Current concepts for short-term and long-term care
Clefts of the lip and palate are considered to be the most common congenital anomalies, affecting approximately one in 750 newborn babies. Patients born with these anomalies require the combined short-term and long-term care of several specialists. They may require a number of surgical procedures and other interventions, from infancy through their teens and early adulthood, to provide them with the necessary functional and aesthetic habilitation. The goals for treatment of such patients include restoration of facial appearance and oral function; improvement of dental skeletal and occlusal relationships; and improvement of speech, hearing, and psychosocial status.
The concept of a team approach for the treatment of patients with cleft lip and/or cleft palate and other craniofacial anomalies has been well-recognized and widely accepted. As a matter of fact, management by a dedicated team of specialists is considered to be the standard of cleft care. Members of the team are responsible for the longitudinal evaluation and coordinated care. Such comprehensive care is designed to meet each patient’s multiple and complex needs, as well as the needs of their families.
Teams provide interactive encounters between professionals representing a diverse variety of disciplines. Team members meet regularly to communicate, collaborate, and consolidate knowledge. From these deliberations, plans of short-term and long-term care are established based on each patient’s individual needs and the team’s personal philosophy and protocols. It is the responsibility of each team to maintain careful and appropriate longitudinal records, including photographs, casts, x-rays and scans. (Figure 1). Outcomes of care should be reviewed periodically, and all collected data should be carefully studied and analyzed to fully appreciate short-term and long-term results of treatment. Following critical analysis of results, protocols and techniques should be reevaluated, modified, changed, or improved accordingly.
Parameters of Care
Existing parameters for evaluation and treatment of patients with cleft lip and cleft palate, and other craniofacial or maxillofacial anomalies—including the guidelines established by the American Cleft Palate– Craniofacial Association—are based on fundamental principles regarding the optimal treatment of such patients, regardless of the specific type of the deformity. These guidelines provide an excellent framework of care and include recommendations regarding composition and functions of interdisciplinary teams, and longitudinal patients’ evaluation and treatment from the neonatal period through infancy, adolescence, and adulthood.
The establishment of a large number of cleft/craniofacial teams over the last 20 years resulted in a dramatic improvement in cleft care. This is due to improved knowledge and experience of health care professionals, the appropriate coordinated care, and cooperation among specialists, with close monitoring and analysis of results. Superior multidisciplinary care management is thus provided; protocols of care and surgical techniques are adjusted and refined; and ultimately, the long-term results are superior. An additional reason for this significant improvement of outcomes is the accumulated body of knowledge that is widely disseminated through presentations and publications, which are based on prospective and retrospective studies.
Unfortunately, despite these advances, a number of patients still receive suboptimal care, because they are not referred to a local team in a timely fashion and follow-up appointments are not kept as needed. Most importantly, funding is limited, and several necessary services—including revisions of surgical procedures, dental and orthodontic care, speech therapy, and psychological care, when needed—are still either inadequately funded or not covered at all by various state agencies and third-party payors.
Timeline of Care
Patients with clefts should be referred to a craniofacial team as soon after birth as possible. A timely, complete evaluation is necessary to fully appreciate the extent of the deformity, the general condition of the patient, and the possible presence of other medical conditions.
A personalized, detailed short-term and long-term plan of care is then formulated in consultation with the team members. This plan, including feeding instructions, should be communicated to the family and discussed in detail.
The value of preoperative orthopedics with both passive and active devices in the final outcome of lip repair is still debated. We have incorporated a passive preoperative treatment protocol in our preoperative protocols of care. The rationale for this approach is to reposition the maxillary/ alveolar segments in a more anatomically correct position before the lip repair. With improved skeletal relationships, the surgeon is given the opportunity to repair the lip and correct the associated nasal deformity, under less tension and under more optimal conditions. When possible, a periostoplasty can be performed simultaneously as well.
In patients with bilateral clefts of the lip and palate, the protruding premaxilla and prolabium make successful lip repair an even greater challenge. Several techniques have been described to reposition the premaxilla preoperatively. In our center, we use a premaxillary position appliance fabricated by our prosthodontists (Figure 2). This appliance is extremely helpful for repositioning of the premaxilla in a more favorable downward and backward movement. Its cost is minimal, and the use of pins and other invasive devices is not necessary. We have used this modality for almost 20 years and have observed a significant improvement of soft tissue and bony relationships preoperatively, without any negative effects on facial growth and development. Several weeks of treatment with regular adjustments is necessary to achieve optimal repositioning of the premaxilla and improved alignment with the maxillary segments prior to lip repair (Figure 3).
Correction of the coexisting nasal deformity has been incorporated with the lip repair. Several attempts have been made to manipulate the position and shape of the lower alar nasal cartilages preoperatively, and to lengthen or stretch the short columella in bilateral clefts. We have incorporated this principle in our preoperative care as well. We currently use a nasal alveolar molding device, which is incorporated in the intraoral plate (Figure 4). Several weeks of treatment and regular adjustments are needed to "mold" the alar cartilages in a more favorable position, thus facilitating the surgical correction of the nasal deformity and assisting in optimizing the surgical outcome.
Drawbacks from the use of this protocol, as with the use of other devices, include the additional expenses for material and personnel time, the need for additional visits for adjustment, the need for compliance, and the possible delay of the surgical procedure by some weeks. These drawbacks, however, are definitely offset by the gains of a superior outcome of the lip and nose, and the subsequent reduction in the number of surgical revisions—including total elimination of lip adhesions—prior to definitive lip repair.
Cleft Lip: Protocols of Care
The objectives of primary repair of unilateral and bilateral clefts of the lip are to establish anatomy and symmetry of the upper lip and nose, and to improve form and function. Criteria for a successful cleft lip repair include:
accurate skin, orbicularis oris muscle, and mucous membrane junction without undue tension;
slight eversion of the upper lip;
appropriate lip length;
symmetry of all lip elements, including the vermilion and the vermilion border;
minimal scarring; and
simultaneous correction of the nasal deformity, including nostril symmetry and tip projection.
Currently, unilateral cleft lips are repaired around the third month of age, and bilateral cleft lips are repaired around the fifth month of age, as long as the babies are otherwise healthy and can tolerate general anesthesia.
Unilateral clefts of the lip are primarily repaired with the rotation and advancement technique or its modifications. For patients with very wide clefts, and in cases where the lip is very short on the cleft side, I still use a modified triangular flap technique to avoid a short lip. After appropriate and detailed marking of all important landmarks of the lip and nose, extensive dissection of all layers of the lip, including the skin, orbicularis oris, and mucosa, is performed. It is extremely important to fully release all muscle fibers from their abnormal attachments prior to the repair. Attention is then turned to the nose. Some authors recommend wide undermining of the entire skin envelope of the nose.
I prefer a more conservative dissection of the tip area, completely undermining the skin and lining from the lower alar cartilage. The repair starts by repositioning and stabilizing the alar cartilages in a symmetric position, and by repairing of the nostril floor. We then proceed with the layered lip repair, with careful approximation of all layers, including mucosa, muscle skin, and vermilion. When possible, the dry and moist portions of the vermilion should be also aligned (Figure 5).
Patients with bilateral clefts present with a significantly more difficult deformity, and the results from repair have for years been clearly suboptimal. In recent years, several additional principles have evolved and are still evolving. The final results are becoming better and better, the nasal deformity is addressed during the same setting, and the need for revisions has been reduced. The lip repair is no longer staged but is carried out in one stage and after preoperative repositioning of the premaxilla in as favorable a position as possible. Both sides are repaired simultaneously, and every effort is made to align all lip elements and to provide symmetry at one setting. The orbicularis oris fibers are extensively mobilized from their position in the lateral lip segments and are sutured in the midline. The lower alar cartilages are dissected from the soft-tissue envelope through a combined approach from the prolabium, lateral, and medial intranasal incisions. They are approximated and sutured together from the dome to the medial cruses. If the alveolar segments are well-aligned and approximated, a periostoplasty is also performed. All layers of the lip are repaired and sutured without tension (Figure 6).
Cleft Palate: Protocols of Care
The primary objectives of cleft palate repair are to establish the anatomy of the hard and soft palate as close to normal as possible, to provide an adequate mechanism for velopharyngeal function for speech, and to improve middle ear function. Regardless of the surgical technique used, the surgeon should plan to provide a palate of adequate length, reconstitute the muscular velopharyngeal sling, and approximate the tissues without tension, to achieve the best functional results.
Timing for cleft palate repair is still a controversial issue due to the potential negative effects of surgery on facial growth. Patients treated with delayed palatal closure protocols have ended up with near-normal facial appearance but significant speech problems, which are very difficult or almost impossible to correct. For these reasons, an earlier palatal repair was advocated about 20 years ago. Currently, there is enough clinical and experimental evidence to support the concept that early cleft palate repair (within the first year of life) results in better speech, and most authors agree that proper speech production should be given priority.
Nevertheless, surgeons should always be aware of the potential detrimental effects of palatoplasty on facial growth. Timing for palatoplasty should be individualized and based not only on age, but on individual anatomical findings as well. Currently, I favor the two-flap palatoplasty technique for complete clefts and the Furlow double-opposing Z-plasty for clefts of the secondary palate. Regardless of choice of technique, however, adherence to basic surgical principles is always important to the outcome. Careful palatal-flap dissection techniques should be used, causing as little tissue trauma as possible. If adequate tissue for repair of the nasal floor is not available, unilateral or bilateral mucoperiosteal flaps from the vomer should also be used. The continuity of the muscular sling should be established, and the palatal flaps should be approximated in the midline without undue tension; we are very careful to avoid leaving large, raw surfaces laterally, since they will ultimately heal with additional scarring, which will further impair facial growth (Figure 7).
Several patients with cleft lip and/or cleft palate present with middle ear pathology. Audiologic evaluation should be performed preoperatively. When there is indication, a full microscopic otologic evaluation is recommended. Pressure-equalizing tubes, if needed, should be inserted at the same setting.
Additional Follow-Up Procedures
Following cleft lip and cleft palate repair, patients are evaluated periodically by the various team members. Oral hygiene and dental care should be promoted, and psychosocial evaluation and treatment should be available, as needed.
Complete perceptual speech evaluation should be performed by an experienced speech pathologist as soon as the toddler is able to cooperate. Anatomic and physiologic speech evaluation should be performed as needed to obtain detailed information and prescribe treatment with speech therapy, but also with surgical procedures, when indicated. Follow-up evaluations should be continued until normal or near-normal speech is achieved through speech therapy, surgery, or a combination of both.
The role of the team orthodontist is no longer limited to the evaluation and monitoring of facial growth and orthodontic treatment at the stage of mix dentition. Since the development of distraction osteogenesis techniques, some patients with clefts and other craniofacial anomalies are treated at a relatively young age. The contribution of the team orthodontist is extremely valuable in the planning and perioperative management of these patients, as well as with other patients requiring other orthognathic procedures of the maxilla and/or the mandible.
Reconstruction of the alveolar process and the hypoplastic maxilla by secondary bone grafting of the residual bony cleft and simultaneous closure of a coexisting oronasal communication in the transitional dentition stage is a time-honored procedure. Close cooperation with the orthodontist is a prerequisite for success. Preoperative orthodontic preparation and postoperative treatment result in superior reconstruction, function, and appearance.
Despite technical advances and simultaneous correction of the nasal deformity at the time of lip repair, a number of patients still require a secondary procedure to restore symmetry and improve function. Such procedures should be individualized and include, in addition to the complete correction of the nasal deformity, a septoplasty and bilateral inferior turbinectomies, as needed.
Every effort should be made to restore function and appearance for patients with clefts and allow them to grow and become normal adults. The vast majority of these patients have all the potential to become normal, productive citizens; they deserve and should expect complete habilitation from their congenital anomalies.
Superior results can be achieved with management by a dedicated team of experts who will be able to address and correct all issues related to the deformity. Appropriate and extensive evaluation, short-term and long-term planning with optimal timing for each procedure, and close cooperation among various members of the cleft team is necessary for the optimal outcome.
Mimis Cohen, MD, FACS, FAAP, is professor and chief of the Divisions of Plastic, Reconstructive and Cosmetic Surgery, The Craniofacial Center, The University of Illinois at Chicago, and John Stroger Jr. Hospital of Cook County. He can be reached at [email protected]