Written for Prescription Parents. Dr. Lewis can be reached at Department of Plastic Surgery, New England Medical Center, 171 Harrison Ave., Boston, MA 02111
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Secondary surgery is that done sometime following the initial (primary) repair of the cleft lip and palate, which has usually been completed during the first year of life. The range of surgery encompassed in this category is quite wide and varied. It includes procedures to improve the appearance of the lip and nose, to improve the function of speech, to close abnormal communications between the mouth and nose, and even procedures to move and stabilize the upper and lower jaws. Not all children will require or benefit from these secondary procedures. It is impossible to predict with certainty at an early age who will need these procedures. However, in general, the more severe the initial cleft problem, the more likely the need for secondary or revisional surgery. Timely visits with the plastic surgeon and other specialist is the best way of determining whether your child is a candidate for this surgery.
In thinking about these procedures and discussing them with the plastic surgeon many factors must be considered. The limitation as well as the purpose and goals of the secondary surgical procedure must be understood. Some of the technical detail should be discussed so as to better understand the side effects (sequelae) and possible complications. Special care and precautions necessary during, and the length of the convalescent period should be fully appreciated. Older children can be part of the decision making process related to these secondary procedures. The timing of many of these operations is not urgent nor critical. However, it is important that they be coordinated with the total needs of the child, both physical and emotional.
Major revisional operations on the lip are not common. Although many children with cleft lips and their parents seem to focus on the actual lip scar as the object for improvement, this is usually not the real need. Most often it is the lack of a cupid's bow, asymmetry, or "tightness" of the lip that should be corrected. Oftentimes, in the process of doing this the lip scar is altered and improved but can never be removed. The range of procedures in this category is great. In fact, the needs of any two patients is rarely the same. Some of the simpler procedures, which might involve only the trimming of the thickened vermilion (red portion of the lip) or minor cupid's bow alignment can be done on an outpatient basis. More complete revisional surgery can require a short hospital stay and several weeks of convalescence. The most extreme procedure in this category involves the use of tissue from the lower lip (cross-lip or Abbe flap) to augment and improve the upper lip and requires the lips to be "hooked" together in the center by a bridge of tissue for about two weeks. This bridge is then divided. Not infrequently lip revision is combined with nasal surgery.
Almost all children who were born with a complete cleft of the lip and many with an incomplete cleft will have an associated nasal deformity. It is unusual to have this totally corrected by the initial or primary surgery so that secondary surgery on the nose is quite common. The two basic deformities are those associated with a unilateral (one-sided) cleft lip and a bilateral (double) cleft lip. The degree of abnormality varies but is usually directly related to the severity of the cleft. In the unilateral case, the nose has a twisted appearance, the nostril rim on the involved side droops, the nostrils are asymmetric, and the septum is deviated. Much can be done to improve the appearance of the nose although absolute perfection is rarely achieved. Nasal tip and nostril revision is possible in early childhood although septal and bony surgery to totally straighten the nose await full or near full growth of the facial features.
In the case of children with bilateral cleft of the lips, the nose is usually broad and flat with a short columella and flaring nostrils. The septum can be deviated but oftentimes is not. Procedures to lengthen the columella and correct the wide and flaring nostrils are possible at a young age, but the final bony narrowing and septal work, if required, cannot be done until facial growth is nearly complete. Most nasal surgery can be done on an outpatient basis and necessitates a two to three week period for convalescence during which the swelling and bruising resolves and the wounds strengthen.
Not all speech problems in children with repaired cleft palates are related to poor palate function, but when palate function is inadequate (a condition referred to as velopharyngeal inadequacy) a secondary surgical procedure can be done to improve the condition. Almost always this operative procedure involves a pharyngeal flap or pharyngoplasty. It is usually impossible to do something surgically to improve palate function, but it is possible to reduce the space that must be closed off during most of speech. This in turn reduces air lost through the nose during speech. This space is reduced in size by bridging the gap between the palate and back wall of the throat (nasopharynx) with tissue taken from the back wall or side wall. This tissue is left attached in one area and is referred to as a pharyngeal flap or pharyngoplasty. Usually following the surgical procedure, speech therapy will be required to correct the bad speech patterns which are often associated with velopharyngeal inadequacy. This operation has some important side effects. Because the flap reduces the space in the nasopharynx, it restricts to a varying degree the ability of the child to breath through his nose, blow his nose, and may increase the stuffiness associated with "colds". Also, respirations while the child is sleeping are frequently noisy. Most children seem to adjust nicely to these changes and are seldom bothered by them. Rarely a condition called obstructive sleep apnea occurs requiring aggressive management. This type of surgery can be done at almost any age. It should probably be done when it is absolutely clear that palate function is consistently inadequate and will not be responsive to speech therapy. A two or three day hospitalization is necessary and a restricted diet will have to be followed for about two weeks.
An oronasal fistula is an abnormal communication between the mouth and nose. A certain number of children with cleft palates, with or without cleft lips, will be left with an oronasal fistula after the primary repair. In some cases the fistula has been left intentionally, in others it has developed because of poor healing in the area. Early on these fistulae are a nuisance in that liquids, and occasionally solid food goes through into the nose. As the children get older, most learn to control this problem completely. Rarely, the fistulae are so large they create a speech problem. These larger fistulae and those smaller ones that continue long term to create a problem can be closed surgically. Most can be closed by using local tissue from the roof of the mouth but occasionally, where more tissue is needed, some is borrowed from the tongue or inside of the cheek. Because of the narrow confines of the space to be closed and scar tissue from previous surgery, closure is not always successful. A short stay in the hospital may be required and dietary restrictions for two to three weeks are necessary.
Isolated bone grafts to stabilize the segments of the upper jaw and replace the missing bone are often recommended. Virtually all cases of complete unilateral and complete bilateral cleft lip and palate will benefit from this procedure. The bone graft not only joins and fuses the segments of the upper jaw, it provides bone matrix in the cleft area. This can allow an unerupted tooth to erupt through the graft. The graft also provides the orthodontist the opportunity to straighten and move the teeth adjacent to the cleft.
The best bone grafts are autogenous, i.e., from the individual receiving the graft. The donor side is usually from the edge of the pelvis (iliac crest) although some surgeons prefer bone from the skull. The surgery is usually performed in the mixed dentition phase (age 8-10) and must be coordinated with the orthodontist.
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