes that cleft palate may be the most obvious thing initially, but there are other systems involved. So geneticists would typically search for those and do some genotyping or looking for gene mutations .
Is imaging necessary?
Dr. Mankarious: Later in life as you’re trying to assess the fine motor skills of the palate we often will do what’s called fluoroscopy, which is an x-ray that looks like a movie where you’re actually looking at the movement of the palate and you can look at it from the front and you can also look at it from the side, so you can get a pretty good three-dimensional view of what is happening with the palate. We also do aside from x-rays, we also do a fiber optic examination , which is a small spaghetti like structure that allows us to visualize in the nose, we pass it into the nose and that way you can see the palatable movement from the top so you can get a sense as to whether the side walls are closing, is the palate making adequate closure as well or is there leaking? Does the palate have good strong function and there’s just a big gap or is the palate weak and not moving properly?
Dr. Mankarious: So if I had a newborn who had difficulty feeding, first things first you want to make sure that the patient is able to eat, feed, grow. Not to uncomfortable for food coming up through the nose. There is one option for initial non-surgical management, you can have an obturator made which is a piece of plastic that can fit and close the hole, it’s a little bit difficult in patients who don’t have teeth. But then cleft palate is often closed at around one year of age. You’re trying to strike a good balance of allowing your patient to grow and not causing growth restriction due to scarring versus the patient developing abnormal speech because they have a large miscommunication between the nose and the mouth. So usually around the age of one patients are corrected, after that as the patient ages and speech becomes more of an issue then we do testing, initially a speech evaluation and that’s followed by a fluoroscopy or fiber optic examination to look at the fine motor skills of the palate. And then there are always corrective actions that can be done either through speech therapy, strengthening the muscles or through surgical intervention where you basically close off a large gap between the nose and the mouth.