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Our responsibility lies in two specific areas in our team. First we help to manage the residual oronasal fistula that remains in a cleft lip and palate patient. Our job is to close this fistula by placing bone into the gap of the jaw in this area. This closes off a hole to the nose and provides bone support to the developing teeth in the cleft site. It also supports the side or alar rim of the nose.
The other area we follow closely is the growth of the upper and lower jaws in conjunction with one another. Approximately 70 percent of patients that have this problem have a restriction of growth of the upper jaw. This we speculate has to do with the multiple surgical procedures done during growth to allow the child to eat and develop speech properly. Sometimes these children will require further surgery of the jaw to align the bones. Here we see a case of a patient that we followed during growth to maturity that needed corrective jaw surgery.
Cleft Lip and Palate Deformities
The Oral and Maxillofacial Surgeon's Role in the Care of Patient with Cleft Lip and Palate Deformities
Abstract
Perhaps no conditions other than cleft lip and palate and oral cancer assemble teams of dentists and physicians intensely focused on treatment and rehabilitations. The oral and maxillofacial surgeon's role is often pivotal especially in the 20-year term of care for the child born with a cleft lip and palate deformity (CLPD). Issues of basic orofacial functions of mastication, respiration and communication overlay human considerations of self-esteem and image. From the moment of birth the child with a CLPD is special and challenged. Early issues concern parent and family acceptance and insuring basic life functions. While not generally a part of the immediate surgical unit, the OMFS often provides surgical consultation, educational and emotional support for the family. A clear picture must be painted of the treatment that will unfold over the next two decades. Realistic optimism is the watchword. Indeed, today there is every reason to anticipate an outcome that will result in a balanced, functional and esthetic face.
Treatment protocols for the care of patients with CLPD are constantly under review and revision. Unfortunately, it may take a whole generation to learn that a given technique that produced esthetic results in childhood had a negative effect on facial growth as the patient matured through adolescence and adulthood. There is no question that all intervention further modifies the already impaired potential for normal growth and development in the child with CLPD. Early approaches that required wide areas of surgical undermining often produced a typical midfacial deformity. Current therapy, which will be outlined in this paper, is not without controversy and uncertainty, and contains the potential for similar complications.
When a child is born with a CLPD a team of individuals is put into motion. Their goals are to support the patient's basic physiologic needs and to deal with the emotional devastation that occurs in the child's family.
As a general rule, the lip is not closed until 10 weeks of age, 10 grams of hemoglobin and 10 pounds of weight have been achieved (the rule of 10s). In the interim, a member of the dental team (orthodontist, prosthodontist or pediatric dentist) will often construct an obturator to alter the cleft or mold its shape, size and the position of its components. At times, an active orthopedic appliance is secured to the cleft segments and engineered to directly transport them into proper alignment (Latham type appliance). Occasionally, the primary surgical procedures is a simple lip adhesion, which also has the effect of approximating the displaced components. When the rule of 10s is met and these segments are in good alignment, the lip is reconstructed.
Some teams will perform a primary palatogingivoplasty at that time as well. This is an area of debate as the effect of this step on facial growth is unclear. During this period the other team members counsel the parents. At 12 to 24 months the hard and soft palates are repaired. On rare occasions, surgical repositioning of the premaxillary segment in bilateral CLPD is required (Figures 1A, B). Soon after, speech, hearing, and psychosocial and developmental assessments are undertaken.
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Figures 1A, B. Clinical photographs of 3-year-old child who required premaxillary repositioning. |
Eustachian tube dysfunction often accompanies CLPDs and may require myringotomy. Close audiologic and otolaryngological monitoring is mandatory. Intensive speech therapy is started preschool, and, when indicated, a nasopharyngoscopy is performed to help diagnose velopharyngeal incompetence (VPI). If diagnosed, a pharyngeal flap is performed before school commences. It is most important to give the child an anatomically correct speech apparatus to allow proper mechanics and to prevent development of poor speech habits.
Assessment Process
At about age five, the patient undergoes a comprehensive orthodontic examination. Cephalometric as well as routine radiographic assessment is complimented by a careful clinical examination. The bony and soft tissue defects are characterized and the facial morphology assessed. On occasion, active orthodontics to widen and even protract the maxilla is performed. The oral and maxillofacial surgeon then evaluates the alveolar cleft if present. This is typically closed, along with any residual oral nasal fistulas, between ages six and nine the presence of a permanent lateral incisor and the state of cuspid development determine the timing for this step. Next the orthodontist commences formal and active therapy. Speech therapy continues, and, when indicated, psychosocial counseling is provided. General dental care proceeds throughout this period. Often the plastic and reconstructive surgeon will perform secondary surgery to the lip and nose. During late adolescence, orthognathic surgery is performed if indicated. Finally the cleft dental gap is restored and a last lip/nose revision performed. At this point the patient is ready to move on to college or employment. It is hoped that through the efforts of all team members and particularly the child's family, the patient will emerge with a positive level of self-esteem.
Alveolar Cleft Grafting
Reconstruction of the alveolar cleft and closure of concomitant oral nasal fistulae are the primary responsibilities of the oral and maxillofacial surgeon. There are several clinical indications to repair this defect. Placing bone into this site provides a substrate for the eruption of the proximate teeth. Separating the mouth from the nose eliminates the embarrassing and unpleasant flow of fluids from the mouth to the nose and visa versa
Reconstruction of the nasal aperture provides support for the alar base and a solid foundation for future nasal reconstruction. The effect of anterior oral nasal communications on speech is not clear. However, there is no doubt that large palatally placed defects produce a hypernasal resonance that improves after surgical closure. Finally, patient and family perceive a great psychological lift when the left is finally completely eliminated (Figures 2A, B).
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There is no doubt that large palatally placed defects produce a hypernasal resonance that improves after surgical closure. |
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Figures 2 A, B. Illustration depicts the alveolar cleft before and after grafting (AAOMS Surgical Update). |
When present, the alveolar cleft is a funnel-shaped defect that extends from the nasal aperture to the alveolar crest (Figure 3). Typically, it is lined with an epithelial tract that extends from the nasal cavity to the mouth. Often this soft tissue fistula extends into the labial vestibule and onto and through the hard palate. Invariably, the bony defect is considerably wider than the clinical examination suggests.
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| Figure 3. Clinical photograph of typical alveolar cleft |
Figures 4A, B. Illustration and surgical photograph of bone graft being placed into alveolar cleft |
The goal at surgery is to partition the mouth and nose. Flaps are elevated, which permits direct closure of the nasal mucosa, and allows placement of the bone graft and complete closure with a "water tight" layer of oral-attached mucosa. While various homografts and alloplasts have been used for this purpose, the material of choice is autologous bone. This graft is harvested from the ileum, calvaria, mandible, tibia and rib (Figures 4A, B). In our experience hip grafts are performed in over 90 perfect of cases.
The timing for alveolar cleft reconstruction is both critical and controversial. Some teams advocate early grafting (before age two). Typically that approach involves onlay grafts of rib. This technique often falls short in bone stock and is reported to have a negative effect on facial growth. The mainstream thinking today is that this graft should be performed between the ages of six and nine (Figures 5 A,B,C,D).
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Figures 5 A-D. Radiographs and clinical picture depicting sequential maturation of grafted alveolar cleft. |
The decision regarding when to graft depends on the regional dental development. When a useful lateral incisor is present or a central incisor may be jeopardized if it erupts or is orthodontically guided into the cleft site, the procedure is done early (age six). When the lateral incisor is absent then the general rule is to allow the cuspid root to develop 50 percent of its root length and then graft. Prior to grafting, the field should receive a dental prophylaxis.
Late grafting is performed when the dentition has already erupted. This represents a failure in the system. As time passes, the interproximal bone on the cleft side is lost, creating significant periodontal bone loss and early tooth loss. In addition, the lining mucosa becomes hyperplastic and difficult to mobilize and suture. After grafting. The orthodontist develops proper arch form and monitors the eruption of teeth adjacent to the grafted cleft. Occasionally the cuspid requires surgical exposure and orthodontic traction, and from time to time, attached tissue grafting is indicted (X-ray series).
Cleft Dental Gap
In about 50 percent of cases there is a congenitally absent lateral incisor. When this occurs there are several treatment alternatives. The first is to allow the erupting cuspid to lateralize; and when orthodontics is completed, this tooth can undergo odontoplasty. Another approach is to allow the cuspid to erupt into its proper anatomic position while maintaining the lateral edentulous position. When growth is completed, an osseointegrated implant can be placed. This is a technique that was introduced by Frank Verdi and is, perhaps, the favored approach, as it maintains proper arch form and tooth mass, thus providing support for the overlying facial soft tissues (Figures 6A, B). Care must be taken that there is adequate projection of the graft. If not, lateral bony augmentation must be performed prior to implant placement.
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Figures 6 A, B. Pre- and post-operative radiographs of young man who underwent late alveolar bone grafting and secondary osseointegration. |
A final approach is to construct a fixed anterior dental bridge. This requires preparation of healthy teeth in young individuals and is the least preferred solution.
Facial Growth Abnormalities
Deficient midfacial growth is the hallmark of CLPD. While sometimes mild, growth deficiency in all planes of space characterizes these patients. At times, mandibular growth is excessive. Often, however, a pseudo prognathic appearance results from the overclosed, counterclockwise-rotated position of the mandible occluding with a vertically deficient maxilla. As discussed earlier, this hypoplasia is only minimally related to the intervention and secondary scar formation. Indeed, a study of totally unrelated clefts in Sri Lanka demonstrated wide-open clefts in patients with normal facial proportion and development. The approach to this problem is multifaceted and is dependent on the degree of deformity and the age of the patient.
In the young patient, five to ten years old, the transverse and anterior posterior deficiency of the maxilla can be approached with a nonsurgical orthopedic widening and protraction of the midface. While still a subject of study, this approach seems to have some control over excessive mandibular growth, which would magnify the jaw-to-jaw discrepancy. In a cooperative patient this technique is exciting and dramatic.
Another opportunity to treat maxillary horizontal deficiency presents itself in the early adolescent phase. This approach involves surgically assisted osteodistraction. The final approach involves surgically assisted osteodistraction. The final approach involves formal orthognathic surgery (Figures 7AB, 8AB, 9A-D). This is indicated in approximately 25 percent of CLPD patients. Formal osteotomy for advancement is routinely and safely performed. At the time of this surgery, residual fistulas and additional bone grafting can be performed. In a review of 300 CLPD patients operated on at our center, 15 percent required orthognathic surgery. More than half of the cases required both upper and lower jaw corrections. It appears that the number of double jaw cases is declining, which may be a consequence of aggressive growth enhancement by the orthodontists on our team with a secondary containment of compensatory mandibular excess.
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Figures 7 A, B. Pre- and post-operative photographs of patient who underwent Le fort 1 osteotomy, iliac grafting and genioplasty. |
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Figures 8 A - D. This patient underwent double jaw surgery along with fistula closure and bone grafting. Future implant placement is planned. |
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Figures 9 A - D. 19-year-old with BCLPD treated with jaw advancement and grafting. |
Conclusion
Oral and maxillofacial surgery for patient with CLPD is both challenging and rewarding. It gives the surgeon the unusual and special opportunity to follow patients from birth through adulthood. By combining the knowledge and skills obtained in dental education with a surgical expertise developed while performing major and minor oral surgery, today's oral and maxillofacial surgeons find themselves working as an integral part of a team, contributing knowledge and skill in a meaningful way.
The literature contains numerous articles on cleft lip and palate management. Its review is clearly beyond the scope of this article. The reader is referred, however, to the text Facial Clefts and Craniosynostosis, edited by Turvey, Vig and Fonseca and published by W.B. Saunders in 1996. This is a current and comprehensive work.
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