Systematic review
The scientific knowledge base for treatment of patients with cleft lip, alveolus and palate
Systematic review
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In this review we present the evidence base for the effectiveness of interventions in treatment and follow-up of patients with cleft lip, alveolus and/or palate.
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Key message
In this review we present the evidence base for the effectiveness of interventions in treatment and follow-up of patients with cleft lip, alveolus and/or palate. We searched for systematic reviews and prospective primary studies with a simultaneous control group.
We found no scientific studies that met the inclusion criteria for the themes examination of or composition, duration and intensity of treatment for patients with cleft lip, alveolus and/or cleft palate. We did not identify any studies of measures that satisfied our design requirements for patients with velocardiofacial syndrome. We included 1 systematic review and 46 primary studies within the categories treatment and follow-up. Of these 39 studies were classifieds as treatment (mostly surgery and surgery-related measures) and 1 systematic review and 7 primary studies as follow-up (feeding, speech and otitis media).
The quality of the evidence of the results of different forms of pre-surgical orthopaedics, lip-alveolus-palate closure and of correction of nasal septal deformity is too low to draw any conclusions. For secondary surgical procedures pharyngeal flap may be similar to sphincterplasty for velopharyngeal insufficiency, but the evidence for this is of low quality. For the other five comparisons of measures within secondary surgery, the quality of the evidence is too low to draw any conclusions. The results from the comparisons of interventions within orthodontics, speech training, otitis media and feeding methods are also too uncertain to draw any conclusions.
The evidence base for current practice in treatment and follow-up of children and adults with cleft lip, alveolus and/or palate is low or very low. This does not mean that current practice is poor. It means that existing research is too uncertain to provide good evidence for conclusions about the relative effectiveness of different interventions, sequencing and optimal times for cleft lip and palate surgery, of effectiveness of interventions for maxillary protraction and of the different follow-up and habilitation interventions.
Summary
Background
In connection with their disciplinary review of treatment for patients with cleft lip, alveolus and/or palate, the Norwegian Directorate of Health commissioned a systematic review of the evidence base for the effectiveness of treatment interventions of patients within this target group. Our task included finding and summarising research on the effects of interventions within all treatment and treatment related aspects concerning children with cleft lip, alveolus and/or palate. Also, the Norwegian Directorate of Health wanted us to summarise the research on interventions for children born with velocardiofacial syndrome.
Method
The systematic review is performed in accordance with the handbook of the Norwegian Knowledge Centre for the Health Services. We conducted a thematically broad search for references about cleft lip, alveolus or palate or velocardiofacial syndrome. The search was then limited to systematic reviews and primary studies with a prospective, controlled design. We had no language restrictions. The search was done in the Cochrane Library, MEDLINE, EMBASE, CINAHL, ERIC and Norart (a Norwegian database of Nordic journal articles). The methodological quality of the included articles was independently assessed by two persons. Likewise, two persons decided which data to extract and present in result tables. In those instances where data were presented for different follow-up periods, we emphasized the last reported data. Except for a meta-analysis of results from one comparison, we did a descriptive summary of data.
Results
The search generated 2511 references to potentially systematic reviews and primary studies. Of these, 46 primary studies (29 randomised controlled trials, 11 controlled before and after and 6 quasi randomised studies) and 1 systematic review were included. No studies within the themes interdisciplinary study or composition of team treatment, duration and intensity of treatment were identified. No studies that fulfilled the inclusion criteria for design of children with velocardiofacial syndrome were identified.
We included 5 studies that examined the effect of pre-surgical orthopedics before surgery. Within the category surgery and surgery-related measures, we included 5 studies of surgery for cleft lip, 16 studies for surgical closure of cleft palate, 1 study of immobilization of the child’s arms after palate surgery, 3 studies for the closure of the alveolar bony cleft, 1 study for the correction of nasal deviation, 1 study of maxillary distraction and 6 studies of different types of secondary operations. Within the category follow-up and habilitation, we included 1 systematic review and 1 primary study on effectiveness of various interventions for feeding of the child, 5 studies of speech therapy techniques, and 2 studies of the effect of ventilation tubes for otitis media with effusion.
Most of the studies were judged to be of low quality. If a study has major methodological weaknesses and there is only one study with few participants in the comparison, the quality of the evidence for the estimate of effect on that outcome is very low. We cannot determine whether the intervention has affected the outcome or not. We judged this to apply to all comparisons, except for one, in which we judged the quality of the evidence of the results as low: pharyngoplasty compared with sphincterplasty for velopharyngeal insufficiency. When the quality of the evidence is low, it is likely that further research will have an important impact on our confidence in the results and that the results may change.
Discussion
The aim of this systematic review was to find prospective controlled studies within the field examination, treatment and follow-up of persons with cleft lip, alveolus and/or palate, or persons with velocardiofacial syndrome. We found no studies that compared interventions within the category interdisciplinary examination or the category composition of team treatment, duration and intensity of treatment. Neither did we find a single study which met the inclusion criteria of the interventions targeted at persons with velocardiofacial syndrome. We found several studies of treatment of patients with cleft lip, alveolus and/or cleft palate, but almost all tested various interventions. This means that the evidence base for each treatment is sparse.
The research that has been done reflects that there are a variety of relevant and current issues in this field. However, patients are different in terms of cleft type, shape and size, while some has a syndrome diagnosis as well. Therefore, it is difficult to recruit enough patients with the same characteristics to carry out a prospectively controlled research project. Because patients must be followed for a long time to assess the success of treatment, follow-up is also very challenging.
Conclusion
Interdisciplinary team examination
- We found no studies satisfying the inclusion criteria for this topic.
Treatment
- Pre-surgical orthopaedics: The quality of the evidence for the effect of different forms of preoperative forming is too low to determine whether pre-surgical orthopaedics is of importance for the outcomes child’s weight, dimensions of the alveolar arch, relationships between the alveolar segments, and the occlusion of deciduous dentition, appearance, voice, nasal form and septal deviation.
- Closure of cleft lip, alveolus and palate: The quality of the evidence for the results of all comparisons is too low to draw any conclusions.
- Correction of the nasal form: The quality of the evidence of the result of the comparison of Surgicel versus no use of Surgicel is too low to draw any conclusion.
- Secondary surgery: It is possible that there is a small or no difference for velopharyngeal insufficency between a pharyngeal flap and a sphincter pharyngoplasty. The quality of the evidence for the results of the other comparisons is too low to draw any conclusions.
- Orthodontics: The quality of evidence of the result from the comparison of repeated rapid maxillary expansion and contraction to one week rapid maxillary expansion before activation of the 2-hinged protraction device is too low to draw any conclusions.
Follow-up and habilitation
- Feeding: The evidence of no difference between using hard or soft bottles, between using pre-surgical orthopaedics or not or that breastfeeding is a little better than feeding by spoon regarding the weight of the child is of very low quality.
- Speech therapy: The quality of the evidence is too low to decide whether phonological speech therapy is better than phonetic therapy for the mean length of necessary voice therapy, whether it is more effective for the linguistic level or mother’s communication with the child that the child’s mother participates in the speech training or whether training in summer camp is no different from training spread over a whole year.
Otitis media with effusion
- The quality of the evidence of whether hearing was improved with a ventilation tube for otitis media with effusion is too low to draw any conclusions.