Understanding cleft lip and cleft palate
Cleft lip and palate is a relatively common congenital problem occurring in 1 in 750 live births and many of us have probably seen or known at least one person with this condition. Joaquin Phoenix was born with a mild type cleft lip and NFL quarterback Peyton Manning had to have a cleft palate repaired. To many even physicians there is some mystery and confusion behind the diagnosis. The confusion stems partly from the history surrounding its treatment. In ancient Greek and Roman society (and throughout more modern western civilization) those with clefts were seen as cursed and often abandoned as infants. In South American cultures patients with clefts were considered supernatural and many artifacts of clefts have been discovered in Central and South America. Exhumed mummies have demonstrated characteristic skull findings on CT scan of cleft palate and this was likely a familial trait in one of the Egyptian royal families.
One should not confuse cleft lip and cleft palate – they are two distinct things. Cleft lip can occur as either a complete or incomplete cleft. A complete cleft will go from the free border of the lip all the way through to the nostril. Incomplete clefts will have at least a small band of tissue (called Simonart’s band) that separates the lip and nostril. Mild forms of cleft lip may look like a “scar” on the lip a notch or a dimple. The muscle is always affected in cleft lip and will have inappropriate connections with the other tissues. This can cause significant distortion of the nose and can often be the most challenging part to correct.
Cleft lip surgery is often done at three months of age when the infant is old enough to more safely undergo anesthesia. This also allows plenty of time for the parents to bond with the infant and understand the cleft. The surgery to close the cleft varies depending on the extent of the cleft and whether unilateral or bilateral. The surgery can be done as an outpatient or overnight stay. Most times the child can go back to feeding as normal and there is usually very little pain. At the same time as the lip repair a “rhinoplasty” can be done to reshape the nose and make a more symmetric appearance.
Cleft palate involves a cleft of the soft and sometimes hard palate. Cleft palates affect both speech and feeding. These infants often have difficulty latching and feeding and often formula/milk will come out of the nostrils. Repair of this cleft should be done before the time the child develops speech. Usually this is done around 10 months of age. There are two main categories of cleft palate repair – straight-line and Furlow (Z-plasty). Both techniques have benefits and can be tailored to the individual needs of the patient.
There are often many other surgeries that a child with cleft lip and/or palate may need. Almost all children with cleft palate will need “ear tubes” to prevent frequent ear infections. This is often combined with the cleft lip surgery. Speech or VPI surgery often is done around 5 or 6 years of age and is used to reduce air escape through the nose when the child speaks. It can either be done with flaps or fat grafts in the back of the throat. Lip revisions or fat grafting can also be done during the early school years if there is teasing occurring. If there is a gap in the gumline then around age 8 or 9 years an alveolar bone graft may be needed. This is done in order to provide enough bone for the orthodontist to move teeth into with braces. Around adolescence a cleft palate patient may display an under projecting upper jaw. This is often referred to as an “underbite” but more correctly as maxillary hypoplasia. These patients will need surgery at the time of skeletal maturity to correct the bite and restore the appropriate contour to the face. In severe cases where there is scar tissue restricting growth a distraction surgery may be done at an earlier age. Rhinoplasty is often reserved for the final stage near adulthood however in certain cases can be combined with other lip revision surgeries in order to correct extremely troubling asymmetries. The cleft rhinoplasty is more challenging than a conventional rhinoplasty and often requires graft material – sometimes taken from a rib.
Many other specialists can become involved in caring for a cleft patient. Early in life a thorough examination should be performed by a pediatrician to make sure there are no other anomalies that require workup. Cleft palate patients often have feeding difficulties so a consultation with a feeding specialist or dietitian may be needed to assure proper weight gain. Certain families may pursue genetic testing if there are other anomalies or if other first- or second-degree family members are affected. MOST cases of cleft lip are isolated however about 40% of isolated cleft palate cases are associated with a syndrome. An ENT is seen early on as essentially 100% of these children will need at least one set of ear tubes. After development of speech has begun these children will need regular participation in speech therapy in order to prevent irreversible speech compensations. Many cleft palate patients will have difficulties with dental hygiene so regular visits with a dentist are mandatory. Sometimes a tooth extraction will be needed if there is an accessory tooth or an unhealthy tooth in the cleft. An orthodontist will need to see the child in preparation for an alveolar bone graft (if needed) and following because these patients typically all need braces. Some children (or families) have difficult times socially or psychologically dealing with the cleft and sometimes specialists in these areas are needed as well.
Cleft lip and palate surgery can be a very complex journey for a child and their family. It is important that any child with a cleft see a team or provider with firm understanding of treatment . Due to the large number of visits and strain on the family many patients fall through the cracks and become lost to follow-up. These children often miss steps in the process and can require more extensive revisions later in life. I have additional training in pediatric and craniofacial plastic surgery and special interest in cleft lip and palate. If you are interested in learning more or would like to refer a patient or family member with a cleft please call (765) 962-4872.