- Email: [email protected]
The Eurocleft Project 1996–2000: overview
Journal of Cranio-Maxillofacial Surgery (2001) 29, 131–140 # 2001 European Association for Cranio-Maxillofacial Surgery doi:10.1054/jcms.2001.0217, available online at http://www.idealibrary.com on
The Eurocleft Project 1996–2000: overview William C. Shaw,1 Gunvor Semb,1,2 Pauline Nelson,1 Viveca Brattstro¨m,3 Kirsten Mølsted,4 Birte Prahl-Andersen,5 Karsten K.H. Gundlach6 1
Department of Oral Health and Development, University Dental Hospital of Manchester, UK; 2National Hospital and Dental Faculty of Oslo, Bretdvet Kompetansesenter, Norway; 3Eastman Institutet, Dalagatan 11, Stockholm, Sweden; 4Cleft Palate Department, Dental Clinic, Speech And Hearing Institute, Hellerup, Denmark; 5Department of Orthodontics, Academic Centre for Dentistry Amsterdam, Amsterdam, The Netherlands; 6Department of Maxillofacial and Facial Plastic Surgery, University Hospital, Rostock, Germany SUMMARY. Introduction: The original Eurocleft project, a European intercentre comparison study, revealed dramatic differences in outcome, which were a powerful stimulus for improvement in the services of respective teams. The study developed a preliminary methodology to compare practices and the potential for wider European collaboration including opportunities for the promotion of clinical trials and intercentre comparison was recognized by the European Commission. Therefore, the project: ‘Standards of Care for Cleft Lip and Palate in Europe: Eurocleft’ ran between 1996 and 2000 and aimed to promote a broad uplift in the quality of care and research in the area of cleft lip and palate. Results: The results of the 1996–2000 project include: . a register of services in Europe, with details of professionals and teams involved in cleft care, service organization, clinical protocols and special facilities for research; . a set of common Policy Statements governing clinical practice for European cleft teams, Practice Guidelines describing minimum recommendations for care that all European children with clefts should be entitled to and recommendations for Documentation governing minimum records that cleft teams should maintain; . encouraging initial efforts to compare outcomes (results) of care between centres. A survey showed a wide diversity in models of care and national policies as well as clinical practices in Europe. Of the 201 centres that registered with the network, the survey showed 194 different protocols being followed for only unilateral clefts. Conclusion: Cleft services, treatment and research have undoubtedly suffered from haphazard development across Europe. Attainment of even minimum standards of care remains a major challenge in some communities and both the will to reform and a basic strategy to follow are overdue. It is hoped that the Eurocleft Consensus Recommendations reached during the present project will assist in improving the opportunities for tomorrow’s patients. It is also hoped that the collaborative research now beginning under the European Commission’s Framework V Programme will provide a focus for European researchers wishing to improve understanding, treatment and prevention of clefts of the lip, alveolus and palate in the years ahead. # 2001 European Association for Cranio-Maxillofacial Surgery
The ultimate goal of the proposed project was to improve the effectiveness and efficiency of care for European children with clefts of the lip, alveolus and/ or palate. Following publication of an European intercentre comparison study (Shaw et al., 1992a, Mølsted et al., 1992; Mars et al., 1992; Asher-McDade et al., 1992; Shaw et al., 1992b), the potential for wider European collaboration was recognized. The original Eurocleft study developed a preliminary methodology to compare practices, and the dramatic differences in outcome revealed were a powerful stimulus for improvement in the services of respective teams. Furthermore, the shared experience of open evaluation, the exchange of views and the identification of wider opportunities for research promoted multicentre collaboration and a series of clinical trials.
INTRODUCTION This paper describes the concerted action project: ‘Standards of Care for Cleft Lip and Palate in Europe: Eurocleft’ funded by the European Commission under its BIOMED II and INCO COPERNICUS Programmes. The project, aimed at developing a network of cleft teams within the European Union and the European Economic Area member states, ran for 3 years between 1996 and 1999. Teams from Central and Eastern European states joined the network via support under INCO for a further year. Support in the form of grants: European Commission BIOMED II Programme, Contract No. BMH4-CT95-0402; European Commission INCO COPERNICUS Programme, Contract No. IC 15-CT98-0301. 131
132 Journal of Cranio-Maxillofacial Surgery
The BIOMED II and INCO project aimed to recreate this approach on a pan-European scale in the hope of promoting a broad uplift in the quality of care and research. A Steering Group of five individuals from cleft teams in Denmark, the Netherlands, Norway, Sweden, and the UK was commissioned to undertake the work. The aim was the attainment of four measurable objectives: .
. .
.
the production of a Register of Services in Europe, with details of professionals and teams involved in cleft care, service organisation, clinical protocols and special facilities for research, a set of common Policy Statements governing clinical practice for European cleft teams, a set of common Practice Guidelines describing minimum recommendations for care that all European children with clefts should be entitled to, and a set of common recommendations for Documentation governing minimum records that cleft teams should maintain.
BACKGROUND Clefts of the lip, alveolus and/or palate affect between 1 per 500 to 1 per 700 live births in Europe (World Health Organization, 1998). Highly specialized corrective surgery in the early months of life is necessary to improve function and appearance. Subsequent impairment of facial and dental development, speech and hearing are common and may be accompanied by social-psychological maladjustment. A large number of specialist services is necessary if the child’s potential is to be maximized: neonatal nursing and community nurse services, surgery, speech, hearing, otolaryngology, dentistry, orthodontics, psychology and genetic counselling. Approaches to cleft care in Europe vary considerably between countries. Within the Nordic countries there is a tradition of centralization of services. Other countries such as France, Germany and Italy (and until recently, the UK) provide cleft care via large numbers of local services who may see but a few patients with clefts. Consequences of poorly organized care The original Eurocleft intercentre study suggested that decentralized care by low volume operators may be associated with inferior outcomes. The ultimate consequences of this are greater hardship for patients and families and increased health costs associated with additional operations and ancillary care. The relatively low case load and independent development of cleft services in parts of Europe make it likely that varied standards are being achieved. The extent of the disparity in outcomes revealed in the previous study was dramatic. The need for a maxillary
osteotomy differed between the best and worst services by a factor of 8 (6% vs 48%; Shaw et al., 1992c). Furthermore, care in the worst service was much more complex and expensive(!): Thus, significant improvements could be achieved at no extra cost through local recognition of the need to change practices. This is particularly important at a time when budgets for health care across Europe are under severe pressure. Lack of evidence-based practices As cleft lip, alveolus and/or palate is a birth anomaly with relatively low frequency and numerous anatomical subtypes, critical studies including large samples are rarely achieved. In effect it is an ‘orphan condition’ and present care is far from ‘evidence based’. Not surprisingly, the diversity of approach to cleft treatment is great. The previous Eurocleft study recommended that intercentre evaluation was crucial to the maintenance of standards and encouraged refinement of methodology for comparative study. By rigorously managed intercentre evaluation, clinicians might compare outcomes for different protocols of care and identify harmful practices. Where no important differences emerge, balanced judgements could then be made on the selection of future protocols based on factors such as the burden on patients and their families, simplicity of treatment and economic cost. The study also proposed multicentre prospective trials as a way forward in refining treatment (Shaw et al., 1992c). As a preliminary step in the present project, we undertook a systematic review of the literature concerning treatment for unilateral and bilateral cleft lip, alveolus and palate with cephalometric measurement of the face as an outcome. This systematic review was planned according to the methodology of the Cochrane Collaboration (Chalmers and Altman, 1995). The search strategy included: . . . .
searching the electronic databases of Medline and Embase from 1966–1997, hand-searching the Cleft Palate-Craniofacial Journal from 1964–1997, searching the Cochrane Trials Register, and following up relevant citations in the papers read.
In all, 5474 titles were identified of which 189 reports were found ‘relevant’. Of these, only three described clinical trials. Robertson and Jolleys conducted two small randomized control trials of primary surgery in the 1960s. One, involving two groups of 20 cases where one group’s anterior palate closure was delayed until age 5, found no benefit for dentofacial growth (Robertson and Jolleys, 1990). In the second study a similar sample was randomized in respect of alveolar bone grafting at the time of lip repair. Follow-up revealed a detrimental effect on facial growth in the grafted group (Robertson and Jolleys, 1968). In a controlled clinical trial alternated
The Eurocleft Project 1996–2000: overview 133
palate repair with or without intravelar veloplasty in 51 subjects with a broad range of palatal cleft types (Marsh et al., 1989). Speech evaluations were made at a 2-year follow-up. No difference in outcome was detected, but the procedure including intravelar veloplasty required a significantly longer operating time. All other available research reports have been retrospective, frequently at case series level. A review of these has also been undertaken and we concluded that comparison between reported outcomes is unreliable because of the invariable likelihood of bias (Semb and Shaw, 1998). This disappointing level of clinical evidence in the craniofacial field necessary for evidence-based medical treatment reflects the fact that funding agencies have hitherto been disinclined to fund, and researchers disinclined to initiate randomized control trials, because of their duration. Recruitment is lengthy owing to the scarcity of cases, and relevant outcomes (e.g. speech or facial growth) can only be studied several years after surgery. The infrastructure that would make such studies possible has until now been lacking, both in Europe and elsewhere. Uncoordinated research in the basic sciences The aetiology of clefts has a definite element of inheritance and could thus be amenable to gene mapping. But in this area of basic medical science there has only been limited progress. For example, the gene for one cleft palate syndrome has been mapped for an extended Icelandic family (Forbes et al., 1995) as has the gene for the Berry–T. Collins– Franceschetti Syndrome (Treacher Collins Syndrome Collaborative Group, 1996). Advances in the more complex genetics of non-syndromic clefts require concertation between highly specialised molecular genetics laboratories, epidemiologists and teams with extended recording of pedigree in their patients. These resources do exist in Europe but are not well linked at present.
MATERIAL AND METHODS A three stage methodology was adopted for this project: . . .
creation of a network, development of consensus recommendations, survey of current services.
Creation of the network Each member of the Steering Group had responsibility for linking with cleft professionals from a specific group of countries where they had particular expertise in terms of language or experience. This strategy facilitated a nomination process amongst the
registered teams who were asked to suggest individuals from each of their countries to act as a key delegate and provide a general link to the network. The criteria for nomination included the requirement that the individual should be an expert in the field of cleft care with a good knowledge and general overview of cleft care organization in their country. National representatives were chosen on the basis of nomination by their national colleagues and a spread of clinical speciality in order to secure a multidisciplinary breadth to the network. During the project some representatives were replaced and others duplicated due to particular local factors (Table 1). Development of consensus recommendations It was evident from previous investigations that the quality of cleft care would vary enormously between one region and another arising from organizational or surgical factors. It was considered inappropriate to prescribe specific technical protocols on the timing, sequence and design of surgical procedures, orthodontic treatment, speech therapy and so forth as many of these choices remain highly controversial and practices currently vary to a great extent across Europe. Resolving such issues would require the initiation of multicentred randomized trials and would need to await the establishment of better infrastructure and research capability across European teams. However, it was hoped that quality improvement of an organizational nature could be promoted by recommendations upon policies and practice guidelines. Therefore, the Steering Group and the national representatives forming the Eurocleft network came together in a series of meetings: As a result of the First Eurocleft BIOMED II European Workshop in Manchester, April 1998 (attended by national representatives from the fifteen EU member states with the addition of Czech Republic, Poland, Iceland, Norway and Switzerland), areas of agreement identified and minimum recommendations in the form of Policy Statements and Practice Guidelines were drawn up. In addition, initial discussions took place and an early draft on the issue of record taking was made. At the First INCO European Workshop in Manchester, February 1999 (attended by national representatives from 10 countries of Central and Eastern Europe: Bulgaria, Czech Republic, Estonia, Hungary, Latvia, Poland, Romania, Slovak Republic, Slovenia and Ukraine) the papers on Policy Statements and Practice Guidelines were finalized. It was proposed that quality improvements of surgical skills would best be achieved by the promotion of clinical audit. If the methodology for assessing outcomes could be defined and established by consensus, this would allow European centres to evaluate their own quality of care, compare it with that achieved by other centres, and to initiate local quality improvement. Thus, at the Second Eurocleft
134 Journal of Cranio-Maxillofacial Surgery Table 1 – National representatives of participating European countries Austria Belgium Bulgaria Czech Republic Czech Republic Denmark Estonia Finland France Germany Germany Greece Hungary Iceland Ireland Italy Italy Latvia Netherlands Netherlands Norway Poland Portugal Portugal Romania Slovak Republic Slovenia Spain Sweden Switzerland UK Ukraine
Hans Ka¨rcher Albert de Mey Youri Anastassov Jitka Vrtisˇ kova´ Ziva Mu¨llerova Anja Bau Siiri Hanstein Jorma Rautio Chantal Trichet Karsten Gundlach Charlotte Opitz Meropi Spyropoulos Gizella Reha´k O´lafur Einarsson Eamonn McKiernan Giovanna Garattini Ferruccio De Stefano Biruta Barkane Emmy Konst Anne Marie Kuijpers-Jagtman Rolf Tindlund Kazimierz Kobus Maria Joa´o Alves de Castro Anto´nio Capelo Lidia Boboc Irena Klı´ mova´ Vesna Kozelj Gonzalo Gonzalez-Landa Jan Lilja Wanda Gnoinski Liz Albery Leonid Kharkov
European Workshop, Manchester, June 1999 (attended by both groups of national representatives, Table 1), general principles governing record taking were agreed and consensus on the timing of records reached. These recommendations were finalized in the paper on Principles Governing Record Taking and Timing of Records. The guidelines were subsequently circulated to all registered centres of the project, national health authorities and governments. As it became obvious that many countries have not yet performed outcome studies the Steering Group organized 2-day Research Methodology Courses for registered centres of the network in February and October 1999. Both courses were well attended by participants from a spread of clinical specialities and European countries. The course content included: research design, measurement of outcome, sources of bias, practical management of research, implementation of research findings and in addition, practical sessions with the opportunity for participants to gain experience using established rating scales. The agreed European guidelines for cleft teams were also disseminated. Survey of current European services and research resources A preliminary register was compiled from a number of sources: cleft association membership lists, international symposia attendance lists and individuals identified by a literature search. The project was also promoted at the outset via an abstract published in
maxillofacial surgeon plastic surgeon plastic surgeon plastic surgeon orthodontist speech therapist maxillofacial surgeon plastic surgeon speech therapist maxillofacial surgeon orthodontist orthodontist orthodontist plastic surgeon orthodontist orthodontist plastic surgeon maxillofacial surgeon speech therapist orthodontist orthodontist plastic surgeon orthodontist paediatric surgeon orthodontist orthodontist maxillofacial surgeon paediatric surgeon plastic surgeon orthodontist speech therapist maxillofacial surgeon
relevant European scientific journals and through papers presented by individual Steering Group members at international meetings of cleft professionals. After an Initial Questionnaire was sent out to approximately 500 possible centres asking for general information about patient caseload, clinical specialities involved in the team, primary surgical protocol and research activity a total of 201 centres registered with the project from 30 European countries. There are also a number of centres elsewhere that received questionnaires but for whatever reason, decided not to participate. RESULTS Policy Statements and Practice Guidelines were drawn up, discussed and finalized in April 1998 and February 1999 (Appendices I and II). Principles Governing Record Taking and Timing of Records had been drawn up in April 1998, discussed in February 1999 and finalized in June 1999 (Appendix III). An European Register of Cleft Centres was assembled including a total of 201 centres from 30 European countries (Table 2). We are also aware of a number of centres elsewhere (including two in Russia) that received questionnaires but for whatever reason, decided not to participate, making the survey 40% representative. Survey Findings I: European diversity in models of care and national policies. The greatest challenge to
The Eurocleft Project 1996–2000: overview 135
the delivery of adequate services across much of Europe appears to be organizational. Compliance with key recommendations such as the creation of fully comprehensive teams with a sufficiently large caseload to optimize clinical experience and outcome evaluation (e.g. 40 new cases per year per centre, i.e. surgeon, orthodontist and speech therapist), is being achieved in a minority of countries. From the workshops in 1998/1999 it was clear that the greatest obstacles to progress were human, not economic. In some countries, policies concerning the provision of cleft care are well established. In Denmark, the provision of primary surgery by one surgical team has been formalized in law since 1937. More recently, in Norway, the long standing arrangement of two national centres was confirmed by a law stating that no care can be provided elsewhere unless requested by one of these cleft teams. In the Czech Republic, Latvia and Estonia, well established regional centres are continuing to provide comprehensive services following recent political and economic changes. In some circumstances, a move towards privatisation of healthcare is considered to present the risk of possible destabilization of established centres. At the other extreme, the concept of team care is still to be adopted. National representatives from Bulgaria, Greece, Italy, Portugal, Romania, Spain and Ukraine for instance, reported that much care was still provided by individual clinicians, working in isolation, and not in government funded centres. Variation in policies is just as apparent among EU states and the non-EU states of Central and Eastern Europe; in countries such as Bulgaria, Romania and Ukraine, services are still fragmented and difficulties in securing adequate clinical resources are reported.
Elsewhere, regionalization of insurance for example, in France and Germany, and regional health care autonomy as in Finland, are tending to favour decentralized care. Even where centres exist, they are often small. Indeed, there are several examples of two, three or four centres co-existing in the same city, eg. Antwerp, Athens, Barcelona, Berlin, Bern, Bologna, Brussels, Bucharest, Budapest, Innsbruck, Lille, Lisbon, London, Madrid, Milan, Oporto, Paris, Parma, Rome, Verona and Warsaw. Survey Findings II: European diversity in clinical practices. Involvement of a wide variety of surgical specialities in cleft surgery was reported (Table 3) but no attempt was made to distinguish the speciality responsible for primary repair of the cleft. The first five surgical specialities are listed according to the order reported by the teams. Those mentioned most often were plastic surgery: 94 (46.7%), maxillofacial surgery: 59 (29.4%), and paediatric surgery: 22 (10.9%). The clear lack of a sound evidence base for selecting treatment protocols, is reflected by a remarkable diversity of practices across Europe for the surgical care of just one cleft sub-type, unilateral complete cleft of lip, alveolus and palate. Seventeen possible sequences of operation to close the cleft are practised (Table 4). Though 86 (42.8%) of teams closed the lip at the first operation and the hard and soft palate together at the second, almost every other conceivable sequence appears to be practised somewhere. Thus the total number of operations taken to complete the closure of the cleft varied from one: 10 (5%), two: 144 (71.1%), three: 43 (21.9%) and four: 4 (2%). Around half the registered teams employ presurgical orthopaedics, of whom 67 (65%), use it
Table 2 – Countries covered by the European Register of Cleft Centres Austria Belgium Bulgaria Croatia Czech Republic
Denmark Estonia Finland France Germany
Greece Hungary Iceland Ireland Italy
Latvia Lithuania Moldova Netherlands Norway
Poland Portugal Romania Slovak Republic Slovenia
Spain Sweden Switzerland UK Ukraine
Table 3 – Specialities involved in cleft surgery Speciality
First speciality (%)
Second speciality (%)
Third speciality (%)
Fourth speciality (%)
Fifth speciality (%)
Plastic surgery Maxillofacial surgery Paediatric surgery Plastic/maxillofacial ENT ENT/phoniatrics Maxillofacial/ENT Paed/maxfac/plastic Paediatric/maxfac Plastic/craniofacial Plastic/paediatric Stomatology Craniofacial surgery Head/neck Neurosurgery Unspecified
46.7 29.4 10.9 3.0 2.0 1.0 0.5 0.5 0.5 0.5 0.5 0.5 0 0 0 4.0
31.0 49.3 6.8 2.0 2.7 0 0 0 0 0 0 0.7 0.7 0 0.7 6.1
21.4 60.0 1.3 2.7 5.3 0 0 0 0 0 0 0 0 1.3 1.3 6.7
6.7 73.3 3.3 3.3 6.7 0 0 0 0 0 0 0 0 0 0 6.7
14.3 57.1 14.3 0 0 0 0 0 0 0 0 0 0 0 0 14.3
136 Journal of Cranio-Maxillofacial Surgery Table 4 – Sequence of operations for the repair of unilateral complete cleft lip, alveolus and palate First operation
Second operation
Lip closure Lip closure Lip and hard palate closure Lip and soft palate closure Lip, hard and soft palate closure Lip closure
Hard and soft palate closure Soft palate closure Soft palate closure Hard palate closure
Lip and soft palate closure
Hard palate closure and gingivo-alveoloplasty Hard and soft palate closure Lip and hard palate closure Lip closure Soft palate closure Lip, hard and soft palate closure Lip and hard palate closure Lip closure
Lip and alveolar closure Soft palate closure Lip adhesion Lip and alveolar closure Lip adhesion Lip adhesion Hard and soft palate closure and alveoloplasty Lip and soft palate Lip adhesion Lip closure Total
Soft palate closure
Hard palate closure and alveolar bone grafting Lip closure Soft palate closure
routinely. Mostly passive plates were used by 74 (70%) and some teams also used the plate as a feeding plate. Not one single European team practices the same protocol as any other (except for those participating in the clinical trial described below). That is to say, 201 teams currently practice 194 different protocols for one cleft sub type. Survey Findings III: Consumer associations in Europe. Of the 28 European countries involved in the network, 18 have some form of organization for parents. Such groups vary in the degree of their activity, effectiveness and influence and take different forms. Some are functioning as officially established national associations whilst others are operating on a more informal basis. In Germany for instance, there are several small regional organizations and one major group which takes part in the annual meetings with the professional association. Denmark has a well established group for parents which has been functioning for over 25 years whilst in other countries such as the Ukraine a priority for the future is the creation of such a group. Interestingly in Bulgaria, the parent group is working to bring together national cleft specialists with a view to instigating an association for professionals.
Third operation
The challenge of improving quality of services Achieving optimal standards of cleft care across Europe remains an outstanding challenge. The basic principles of care described in the Policy Statements and Practice Guidelines (Appendices I and II) are hardly revolutionary, indeed they describe what most individuals (including those in clinical practice or
% 42.8 15.3 10.4 10.0 5.0 3.5
Hard palate closure
Hard palate closure and alveolar bone grafting
2.5
Soft palate closure Hard palate closure
Hard palate closure
Soft palate closure
2.0 2.0 1.5 1.0 1.0 1.0 0.5 0.5
Hard and soft palate closure Gingivo- alveoloplasty
Hard palate closure
0.5 0.5 100
government) would wish for their own children, regardless of country of birth. The potential strength of a European network is that examples of good practice can be more easily shared, challenges of a general nature can be more easily recognized and understood, and solutions that have been successful in one country can be applied elsewhere. The need for this is obvious if one considers the fact that today the variation between centres regarding sequence, technique and timing of cleft repair could hardly be greater! On the other hand, some obstacles, especially those arising from specific local circumstances and personalities, need specific local solutions. Among the difficulties mentioned were: . . . . . .
DISCUSSION
Fourth operation
personal egotism of individuals unwilling to discontinue the practice of treating a few children each year; competition between specialities for preeminence in the field, e.g. plastic-vsmaxillofacial-vs-paediatric-vs-ENT surgery; local pride, with every hospital, town or region desiring its own small team or wish to have local service; lack of clinical leadership; necessity for teaching hospitals to cover a spectrum of clinical practice; lack of responsiveness in the health authorities at local and national level.
This places a heavy responsibility on the national representatives of each country who have taken part in the Eurocleft process over the last 4 years. All of the above problems have confronted the UK in the recent past and a national review was instigated by the government Clinical Standards Advisory Group (CSAG, 1998). The review included a national survey that revealed that Britain’s fragmented, decentralized services were achieving a low standard of clinical care
The Eurocleft Project 1996–2000: overview 137
in some areas. As a result the Government instructed regions to provide care from a single regional centre. Each of these should have a fully comprehensive specialist team, typically with two to three surgeons, each responsible for not less than 40 new cases for primary surgery per year. In this instance, government interest has been essential in trying to improve services when voluntary methods failed (Bearn et al., 2001; Sandy et al., 2001; Sell et al., 2001; Williams et al., 2001). However, some early progress is already being reported by European national representatives. At the time of writing these include: .
.
.
.
Restructuring – in Hungary, the Netherlands and Slovak Republic unification of services has already reduced the number of centres and restructuring in Poland is under way. In Ireland a new unified regional centre has been established and there are hopes for a specialized centre in Greece. New associations – in Spain a new national association for professionals and parents has formed and there are similar plans in Portugal. In Austria the first multidisciplinary meeting of cleft professionals has been held and is set to become an annual event with a new national association to be founded in 2001. Similar efforts are taking place in France. Funding – in Estonia, orthodontic treatment with fixed appliances for children with clefts is now provided free, in Latvia extra funding for cleft services generally has been provided, and a similar announcement is expected in Poland. Registries – new national registries for clefts are being created in Belgium, Romania, Slovak Republic and Ukraine.
The challenge of improving research infrastructure Research activity Centres were given the opportunity to comment on the research interests and activities of their team, and any special resources for research such as databases, equipment, and collaboration with genetics laboratories. Some centres reported comprehensive listings of research studies and publications. The overwhelming majority of centres reported a willingness in principle to take part in future intercentre comparison studies: 190 (94.5%) and randomized control trials: 165 (82.1%). These figures have grown during the time of the project from 173 (86%) and 133 (66%) respectively in 1996, though only an estimated 29 (14.4%) of respondents are currently participating in intercentre studies and 15 (7.5%) in randomized control trials. Five centers have started a prospective intercentre study (Tartu, Tallinn, Riga, Vilnius, and Rostock) and ten other centres have already started with a randomized trial: Aarhus, Belfast, Bergen, Copenha-
gen, Gothenberg, Helsinki, Linko¨ping, Manchester, Oslo, and Stockholm. The latter are currently participating in a randomized control trial of primary surgery where the usual local method is being compared with a common protocol (lip and soft palate closure at 3–4 months, hard palate closure at 12 months). This landmark investigation is designed to compare the relative merits of four surgical methods and will serve as a model paradigm for future research in Europe and elsewhere. New opportunities for initiating collaborative research have become possible for the period 2000– 2004, following the award of a research grant from the European Commission’s Framework V budget. This will encompass the genetic and environmental basis of orofacial clefts and other craniofacial anomalies, and their possible prevention, as well as their treatment under the title ‘EUROCRAN’ 2000– 2004, with the following general objectives and five inter-related work-packages: 1. Initiation of a trial of surgery for unilateral cleft lip, alveolus and palate; 2. European Craniofacial Anomalies Directory including a ‘good practice’ reference archive to assist teams in assessing their results, and a prospective register for distraction osteogenesis; 3. Population based gene-environment interaction study of orofacial clefts; 4. A chromosomal approach to identifying orofacial cleft genes; 5. Molecular diagnosis of monogenic craniofacial anomalies.
CONCLUSION Cleft services, clinical protocols and research have undoubtedly suffered from unrestricted development across Europe. The chances that a child born with a cleft tomorrow in Europe will receive the best care possible depends almost entirely upon where he or she is born. Attainment of even minimum standards of care remains a major challenge in some communities and both, the will to reform and a basic strategy to follow are overdue. It is hoped that the Eurocleft Consensus Recommendations* reached during the present project will assist in improving the opportunities for tomorrow’s patients. It is also hoped that the collaborative research now beginning *Copies of the full report and recommendations are available from: Pauline Nelson, Eurocleft Project Administrator Department of Oral Health and Development University Dental Hospital of Manchester Higher Cambridge Street Manchester M15 6FH UK Tel: +44 161 275 6865 Fax: +44 161 275 6636 E-mail: [email protected]
138 Journal of Cranio-Maxillofacial Surgery
under the European Commission’s Framework V Programme will provide a focus for European researchers wishing to improve understanding, treatment and prevention of clefts of the lip and palate in the years ahead. Acknowledgements This work would not have been accomplished without the efforts of a large number of individuals, not least the individual teams who completed and verified a series of questionnaires. We are indebted to the principal contacts listed in Part I for their contribution to the development of guidelines and policies and for local efforts to validate responses and disseminate information. Each brought to the task a clear commitment to improving the standards of care for children with clefts of the lip, alveolus and palate across all of Europe, especially those yet to be born. We are indebted to Dr Alain Vanvossel of Directorate General XII for providing liaison and advice. Thanks also to: David Bearn, Melanie Walsh and Anette Lohmander-Agerskov for their contributions to the Eurocleft Consensus Recommendations, to Nicky Mandall for assistance with the systematic review, to David Bearn, Nicky Mandall, Alan Jack, Petra Zuurbier and Frances Conboy for facilitating the workshops and to Phil Eyres for handling of research data and tables.
Shaw WC, Dahl E, Asher-McDade C et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 5. General discussion and conclusions. Cleft Palate-Craniofacial J 29: 413–418, 1992b Shaw WC, Asher-McDade C, Brattstro¨m V et al.: Intercentre clinical audit for cleft lip and palate – a preliminary European investigation. In: Jackson IT, Sommerlad BC (eds.), Recent advances in plastic surgery. No. 4: Edinburgh: Churchill Livingstone; p. 1–15, 1992c Robertson NRE, Jolleys A: Effects of early bone grafting in complete clefts of lip and palate. Plastic Reconstr Surg 42: 414–442, 1968 Robertson NRE, Jolleys A: A further look at the effects of delaying repair of the hard palate. In: Huddart AG, Ferguson MWJ (eds.), Cleft lip and palate: long-term results and future prospects, Vol. 2. The older patient and future prospects. Manchester University Press; pp. 176–182, 1990 The Treacher Collins Syndrome Collaborative Group. Positional cloning of a gene involved in the pathogenesis of Treacher Collins syndrome. Nature Genetics 12: 130–136, 1996 Williams AC, Bearn D, Mildinhall S et al.: Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part 2 – Dento-facial outcomes, psychosocial status and patient satisfaction. Cleft PalateCraniofacial J 38: 24–29, 2001 World Health Organization, Human Genetics Programme. World Atlas of Birth Defects, Ist Edition. International Centre for Birth Defects of the International Clearinghouse for Birth Defects Monitoring Systems in collaboration with European Registration of Congenital Anomalies (EUROCAT) 1998
References Asher-McDade C, Brattstro¨m V, Dahl E et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 4. Assessment of nasolabial appearance. Cleft Palate-Craniofacial J 29: 409–412, 1992 Bearn D, Mildinhall S, Murphy T et al.: Cleft Lip and Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part 4 – Outcome comparisons, training and conclusions. Cleft PalateCraniofacial J 38: 38–43, 2001 Chalmers I, Altman DG: Systematic reviews. Brit. Med. J. Publishing, 1995. Clinical Standards Advisory Group. Cleft Lip and/or Palate. Report of a CSAG Committee. London: The Stationery Office, 1998 Forbes SA, Richardson M, Brennan L et al.: Refinement of the Xlinked cleft palate and ankylog lossia (CPX) localisation by genetic mapping in an Icelandic kindred. Hum Genet 95: 342–346, 1995 Mars M, Asher-McDade C, Brattstro¨m V et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 3. Dental arch relationships. Cleft Palate-Craniofacial J 29: 405–408, 1992 Marsh JL, Grames LM, Holtman B: Intravelar veloplasty: A prospective study. Cleft Palate J 26: 46–50, 1989 Mølsted K, Asher-McDade C, Brattstro¨m V et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 2. Craniofacial form and soft tissue profile. Cleft Palate-Craniofacial J 29: 398–404, 1992 Sandy JR, Williams AC, Bearn D et al.: Cleft Lip and Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part I – Background and methodology. Cleft Palate-Craniofacial J 38: 20–23, 2001 Sell D, Grunwell P, Mildinhall S et al.: Cleft Lip and Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part 3 – Speech outcomes. Cleft PalateCraniofacial J 38: 30–37, 2001 Semb G, Shaw WC: Facial growth after different methods of surgical intervention in patients with cleft lip and palate. Acta Odontol Scand 56: 352–355, 1998 Shaw WC, Asher-McDade C, Brattstro¨m V et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 1. Principles and study design. Cleft Palate-Craniofacial J 29: 393–397, 1992a
William C. Shaw Professor of Orthodontics and Dentofacial Development Department of Oral Health and Development University Dental Hospital of Manchester Higher Cambridge Street Manchester M15 6FH, UK Tel: +44-161-275-6620 Fax: +44-161-275-6794 E-mail: [email protected] Paper received 28 April 2000 Accepted 20 February 2001
APPENDIX I – Policy Statements 1. The professional involved in cleft care should provide basic information on cleft care and on the proposed treatment to any potential patient and/or patient’s guardian. Basic information should contain at least: . A general explanation of the condition, the reasons for treatment, what may or may not be achieved, the stages of treatment including examination, record collection and general protocols. This may be supplemented by leaflets, booklets or other kinds of information. . An explanation of why a specific treatment is considered necessary for the individual patient, what specifically is involved: method, timing, duration, cost, what the specific goal is and possible side effects. 2. When a treatment is considered, the professional engaged in cleft care should take into consideration the desires and attitudes of the patient and/or those of the patient’s guardian. The professional should also pay attention to and inform the patient/patient’s guardian of the risks and benefits inherent in the potential alternative treatment options, including no treatment or no further treatment. 3. If requested, it is the professional’s responsibility to provide a procedure for obtaining a second opinion for the patient. If
The Eurocleft Project 1996–2000: overview 139 requested, this procedure should be communicated to the patient before treatment starts. 4. After an episode of treatment, the professional engaged in cleft care should inform the patient and/or patient’s guardian on: . Outcome of treatment relative to the defined goal. . Undesirable effects of treatment. . Expected future development. 5. The professional engaged in cleft care should analyse and document any complaints or praise expressed by the patient and/or the patient’s guardian. 6. The professional engaged in cleft care should give consideration to the burden of the treatment: . Considerations should include financial as well as nonfinancial burden, such as treatment duration, effort from the patient and/or patient’s guardian and discomfort as a result of treatment. 7. During the process of treatment, the professional involved in cleft care should continuously evaluate treatment progress against the planned treatment and act accordingly. 8. Organizations and institutes responsible for the provision of cleft care should: . Encourage the cleft professional to follow the policies described above and to acknowledge the patient’s rights. . Recognize and encourage the professional’s right to provide treatment that can be expected to improve the patient’s condition whilst minimizing adverse effects. . Recognize and encourage that decisions on treatment priority should be based on criteria proposed by the cleft professionals in consultation with the patient and/or patient’s guardian. This is especially so in a situation with insufficient treatment resources. . Recognize and encourage that access to treatment should not depend on the patient’s ability to pay. . Recognize that cooperation of the patient with the advice and instructions of the cleft professional is necessary in order to achieve a successful result.
6. Ear, nose and throat (ENT) ENT disorders should be identified at an early stage and the necessary therapy should be provided. 7. Clinical genetics/paediatric developmental medicine As cleft lip and/or palate may be associated with other anomalies early assessment and diagnosis is necessary. Genetic counselling for patients and families should be available. 8. Emotional support and professional advice for the growing child and its parents Emotional support and professional advice for parents, patients and their environment is often necessary and should be available. 9. Dental care Regular dental care should be available. 10. National register A national register should be in place for accurate recording of children born with cleft lip and/or palate and related craniofacial anomalies.
Part II: Organization of services 1. Cleft care should be provided by a multidisciplinary team of specialists. 2. Members of the team should have special training in cleft care. 3. The team should agree on the stages of treatment including the examination, record collection and general protocols. 4. There should be one person responsible for quality improvement and communication within the team. 5. Coordination of the care of individual patients is important since numerous specialities are involved. This should be the responsibility of one member of the team. 6. The number of patients referred to the team should be sufficient to sustain the experience and specialist skills of all team members and to allow evaluation/audit of the team’s performance within a reasonable period of time. It has been recommended that cleft surgeons, orthodontists and speech therapists should treat at least 40–50 new cases annually. However, it is recognized that individual member states have the right to provide care for their own population.
APPENDIX II – Practice Guidelines Part I: Healthcare needs
Part III: Finances
1. Neonatal emotional support and professional advice In the event of prenatal diagnosis and as soon as possible after the birth of a child with a cleft parents should be given emotional support and advice about the child’s future management by a specialist in cleft care.
Resources should be available to cover the following care for children with cleft lip and palate:
2. Neonatal nursing Difficulties in feeding are common in the early days of life and specialist advice on feeding should be provided. 3. Surgery Primary surgery to close clefts of the lip and/or palate should be performed by an experienced and qualified surgeon according to a protocol agreed by the team. Further corrective procedures may be necessary for some patients in later years and should be performed by an experienced and qualified surgeon according to a protocol agreed by the team 4. Orthodontic/orthopaedic treatment For children with cleft lip and palate orthodontic/orthopaedic treatment should be available when necessary and should be performed by an experienced orthodontist. 5. Speech and language therapy Early assessment of speech and language problems, advice to parents and the availability of corrective therapy by an experienced speech and language therapist should be provided.
1. Emotional support and professional advice during the neonatal period. 2. Neonatal nursing. 3. Surgery. 4. Orthodontic/orthopaedic treatment. 5. Speech and language assessment and therapy. 6. Ear, Nose and Throat treatment. 7. Clinical genetics/paediatric developmental medicine. 8. Emotional support for the growing child and its parents. 9. Travel expenses. 10. General dental care including cleft related prosthodontics.
APPENDIX III – General Principles Governing Record Taking 1. Records for treatment planning/monitoring . Clinical records should be taken for individual patients to allow treatment planning, monitoring treatment progress and treatment evaluation.
140 Journal of Cranio-Maxillofacial Surgery . The timing and nature of these records will depend on the clinical protocols followed by individual teams. . Treatment and associated record taking protocols should be agreed and clearly set out by the cleft team.
2. Cleft Palate Only (CPO) Timing
2. Records for quality improvement/research Additional records may be taken for a number of other reasons: . Follow up of a series of patients to provide an overview of the outcome of care. . To allow retrospective comparisons of different protocols. . As part of a prospective clinical trial with ethical approval. . As part of an agreed protocol for intercentre quality improvement comparisons or comparison against known standards. . As part of an agreed research protocol. . Other reasons such as medico-legal, second opinion. 3. Safeguards . Exposure of patients to unnecessary radiation should be avoided. . Research and quality improvement records should only be taken when there is an established written protocol on how they will be put to use. . Research and quality improvement records should not be taken without the consent of the patient/parent/guardian. . Research and quality improvement records should coincide as far as possible with the records for treatment planning/ monitoring (Statement 1).
TIMING OF MINIMUM RECORDS
Models Lateral Photo- Speech Audioskull graphs metry/ radiotympanograph metry
Primary H surgery 3 Years 5/6 Years H 15/16 Years H
Patient/ parent satisfaction
H
H
H H H
H
H H H
H
3. Clift Lip Only Timing
Models
Photographs
Primary surgery 5/6 Years 18+Years
H*
H
H*
H H
Patient/ parent satisfaction
H
*Only in cases with cleft of the alveolus as well as cleft lip.
4. Alveolar Bone Grafting Timing
Intra-oral radiograph
Photographs
Just before bone graft 6 months after graft After canine fully erupted
H H H
H H
1. Complete Cleft Lip, Alveolus and Palate (UCLAP & BCLAP) Timing
Primary surgery 3 Years 5/6 Years 10 Years 18+Years
Models Lateral Photo- Speech Audioskull graphs metry/ radiotympanograph metry H
H
H H H
H H H
H H
*If hard palate is closed.
H* H H H
Patient/ parent satisfaction
H* H H
5. Pharyngoplasty Timing
Speech sample
Just before operation One year after operation
H H
6. Orthognathic Surgery H
Timing
Lateral cephalogram
Models
Just before operation One year after operation
H H
H H
The Eurocleft Project 1996–2000: overview William C. Shaw,1 Gunvor Semb,1,2 Pauline Nelson,1 Viveca Brattstro¨m,3 Kirsten Mølsted,4 Birte Prahl-Andersen,5 Karsten K.H. Gundlach6 1
Department of Oral Health and Development, University Dental Hospital of Manchester, UK; 2National Hospital and Dental Faculty of Oslo, Bretdvet Kompetansesenter, Norway; 3Eastman Institutet, Dalagatan 11, Stockholm, Sweden; 4Cleft Palate Department, Dental Clinic, Speech And Hearing Institute, Hellerup, Denmark; 5Department of Orthodontics, Academic Centre for Dentistry Amsterdam, Amsterdam, The Netherlands; 6Department of Maxillofacial and Facial Plastic Surgery, University Hospital, Rostock, Germany SUMMARY. Introduction: The original Eurocleft project, a European intercentre comparison study, revealed dramatic differences in outcome, which were a powerful stimulus for improvement in the services of respective teams. The study developed a preliminary methodology to compare practices and the potential for wider European collaboration including opportunities for the promotion of clinical trials and intercentre comparison was recognized by the European Commission. Therefore, the project: ‘Standards of Care for Cleft Lip and Palate in Europe: Eurocleft’ ran between 1996 and 2000 and aimed to promote a broad uplift in the quality of care and research in the area of cleft lip and palate. Results: The results of the 1996–2000 project include: . a register of services in Europe, with details of professionals and teams involved in cleft care, service organization, clinical protocols and special facilities for research; . a set of common Policy Statements governing clinical practice for European cleft teams, Practice Guidelines describing minimum recommendations for care that all European children with clefts should be entitled to and recommendations for Documentation governing minimum records that cleft teams should maintain; . encouraging initial efforts to compare outcomes (results) of care between centres. A survey showed a wide diversity in models of care and national policies as well as clinical practices in Europe. Of the 201 centres that registered with the network, the survey showed 194 different protocols being followed for only unilateral clefts. Conclusion: Cleft services, treatment and research have undoubtedly suffered from haphazard development across Europe. Attainment of even minimum standards of care remains a major challenge in some communities and both the will to reform and a basic strategy to follow are overdue. It is hoped that the Eurocleft Consensus Recommendations reached during the present project will assist in improving the opportunities for tomorrow’s patients. It is also hoped that the collaborative research now beginning under the European Commission’s Framework V Programme will provide a focus for European researchers wishing to improve understanding, treatment and prevention of clefts of the lip, alveolus and palate in the years ahead. # 2001 European Association for Cranio-Maxillofacial Surgery
The ultimate goal of the proposed project was to improve the effectiveness and efficiency of care for European children with clefts of the lip, alveolus and/ or palate. Following publication of an European intercentre comparison study (Shaw et al., 1992a, Mølsted et al., 1992; Mars et al., 1992; Asher-McDade et al., 1992; Shaw et al., 1992b), the potential for wider European collaboration was recognized. The original Eurocleft study developed a preliminary methodology to compare practices, and the dramatic differences in outcome revealed were a powerful stimulus for improvement in the services of respective teams. Furthermore, the shared experience of open evaluation, the exchange of views and the identification of wider opportunities for research promoted multicentre collaboration and a series of clinical trials.
INTRODUCTION This paper describes the concerted action project: ‘Standards of Care for Cleft Lip and Palate in Europe: Eurocleft’ funded by the European Commission under its BIOMED II and INCO COPERNICUS Programmes. The project, aimed at developing a network of cleft teams within the European Union and the European Economic Area member states, ran for 3 years between 1996 and 1999. Teams from Central and Eastern European states joined the network via support under INCO for a further year. Support in the form of grants: European Commission BIOMED II Programme, Contract No. BMH4-CT95-0402; European Commission INCO COPERNICUS Programme, Contract No. IC 15-CT98-0301. 131
132 Journal of Cranio-Maxillofacial Surgery
The BIOMED II and INCO project aimed to recreate this approach on a pan-European scale in the hope of promoting a broad uplift in the quality of care and research. A Steering Group of five individuals from cleft teams in Denmark, the Netherlands, Norway, Sweden, and the UK was commissioned to undertake the work. The aim was the attainment of four measurable objectives: .
. .
.
the production of a Register of Services in Europe, with details of professionals and teams involved in cleft care, service organisation, clinical protocols and special facilities for research, a set of common Policy Statements governing clinical practice for European cleft teams, a set of common Practice Guidelines describing minimum recommendations for care that all European children with clefts should be entitled to, and a set of common recommendations for Documentation governing minimum records that cleft teams should maintain.
BACKGROUND Clefts of the lip, alveolus and/or palate affect between 1 per 500 to 1 per 700 live births in Europe (World Health Organization, 1998). Highly specialized corrective surgery in the early months of life is necessary to improve function and appearance. Subsequent impairment of facial and dental development, speech and hearing are common and may be accompanied by social-psychological maladjustment. A large number of specialist services is necessary if the child’s potential is to be maximized: neonatal nursing and community nurse services, surgery, speech, hearing, otolaryngology, dentistry, orthodontics, psychology and genetic counselling. Approaches to cleft care in Europe vary considerably between countries. Within the Nordic countries there is a tradition of centralization of services. Other countries such as France, Germany and Italy (and until recently, the UK) provide cleft care via large numbers of local services who may see but a few patients with clefts. Consequences of poorly organized care The original Eurocleft intercentre study suggested that decentralized care by low volume operators may be associated with inferior outcomes. The ultimate consequences of this are greater hardship for patients and families and increased health costs associated with additional operations and ancillary care. The relatively low case load and independent development of cleft services in parts of Europe make it likely that varied standards are being achieved. The extent of the disparity in outcomes revealed in the previous study was dramatic. The need for a maxillary
osteotomy differed between the best and worst services by a factor of 8 (6% vs 48%; Shaw et al., 1992c). Furthermore, care in the worst service was much more complex and expensive(!): Thus, significant improvements could be achieved at no extra cost through local recognition of the need to change practices. This is particularly important at a time when budgets for health care across Europe are under severe pressure. Lack of evidence-based practices As cleft lip, alveolus and/or palate is a birth anomaly with relatively low frequency and numerous anatomical subtypes, critical studies including large samples are rarely achieved. In effect it is an ‘orphan condition’ and present care is far from ‘evidence based’. Not surprisingly, the diversity of approach to cleft treatment is great. The previous Eurocleft study recommended that intercentre evaluation was crucial to the maintenance of standards and encouraged refinement of methodology for comparative study. By rigorously managed intercentre evaluation, clinicians might compare outcomes for different protocols of care and identify harmful practices. Where no important differences emerge, balanced judgements could then be made on the selection of future protocols based on factors such as the burden on patients and their families, simplicity of treatment and economic cost. The study also proposed multicentre prospective trials as a way forward in refining treatment (Shaw et al., 1992c). As a preliminary step in the present project, we undertook a systematic review of the literature concerning treatment for unilateral and bilateral cleft lip, alveolus and palate with cephalometric measurement of the face as an outcome. This systematic review was planned according to the methodology of the Cochrane Collaboration (Chalmers and Altman, 1995). The search strategy included: . . . .
searching the electronic databases of Medline and Embase from 1966–1997, hand-searching the Cleft Palate-Craniofacial Journal from 1964–1997, searching the Cochrane Trials Register, and following up relevant citations in the papers read.
In all, 5474 titles were identified of which 189 reports were found ‘relevant’. Of these, only three described clinical trials. Robertson and Jolleys conducted two small randomized control trials of primary surgery in the 1960s. One, involving two groups of 20 cases where one group’s anterior palate closure was delayed until age 5, found no benefit for dentofacial growth (Robertson and Jolleys, 1990). In the second study a similar sample was randomized in respect of alveolar bone grafting at the time of lip repair. Follow-up revealed a detrimental effect on facial growth in the grafted group (Robertson and Jolleys, 1968). In a controlled clinical trial alternated
The Eurocleft Project 1996–2000: overview 133
palate repair with or without intravelar veloplasty in 51 subjects with a broad range of palatal cleft types (Marsh et al., 1989). Speech evaluations were made at a 2-year follow-up. No difference in outcome was detected, but the procedure including intravelar veloplasty required a significantly longer operating time. All other available research reports have been retrospective, frequently at case series level. A review of these has also been undertaken and we concluded that comparison between reported outcomes is unreliable because of the invariable likelihood of bias (Semb and Shaw, 1998). This disappointing level of clinical evidence in the craniofacial field necessary for evidence-based medical treatment reflects the fact that funding agencies have hitherto been disinclined to fund, and researchers disinclined to initiate randomized control trials, because of their duration. Recruitment is lengthy owing to the scarcity of cases, and relevant outcomes (e.g. speech or facial growth) can only be studied several years after surgery. The infrastructure that would make such studies possible has until now been lacking, both in Europe and elsewhere. Uncoordinated research in the basic sciences The aetiology of clefts has a definite element of inheritance and could thus be amenable to gene mapping. But in this area of basic medical science there has only been limited progress. For example, the gene for one cleft palate syndrome has been mapped for an extended Icelandic family (Forbes et al., 1995) as has the gene for the Berry–T. Collins– Franceschetti Syndrome (Treacher Collins Syndrome Collaborative Group, 1996). Advances in the more complex genetics of non-syndromic clefts require concertation between highly specialised molecular genetics laboratories, epidemiologists and teams with extended recording of pedigree in their patients. These resources do exist in Europe but are not well linked at present.
MATERIAL AND METHODS A three stage methodology was adopted for this project: . . .
creation of a network, development of consensus recommendations, survey of current services.
Creation of the network Each member of the Steering Group had responsibility for linking with cleft professionals from a specific group of countries where they had particular expertise in terms of language or experience. This strategy facilitated a nomination process amongst the
registered teams who were asked to suggest individuals from each of their countries to act as a key delegate and provide a general link to the network. The criteria for nomination included the requirement that the individual should be an expert in the field of cleft care with a good knowledge and general overview of cleft care organization in their country. National representatives were chosen on the basis of nomination by their national colleagues and a spread of clinical speciality in order to secure a multidisciplinary breadth to the network. During the project some representatives were replaced and others duplicated due to particular local factors (Table 1). Development of consensus recommendations It was evident from previous investigations that the quality of cleft care would vary enormously between one region and another arising from organizational or surgical factors. It was considered inappropriate to prescribe specific technical protocols on the timing, sequence and design of surgical procedures, orthodontic treatment, speech therapy and so forth as many of these choices remain highly controversial and practices currently vary to a great extent across Europe. Resolving such issues would require the initiation of multicentred randomized trials and would need to await the establishment of better infrastructure and research capability across European teams. However, it was hoped that quality improvement of an organizational nature could be promoted by recommendations upon policies and practice guidelines. Therefore, the Steering Group and the national representatives forming the Eurocleft network came together in a series of meetings: As a result of the First Eurocleft BIOMED II European Workshop in Manchester, April 1998 (attended by national representatives from the fifteen EU member states with the addition of Czech Republic, Poland, Iceland, Norway and Switzerland), areas of agreement identified and minimum recommendations in the form of Policy Statements and Practice Guidelines were drawn up. In addition, initial discussions took place and an early draft on the issue of record taking was made. At the First INCO European Workshop in Manchester, February 1999 (attended by national representatives from 10 countries of Central and Eastern Europe: Bulgaria, Czech Republic, Estonia, Hungary, Latvia, Poland, Romania, Slovak Republic, Slovenia and Ukraine) the papers on Policy Statements and Practice Guidelines were finalized. It was proposed that quality improvements of surgical skills would best be achieved by the promotion of clinical audit. If the methodology for assessing outcomes could be defined and established by consensus, this would allow European centres to evaluate their own quality of care, compare it with that achieved by other centres, and to initiate local quality improvement. Thus, at the Second Eurocleft
134 Journal of Cranio-Maxillofacial Surgery Table 1 – National representatives of participating European countries Austria Belgium Bulgaria Czech Republic Czech Republic Denmark Estonia Finland France Germany Germany Greece Hungary Iceland Ireland Italy Italy Latvia Netherlands Netherlands Norway Poland Portugal Portugal Romania Slovak Republic Slovenia Spain Sweden Switzerland UK Ukraine
Hans Ka¨rcher Albert de Mey Youri Anastassov Jitka Vrtisˇ kova´ Ziva Mu¨llerova Anja Bau Siiri Hanstein Jorma Rautio Chantal Trichet Karsten Gundlach Charlotte Opitz Meropi Spyropoulos Gizella Reha´k O´lafur Einarsson Eamonn McKiernan Giovanna Garattini Ferruccio De Stefano Biruta Barkane Emmy Konst Anne Marie Kuijpers-Jagtman Rolf Tindlund Kazimierz Kobus Maria Joa´o Alves de Castro Anto´nio Capelo Lidia Boboc Irena Klı´ mova´ Vesna Kozelj Gonzalo Gonzalez-Landa Jan Lilja Wanda Gnoinski Liz Albery Leonid Kharkov
European Workshop, Manchester, June 1999 (attended by both groups of national representatives, Table 1), general principles governing record taking were agreed and consensus on the timing of records reached. These recommendations were finalized in the paper on Principles Governing Record Taking and Timing of Records. The guidelines were subsequently circulated to all registered centres of the project, national health authorities and governments. As it became obvious that many countries have not yet performed outcome studies the Steering Group organized 2-day Research Methodology Courses for registered centres of the network in February and October 1999. Both courses were well attended by participants from a spread of clinical specialities and European countries. The course content included: research design, measurement of outcome, sources of bias, practical management of research, implementation of research findings and in addition, practical sessions with the opportunity for participants to gain experience using established rating scales. The agreed European guidelines for cleft teams were also disseminated. Survey of current European services and research resources A preliminary register was compiled from a number of sources: cleft association membership lists, international symposia attendance lists and individuals identified by a literature search. The project was also promoted at the outset via an abstract published in
maxillofacial surgeon plastic surgeon plastic surgeon plastic surgeon orthodontist speech therapist maxillofacial surgeon plastic surgeon speech therapist maxillofacial surgeon orthodontist orthodontist orthodontist plastic surgeon orthodontist orthodontist plastic surgeon maxillofacial surgeon speech therapist orthodontist orthodontist plastic surgeon orthodontist paediatric surgeon orthodontist orthodontist maxillofacial surgeon paediatric surgeon plastic surgeon orthodontist speech therapist maxillofacial surgeon
relevant European scientific journals and through papers presented by individual Steering Group members at international meetings of cleft professionals. After an Initial Questionnaire was sent out to approximately 500 possible centres asking for general information about patient caseload, clinical specialities involved in the team, primary surgical protocol and research activity a total of 201 centres registered with the project from 30 European countries. There are also a number of centres elsewhere that received questionnaires but for whatever reason, decided not to participate. RESULTS Policy Statements and Practice Guidelines were drawn up, discussed and finalized in April 1998 and February 1999 (Appendices I and II). Principles Governing Record Taking and Timing of Records had been drawn up in April 1998, discussed in February 1999 and finalized in June 1999 (Appendix III). An European Register of Cleft Centres was assembled including a total of 201 centres from 30 European countries (Table 2). We are also aware of a number of centres elsewhere (including two in Russia) that received questionnaires but for whatever reason, decided not to participate, making the survey 40% representative. Survey Findings I: European diversity in models of care and national policies. The greatest challenge to
The Eurocleft Project 1996–2000: overview 135
the delivery of adequate services across much of Europe appears to be organizational. Compliance with key recommendations such as the creation of fully comprehensive teams with a sufficiently large caseload to optimize clinical experience and outcome evaluation (e.g. 40 new cases per year per centre, i.e. surgeon, orthodontist and speech therapist), is being achieved in a minority of countries. From the workshops in 1998/1999 it was clear that the greatest obstacles to progress were human, not economic. In some countries, policies concerning the provision of cleft care are well established. In Denmark, the provision of primary surgery by one surgical team has been formalized in law since 1937. More recently, in Norway, the long standing arrangement of two national centres was confirmed by a law stating that no care can be provided elsewhere unless requested by one of these cleft teams. In the Czech Republic, Latvia and Estonia, well established regional centres are continuing to provide comprehensive services following recent political and economic changes. In some circumstances, a move towards privatisation of healthcare is considered to present the risk of possible destabilization of established centres. At the other extreme, the concept of team care is still to be adopted. National representatives from Bulgaria, Greece, Italy, Portugal, Romania, Spain and Ukraine for instance, reported that much care was still provided by individual clinicians, working in isolation, and not in government funded centres. Variation in policies is just as apparent among EU states and the non-EU states of Central and Eastern Europe; in countries such as Bulgaria, Romania and Ukraine, services are still fragmented and difficulties in securing adequate clinical resources are reported.
Elsewhere, regionalization of insurance for example, in France and Germany, and regional health care autonomy as in Finland, are tending to favour decentralized care. Even where centres exist, they are often small. Indeed, there are several examples of two, three or four centres co-existing in the same city, eg. Antwerp, Athens, Barcelona, Berlin, Bern, Bologna, Brussels, Bucharest, Budapest, Innsbruck, Lille, Lisbon, London, Madrid, Milan, Oporto, Paris, Parma, Rome, Verona and Warsaw. Survey Findings II: European diversity in clinical practices. Involvement of a wide variety of surgical specialities in cleft surgery was reported (Table 3) but no attempt was made to distinguish the speciality responsible for primary repair of the cleft. The first five surgical specialities are listed according to the order reported by the teams. Those mentioned most often were plastic surgery: 94 (46.7%), maxillofacial surgery: 59 (29.4%), and paediatric surgery: 22 (10.9%). The clear lack of a sound evidence base for selecting treatment protocols, is reflected by a remarkable diversity of practices across Europe for the surgical care of just one cleft sub-type, unilateral complete cleft of lip, alveolus and palate. Seventeen possible sequences of operation to close the cleft are practised (Table 4). Though 86 (42.8%) of teams closed the lip at the first operation and the hard and soft palate together at the second, almost every other conceivable sequence appears to be practised somewhere. Thus the total number of operations taken to complete the closure of the cleft varied from one: 10 (5%), two: 144 (71.1%), three: 43 (21.9%) and four: 4 (2%). Around half the registered teams employ presurgical orthopaedics, of whom 67 (65%), use it
Table 2 – Countries covered by the European Register of Cleft Centres Austria Belgium Bulgaria Croatia Czech Republic
Denmark Estonia Finland France Germany
Greece Hungary Iceland Ireland Italy
Latvia Lithuania Moldova Netherlands Norway
Poland Portugal Romania Slovak Republic Slovenia
Spain Sweden Switzerland UK Ukraine
Table 3 – Specialities involved in cleft surgery Speciality
First speciality (%)
Second speciality (%)
Third speciality (%)
Fourth speciality (%)
Fifth speciality (%)
Plastic surgery Maxillofacial surgery Paediatric surgery Plastic/maxillofacial ENT ENT/phoniatrics Maxillofacial/ENT Paed/maxfac/plastic Paediatric/maxfac Plastic/craniofacial Plastic/paediatric Stomatology Craniofacial surgery Head/neck Neurosurgery Unspecified
46.7 29.4 10.9 3.0 2.0 1.0 0.5 0.5 0.5 0.5 0.5 0.5 0 0 0 4.0
31.0 49.3 6.8 2.0 2.7 0 0 0 0 0 0 0.7 0.7 0 0.7 6.1
21.4 60.0 1.3 2.7 5.3 0 0 0 0 0 0 0 0 1.3 1.3 6.7
6.7 73.3 3.3 3.3 6.7 0 0 0 0 0 0 0 0 0 0 6.7
14.3 57.1 14.3 0 0 0 0 0 0 0 0 0 0 0 0 14.3
136 Journal of Cranio-Maxillofacial Surgery Table 4 – Sequence of operations for the repair of unilateral complete cleft lip, alveolus and palate First operation
Second operation
Lip closure Lip closure Lip and hard palate closure Lip and soft palate closure Lip, hard and soft palate closure Lip closure
Hard and soft palate closure Soft palate closure Soft palate closure Hard palate closure
Lip and soft palate closure
Hard palate closure and gingivo-alveoloplasty Hard and soft palate closure Lip and hard palate closure Lip closure Soft palate closure Lip, hard and soft palate closure Lip and hard palate closure Lip closure
Lip and alveolar closure Soft palate closure Lip adhesion Lip and alveolar closure Lip adhesion Lip adhesion Hard and soft palate closure and alveoloplasty Lip and soft palate Lip adhesion Lip closure Total
Soft palate closure
Hard palate closure and alveolar bone grafting Lip closure Soft palate closure
routinely. Mostly passive plates were used by 74 (70%) and some teams also used the plate as a feeding plate. Not one single European team practices the same protocol as any other (except for those participating in the clinical trial described below). That is to say, 201 teams currently practice 194 different protocols for one cleft sub type. Survey Findings III: Consumer associations in Europe. Of the 28 European countries involved in the network, 18 have some form of organization for parents. Such groups vary in the degree of their activity, effectiveness and influence and take different forms. Some are functioning as officially established national associations whilst others are operating on a more informal basis. In Germany for instance, there are several small regional organizations and one major group which takes part in the annual meetings with the professional association. Denmark has a well established group for parents which has been functioning for over 25 years whilst in other countries such as the Ukraine a priority for the future is the creation of such a group. Interestingly in Bulgaria, the parent group is working to bring together national cleft specialists with a view to instigating an association for professionals.
Third operation
The challenge of improving quality of services Achieving optimal standards of cleft care across Europe remains an outstanding challenge. The basic principles of care described in the Policy Statements and Practice Guidelines (Appendices I and II) are hardly revolutionary, indeed they describe what most individuals (including those in clinical practice or
% 42.8 15.3 10.4 10.0 5.0 3.5
Hard palate closure
Hard palate closure and alveolar bone grafting
2.5
Soft palate closure Hard palate closure
Hard palate closure
Soft palate closure
2.0 2.0 1.5 1.0 1.0 1.0 0.5 0.5
Hard and soft palate closure Gingivo- alveoloplasty
Hard palate closure
0.5 0.5 100
government) would wish for their own children, regardless of country of birth. The potential strength of a European network is that examples of good practice can be more easily shared, challenges of a general nature can be more easily recognized and understood, and solutions that have been successful in one country can be applied elsewhere. The need for this is obvious if one considers the fact that today the variation between centres regarding sequence, technique and timing of cleft repair could hardly be greater! On the other hand, some obstacles, especially those arising from specific local circumstances and personalities, need specific local solutions. Among the difficulties mentioned were: . . . . . .
DISCUSSION
Fourth operation
personal egotism of individuals unwilling to discontinue the practice of treating a few children each year; competition between specialities for preeminence in the field, e.g. plastic-vsmaxillofacial-vs-paediatric-vs-ENT surgery; local pride, with every hospital, town or region desiring its own small team or wish to have local service; lack of clinical leadership; necessity for teaching hospitals to cover a spectrum of clinical practice; lack of responsiveness in the health authorities at local and national level.
This places a heavy responsibility on the national representatives of each country who have taken part in the Eurocleft process over the last 4 years. All of the above problems have confronted the UK in the recent past and a national review was instigated by the government Clinical Standards Advisory Group (CSAG, 1998). The review included a national survey that revealed that Britain’s fragmented, decentralized services were achieving a low standard of clinical care
The Eurocleft Project 1996–2000: overview 137
in some areas. As a result the Government instructed regions to provide care from a single regional centre. Each of these should have a fully comprehensive specialist team, typically with two to three surgeons, each responsible for not less than 40 new cases for primary surgery per year. In this instance, government interest has been essential in trying to improve services when voluntary methods failed (Bearn et al., 2001; Sandy et al., 2001; Sell et al., 2001; Williams et al., 2001). However, some early progress is already being reported by European national representatives. At the time of writing these include: .
.
.
.
Restructuring – in Hungary, the Netherlands and Slovak Republic unification of services has already reduced the number of centres and restructuring in Poland is under way. In Ireland a new unified regional centre has been established and there are hopes for a specialized centre in Greece. New associations – in Spain a new national association for professionals and parents has formed and there are similar plans in Portugal. In Austria the first multidisciplinary meeting of cleft professionals has been held and is set to become an annual event with a new national association to be founded in 2001. Similar efforts are taking place in France. Funding – in Estonia, orthodontic treatment with fixed appliances for children with clefts is now provided free, in Latvia extra funding for cleft services generally has been provided, and a similar announcement is expected in Poland. Registries – new national registries for clefts are being created in Belgium, Romania, Slovak Republic and Ukraine.
The challenge of improving research infrastructure Research activity Centres were given the opportunity to comment on the research interests and activities of their team, and any special resources for research such as databases, equipment, and collaboration with genetics laboratories. Some centres reported comprehensive listings of research studies and publications. The overwhelming majority of centres reported a willingness in principle to take part in future intercentre comparison studies: 190 (94.5%) and randomized control trials: 165 (82.1%). These figures have grown during the time of the project from 173 (86%) and 133 (66%) respectively in 1996, though only an estimated 29 (14.4%) of respondents are currently participating in intercentre studies and 15 (7.5%) in randomized control trials. Five centers have started a prospective intercentre study (Tartu, Tallinn, Riga, Vilnius, and Rostock) and ten other centres have already started with a randomized trial: Aarhus, Belfast, Bergen, Copenha-
gen, Gothenberg, Helsinki, Linko¨ping, Manchester, Oslo, and Stockholm. The latter are currently participating in a randomized control trial of primary surgery where the usual local method is being compared with a common protocol (lip and soft palate closure at 3–4 months, hard palate closure at 12 months). This landmark investigation is designed to compare the relative merits of four surgical methods and will serve as a model paradigm for future research in Europe and elsewhere. New opportunities for initiating collaborative research have become possible for the period 2000– 2004, following the award of a research grant from the European Commission’s Framework V budget. This will encompass the genetic and environmental basis of orofacial clefts and other craniofacial anomalies, and their possible prevention, as well as their treatment under the title ‘EUROCRAN’ 2000– 2004, with the following general objectives and five inter-related work-packages: 1. Initiation of a trial of surgery for unilateral cleft lip, alveolus and palate; 2. European Craniofacial Anomalies Directory including a ‘good practice’ reference archive to assist teams in assessing their results, and a prospective register for distraction osteogenesis; 3. Population based gene-environment interaction study of orofacial clefts; 4. A chromosomal approach to identifying orofacial cleft genes; 5. Molecular diagnosis of monogenic craniofacial anomalies.
CONCLUSION Cleft services, clinical protocols and research have undoubtedly suffered from unrestricted development across Europe. The chances that a child born with a cleft tomorrow in Europe will receive the best care possible depends almost entirely upon where he or she is born. Attainment of even minimum standards of care remains a major challenge in some communities and both, the will to reform and a basic strategy to follow are overdue. It is hoped that the Eurocleft Consensus Recommendations* reached during the present project will assist in improving the opportunities for tomorrow’s patients. It is also hoped that the collaborative research now beginning *Copies of the full report and recommendations are available from: Pauline Nelson, Eurocleft Project Administrator Department of Oral Health and Development University Dental Hospital of Manchester Higher Cambridge Street Manchester M15 6FH UK Tel: +44 161 275 6865 Fax: +44 161 275 6636 E-mail: [email protected]
138 Journal of Cranio-Maxillofacial Surgery
under the European Commission’s Framework V Programme will provide a focus for European researchers wishing to improve understanding, treatment and prevention of clefts of the lip and palate in the years ahead. Acknowledgements This work would not have been accomplished without the efforts of a large number of individuals, not least the individual teams who completed and verified a series of questionnaires. We are indebted to the principal contacts listed in Part I for their contribution to the development of guidelines and policies and for local efforts to validate responses and disseminate information. Each brought to the task a clear commitment to improving the standards of care for children with clefts of the lip, alveolus and palate across all of Europe, especially those yet to be born. We are indebted to Dr Alain Vanvossel of Directorate General XII for providing liaison and advice. Thanks also to: David Bearn, Melanie Walsh and Anette Lohmander-Agerskov for their contributions to the Eurocleft Consensus Recommendations, to Nicky Mandall for assistance with the systematic review, to David Bearn, Nicky Mandall, Alan Jack, Petra Zuurbier and Frances Conboy for facilitating the workshops and to Phil Eyres for handling of research data and tables.
Shaw WC, Dahl E, Asher-McDade C et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 5. General discussion and conclusions. Cleft Palate-Craniofacial J 29: 413–418, 1992b Shaw WC, Asher-McDade C, Brattstro¨m V et al.: Intercentre clinical audit for cleft lip and palate – a preliminary European investigation. In: Jackson IT, Sommerlad BC (eds.), Recent advances in plastic surgery. No. 4: Edinburgh: Churchill Livingstone; p. 1–15, 1992c Robertson NRE, Jolleys A: Effects of early bone grafting in complete clefts of lip and palate. Plastic Reconstr Surg 42: 414–442, 1968 Robertson NRE, Jolleys A: A further look at the effects of delaying repair of the hard palate. In: Huddart AG, Ferguson MWJ (eds.), Cleft lip and palate: long-term results and future prospects, Vol. 2. The older patient and future prospects. Manchester University Press; pp. 176–182, 1990 The Treacher Collins Syndrome Collaborative Group. Positional cloning of a gene involved in the pathogenesis of Treacher Collins syndrome. Nature Genetics 12: 130–136, 1996 Williams AC, Bearn D, Mildinhall S et al.: Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part 2 – Dento-facial outcomes, psychosocial status and patient satisfaction. Cleft PalateCraniofacial J 38: 24–29, 2001 World Health Organization, Human Genetics Programme. World Atlas of Birth Defects, Ist Edition. International Centre for Birth Defects of the International Clearinghouse for Birth Defects Monitoring Systems in collaboration with European Registration of Congenital Anomalies (EUROCAT) 1998
References Asher-McDade C, Brattstro¨m V, Dahl E et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 4. Assessment of nasolabial appearance. Cleft Palate-Craniofacial J 29: 409–412, 1992 Bearn D, Mildinhall S, Murphy T et al.: Cleft Lip and Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part 4 – Outcome comparisons, training and conclusions. Cleft PalateCraniofacial J 38: 38–43, 2001 Chalmers I, Altman DG: Systematic reviews. Brit. Med. J. Publishing, 1995. Clinical Standards Advisory Group. Cleft Lip and/or Palate. Report of a CSAG Committee. London: The Stationery Office, 1998 Forbes SA, Richardson M, Brennan L et al.: Refinement of the Xlinked cleft palate and ankylog lossia (CPX) localisation by genetic mapping in an Icelandic kindred. Hum Genet 95: 342–346, 1995 Mars M, Asher-McDade C, Brattstro¨m V et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 3. Dental arch relationships. Cleft Palate-Craniofacial J 29: 405–408, 1992 Marsh JL, Grames LM, Holtman B: Intravelar veloplasty: A prospective study. Cleft Palate J 26: 46–50, 1989 Mølsted K, Asher-McDade C, Brattstro¨m V et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 2. Craniofacial form and soft tissue profile. Cleft Palate-Craniofacial J 29: 398–404, 1992 Sandy JR, Williams AC, Bearn D et al.: Cleft Lip and Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part I – Background and methodology. Cleft Palate-Craniofacial J 38: 20–23, 2001 Sell D, Grunwell P, Mildinhall S et al.: Cleft Lip and Palate Care in the United Kingdom (UK). The Clinical Standards Advisory Group (CSAG) Study: Part 3 – Speech outcomes. Cleft PalateCraniofacial J 38: 30–37, 2001 Semb G, Shaw WC: Facial growth after different methods of surgical intervention in patients with cleft lip and palate. Acta Odontol Scand 56: 352–355, 1998 Shaw WC, Asher-McDade C, Brattstro¨m V et al.: A six-center international study of treatment outcome in patients with clefts of the lip and palate: Part 1. Principles and study design. Cleft Palate-Craniofacial J 29: 393–397, 1992a
William C. Shaw Professor of Orthodontics and Dentofacial Development Department of Oral Health and Development University Dental Hospital of Manchester Higher Cambridge Street Manchester M15 6FH, UK Tel: +44-161-275-6620 Fax: +44-161-275-6794 E-mail: [email protected] Paper received 28 April 2000 Accepted 20 February 2001
APPENDIX I – Policy Statements 1. The professional involved in cleft care should provide basic information on cleft care and on the proposed treatment to any potential patient and/or patient’s guardian. Basic information should contain at least: . A general explanation of the condition, the reasons for treatment, what may or may not be achieved, the stages of treatment including examination, record collection and general protocols. This may be supplemented by leaflets, booklets or other kinds of information. . An explanation of why a specific treatment is considered necessary for the individual patient, what specifically is involved: method, timing, duration, cost, what the specific goal is and possible side effects. 2. When a treatment is considered, the professional engaged in cleft care should take into consideration the desires and attitudes of the patient and/or those of the patient’s guardian. The professional should also pay attention to and inform the patient/patient’s guardian of the risks and benefits inherent in the potential alternative treatment options, including no treatment or no further treatment. 3. If requested, it is the professional’s responsibility to provide a procedure for obtaining a second opinion for the patient. If
The Eurocleft Project 1996–2000: overview 139 requested, this procedure should be communicated to the patient before treatment starts. 4. After an episode of treatment, the professional engaged in cleft care should inform the patient and/or patient’s guardian on: . Outcome of treatment relative to the defined goal. . Undesirable effects of treatment. . Expected future development. 5. The professional engaged in cleft care should analyse and document any complaints or praise expressed by the patient and/or the patient’s guardian. 6. The professional engaged in cleft care should give consideration to the burden of the treatment: . Considerations should include financial as well as nonfinancial burden, such as treatment duration, effort from the patient and/or patient’s guardian and discomfort as a result of treatment. 7. During the process of treatment, the professional involved in cleft care should continuously evaluate treatment progress against the planned treatment and act accordingly. 8. Organizations and institutes responsible for the provision of cleft care should: . Encourage the cleft professional to follow the policies described above and to acknowledge the patient’s rights. . Recognize and encourage the professional’s right to provide treatment that can be expected to improve the patient’s condition whilst minimizing adverse effects. . Recognize and encourage that decisions on treatment priority should be based on criteria proposed by the cleft professionals in consultation with the patient and/or patient’s guardian. This is especially so in a situation with insufficient treatment resources. . Recognize and encourage that access to treatment should not depend on the patient’s ability to pay. . Recognize that cooperation of the patient with the advice and instructions of the cleft professional is necessary in order to achieve a successful result.
6. Ear, nose and throat (ENT) ENT disorders should be identified at an early stage and the necessary therapy should be provided. 7. Clinical genetics/paediatric developmental medicine As cleft lip and/or palate may be associated with other anomalies early assessment and diagnosis is necessary. Genetic counselling for patients and families should be available. 8. Emotional support and professional advice for the growing child and its parents Emotional support and professional advice for parents, patients and their environment is often necessary and should be available. 9. Dental care Regular dental care should be available. 10. National register A national register should be in place for accurate recording of children born with cleft lip and/or palate and related craniofacial anomalies.
Part II: Organization of services 1. Cleft care should be provided by a multidisciplinary team of specialists. 2. Members of the team should have special training in cleft care. 3. The team should agree on the stages of treatment including the examination, record collection and general protocols. 4. There should be one person responsible for quality improvement and communication within the team. 5. Coordination of the care of individual patients is important since numerous specialities are involved. This should be the responsibility of one member of the team. 6. The number of patients referred to the team should be sufficient to sustain the experience and specialist skills of all team members and to allow evaluation/audit of the team’s performance within a reasonable period of time. It has been recommended that cleft surgeons, orthodontists and speech therapists should treat at least 40–50 new cases annually. However, it is recognized that individual member states have the right to provide care for their own population.
APPENDIX II – Practice Guidelines Part I: Healthcare needs
Part III: Finances
1. Neonatal emotional support and professional advice In the event of prenatal diagnosis and as soon as possible after the birth of a child with a cleft parents should be given emotional support and advice about the child’s future management by a specialist in cleft care.
Resources should be available to cover the following care for children with cleft lip and palate:
2. Neonatal nursing Difficulties in feeding are common in the early days of life and specialist advice on feeding should be provided. 3. Surgery Primary surgery to close clefts of the lip and/or palate should be performed by an experienced and qualified surgeon according to a protocol agreed by the team. Further corrective procedures may be necessary for some patients in later years and should be performed by an experienced and qualified surgeon according to a protocol agreed by the team 4. Orthodontic/orthopaedic treatment For children with cleft lip and palate orthodontic/orthopaedic treatment should be available when necessary and should be performed by an experienced orthodontist. 5. Speech and language therapy Early assessment of speech and language problems, advice to parents and the availability of corrective therapy by an experienced speech and language therapist should be provided.
1. Emotional support and professional advice during the neonatal period. 2. Neonatal nursing. 3. Surgery. 4. Orthodontic/orthopaedic treatment. 5. Speech and language assessment and therapy. 6. Ear, Nose and Throat treatment. 7. Clinical genetics/paediatric developmental medicine. 8. Emotional support for the growing child and its parents. 9. Travel expenses. 10. General dental care including cleft related prosthodontics.
APPENDIX III – General Principles Governing Record Taking 1. Records for treatment planning/monitoring . Clinical records should be taken for individual patients to allow treatment planning, monitoring treatment progress and treatment evaluation.
140 Journal of Cranio-Maxillofacial Surgery . The timing and nature of these records will depend on the clinical protocols followed by individual teams. . Treatment and associated record taking protocols should be agreed and clearly set out by the cleft team.
2. Cleft Palate Only (CPO) Timing
2. Records for quality improvement/research Additional records may be taken for a number of other reasons: . Follow up of a series of patients to provide an overview of the outcome of care. . To allow retrospective comparisons of different protocols. . As part of a prospective clinical trial with ethical approval. . As part of an agreed protocol for intercentre quality improvement comparisons or comparison against known standards. . As part of an agreed research protocol. . Other reasons such as medico-legal, second opinion. 3. Safeguards . Exposure of patients to unnecessary radiation should be avoided. . Research and quality improvement records should only be taken when there is an established written protocol on how they will be put to use. . Research and quality improvement records should not be taken without the consent of the patient/parent/guardian. . Research and quality improvement records should coincide as far as possible with the records for treatment planning/ monitoring (Statement 1).
TIMING OF MINIMUM RECORDS
Models Lateral Photo- Speech Audioskull graphs metry/ radiotympanograph metry
Primary H surgery 3 Years 5/6 Years H 15/16 Years H
Patient/ parent satisfaction
H
H
H H H
H
H H H
H
3. Clift Lip Only Timing
Models
Photographs
Primary surgery 5/6 Years 18+Years
H*
H
H*
H H
Patient/ parent satisfaction
H
*Only in cases with cleft of the alveolus as well as cleft lip.
4. Alveolar Bone Grafting Timing
Intra-oral radiograph
Photographs
Just before bone graft 6 months after graft After canine fully erupted
H H H
H H
1. Complete Cleft Lip, Alveolus and Palate (UCLAP & BCLAP) Timing
Primary surgery 3 Years 5/6 Years 10 Years 18+Years
Models Lateral Photo- Speech Audioskull graphs metry/ radiotympanograph metry H
H
H H H
H H H
H H
*If hard palate is closed.
H* H H H
Patient/ parent satisfaction
H* H H
5. Pharyngoplasty Timing
Speech sample
Just before operation One year after operation
H H
6. Orthognathic Surgery H
Timing
Lateral cephalogram
Models
Just before operation One year after operation
H H
H H