Text supplied by Glenn Bartlett and Tristan de Chalain, Plastic Surgeons, Middlemore Hospital.
When you first realise your child has a cleft you may not be aware that there is great variation in the types of clefts.
The range of these clefts extends from a simple partial cleft or incomplete cleft of the lip to a complete cleft of the lip and the palate. The cleft may be on one or both sides. The greater the cleft the more complicated the treatment becomes, as there are more structures involved.
Often there is just a simple cleft of the soft palate without involving the front or hard palate or the lip. Obviously the surgery of the simple clefts of either lip or palate is much more straightforward than more complicated clefts.
Although an individual with a cleft of the lip or a cleft of the lip and palate has an obvious cosmetic problem, it is the potential for speech difficulties that concerns the cleft team most.
In the normal soft palate (check your own in a mirror – the soft palate is the back portion of the roof of the mouth, ending in the uvula) there is a sling of muscle that moves the soft palate when you speak. This sling of muscle pulls the soft palate back and up against the back wall of the throat. Now your nose is separated from your mouth and you can project your voice in the normal manner from your mouth.
If you have a cleft palate this sling of muscles is interrupted. Instead of the muscles from each side meeting the opposite muscle in the soft palate it runs forward in its own half soft palate to become attached to the back edge of the hard palate.
There are several different techniques for repairing the soft palate and its sling of muscle. The type of operation used depends on the length and width of the cleft. The technique also varies from surgeon to surgeon, and some units will have different protocols regarding specific techniques used and the timing of surgery.
The principles of treatment
In essence our first aim in treatment of babies with clefts is to repair the soft palate in such a way that the muscle sling is reconstructed and the palate is long enough to touch the back wall of the throat when it moves. Normal speech is our first goal. If an individual can communicate clearly and normally with his family, friends and the world around them then half the battle is won.
As a very close second, we try to achieve as near a normal appearance as possible. Although perfection is not usually achieved, the results these days are so good that often the scars of surgery are difficult to see.
Finally at a later stage the Orthodontic Specialist aims to treat the teeth in such a way as to achieve two normal arches of teeth. Most of the individuals treated by well-established multidisciplinary cleft teams eventually have such good teeth it is difficult to pick that they had a cleft in the first place.
The surgical solutions
In most units in New Zealand, the basic approach is to repair a simple cleft lip at around 3 to 5 months and a simple cleft palate at around 9 to 12 months. For each operation your child is usually admitted to hospital the day of, or the day before surgery and may stay up to 5 days afterwards. The operations are done under general anaesthesia, which is administered by a specialist anaesthetist whose special duty it is to keep your child safe and well. Although nobody can stop parents from worrying about the operations, it is very rare indeed that anything goes wrong with the anaesthetic.
Cleft Lip Procedures
If your child has a cleft lip alone you may have noticed that the normal lines and folds seen in the normal lip are absent or distorted and the height or length of the cleft side is different from the normal side. The aim of the surgery is to unfurl the cleft edges to make the lip the same length or height as the normal side. The portions of the lips are then stitched together in such a way as to leave the scars in natural lines.
Cleft Lip & Palate
If there is a complete cleft of the lip and palate, your surgeon may advocate a two stage palate repair; in these cases, not only is the lip repaired at the first stage at 5 months but the front of the palate also (the hard part of the palate). Other surgeons favour a one stage repair and may repair the lip only at the first operation and then address the whole palate at a second procedure. You should ask your surgeon about his plan for your child and the reasoning behind it.
Babies with complete clefts of the lip or complete clefts of the lip and palate nearly always have asymmetric noses. The cartilages that form the shape of the tip of the nose are not the same on each side. The cartilage in the tip of the nose on the side of the cleft is pulled outwards and slumps. During the first operation in which we repair the lip, we also attend to this asymmetry of the nose and try to get this as symmetrical as possible. Again, there are many different ways to approach this problem and you should ask your surgeon about their particular approach.
A few months after the lip (and nose) have been operated on, attention is turned to the palate. Here, as stated, there may be a cleft of both the hard and soft palate, or just the soft palate (this may have been the original extent of the cleft, or what was left after a hard palate repair). Your surgeon will be happy to explain the exact surgical technique to be used to repair your child’s cleft, but there are several different methods available. Most rely on the principles of mobilising tissues on either side of the cleft and bringing these across the midline to be sutured together to achieve a watertight seal, separating the nose from the mouth.
Clefts of the palate are repaired in a variety of ways, for example:
Veau type procedure
One of the standard methods is to raise long flaps from each side of the hard palate with a pointed end at the front of the mouth. After cutting open the inner edges of the clefts the muscles are freed from the back edge of the hard palate. These side flaps can now be pushed backwards and stitched together in the middle as well.
Von Langenbeck type repair
Another common method is to make cuts on the inner edges of the cleft and parallel cuts near the gums. The muscles are again dissected free from the back edges of the hard palate and sewn together in the midline at the back of the soft palate. Again, the linings of these flaps are stitched together in the midline to close the gap.
If the cleft of the soft palate is a narrow or short one, a Furlow method of palate repair may be used. This uses a system of triangular flaps, which both lengthen the palate and place the muscle bundle as far back in the soft palate as possible, allowing the muscle to work most efficiently. The method also has a major advantage of not causing any scarring at the front of the mouth. Scarring at the front of the mouth is thought to play a part in holding up normal growth of the upper jaw and this distorts the growth of teeth.
Because each child’s cleft is different, it is not possible to predict exactly what surgery will be necessary. Each child is regularly seen in clinic and assessed by a team of specialists. Along the way, as a child grows and develops, cleft-related problems may arise that will need further surgery. These operations range from very simple ones to fairly major adjustments.
The simplest one usually consists of minor adjustments to the lip repair because even with the best techniques and efforts there are often minor abnormalities left after the first operation. Usually an attempt is made to correct these before or during early school years.
Very occasionally the repair of the palate does not hold over the whole length of the repair. Such breakdowns create a connection between the nose and mouth called a fistula. These too are usually repaired early in life to stop the inevitable escape of fluid or food through the nose and prevent the speech sounding nasal.
Some children with a cleft, despite having had a successful repair, may go on to develop what is called velo-pharyngeal incompetence or VPI. In this condition, the child is unable to prevent the escape of air into the nose, during speech. There may also be nasal regurgitation of food and fluid, which is both uncomfortable and distressing, and is also a social problem for the child.
The causes of VPI are many, but to help in planning surgery to correct this, investigations such as a “movie x-ray” of the palatal structures during speech, a videofluoroscopy, may be called for. In some older children, a nasendoscopy, may be required, in which a thin endoscope, carrying a TV camera is fed into the nose and the movements of the back of the palate and throat can be studied during speech.
Once the team has established where the air/ fluid leak is occurring, an appropriate operation can be planned to correct this. Usually the problem is one of a palate which is too short to make adequate contact with the back of the throat, or the throat itself is too wide and roomy. On occasion, very large tonsils may be obstructing palatal closure.
There are several procedures available to treat this type of problem, but most surgeons will use some form of pharyngoplasty, aiming to narrow the throat from side to side and build up the back of the throat. This directs the air stream centrally and maximises the potential contact between palate and throat wall. They will sometimes use a flap of tissue from the back of the throat, sewn directly to the palatal soft tissue – a pharyngeal flap. Again, your surgeon will be happy to explain the nature of the problem and the proposed solution to you.
In those children who have had a complete cleft of the lip and palate repaired, there is one area that is not touched. This is the gum or alveolus. Early surgery to repair this is also thought to stop normal growth of the upper jaw. Click here to read more
If there is enough abnormal growth of the upper and lower jaws to prevent orthodontic treatment to line up the teeth adequately, surgery may be required to move the jaws into a better relationship. Click here to read more
Finally the nose may need further surgery because not only are the bones of the nose in need of straightening and often narrowing, but the cartilage in the middle of the nose is very commonly twisted. This interferes with the air passages in the nose and sometimes the drainage from the sinuses. An operation called a Septorhinoplasty deals with both the central cartilage and also the outside bony shape of the nose. Although it is usually not possible to produce a thin fine “Hollywood” nose, very great improvements are usually achieved, and the individual looks normal.
The Rhinoplasty is usually done after the jaw operations because moving the jaw changes the shape of the nose. The Septorhinoplasty is therefore often the final correction for an individual who is born with a cleft.