General introduction

Rationale behind the creation of this clinical practice guideline

Clefts of the lip, alveolus, and/or palate (CLA/Ps) are one of the most commonly occurring congenital abnormalities in The Netherlands, with a prevalence of about 16.6 per 10,000 live births per year (Luijsterburg and Vermeij-Keers, 2011). CLA/Ps differs from many other congenital defects due to the fact that effective and lasting treatment is available, which is generally finite around age 22. Children with an isolated cleft generally have the similar social chances and opportunities as children without a cleft.

Following the examples set by The United Kingdom and the Scandinavian countries, the care of CLA/Ps in the Netherlands is offered by a limited number of multidisciplinary cleft teams. In 2021, the care offered by a cleft team usually stretches from antenatal care or care at birth to the age of approximately 22 years. Transitional care by the teams into adulthood is mandatory. Preferably cleft teams should remain available for lifetime consultation and service for their patients.

The first initiative to create this multidisciplinary guideline was made when advancement of ultrasound technology and new legislation regarding centralisation of prenatal screening in the Netherlands (Advies Wet bevolkingsonderzoek: prenatale screening op downsyndroom en neuralebuisdefecten 17 december 2007) forced cleft teams to rethink their role in prenatal counselling for CLA/Ps. Consequently, a multidisciplinary clinical guideline regarding prenatal counselling for CLA/Ps was completed in 2009. Upon completing this latter guideline in 2009, it was decided that a second guideline should be composed on the postnatal trajectory. This postnatal guideline, including several additional modules, was eventually completed and authorized in 2018.

National clinical guidelines in the Netherlands are updated every 5 years (if advancement of scientific evidence mandates such a procedure). In 2019 funds became available to update the prenatal guideline on CLA/Ps and check and extend the postnatal guideline. This provided the opportunity to combine both guidelines - the prenatal and the postnatal guideline - into one comprehensive evidence based clinical practise guideline on CLP. It will be stated per module whether it was either a newly drafted module or a revised module from an earlier version. A new working group was formed for this new project, which was partly comprised of people who previously worked on these guidelines, as well as new representatives from various scientific associations and patient/parent groups.

Key issues were analysed prior to the establishment of both the orgininal prenatal (2011) and the postnatal guideline (2018). The main key issue turned out to be the large variation in practices between CLA/Ps care teams, which was considered confusing and undesirable by both patients and care givers. The new working group assessed the key issues again at the start of the development of this guideline. It turned out that significant practice variation between cleft teams in the Netherlands still is existed in 2019.

Patients and parents recognize that differences in practices on one hand can be explained by the fact that the treatment of CLA/Ps should remain tailormade for the individual patient, yet on the other hand they experience differences in protocols between teams as frustrating as they cannot judge which protocol is best or most suitable for an individual child. For both patients and parents, it is very important that the professionals should work together to determine what they see as the scientific foundation for their medical actions and define the standard of care the patient and parents may expect. Patient representatives from the patient organisation ‘Schisis Nederland’ participated in the working group and were involved in every step of the guideline development.

In summary, patients and parents have expressed their desire to identify and diminish variations in practices based on scientific evidence. Moreover, the availability of high quality and trustworthy information about treatments should be improved.

Objectives of the guideline

The aim of this guideline is to improve the care of children/patients with CLA/Ps in The Netherlands ranging from prenatal detection to young adulthood, substantiated by scientific knowledge from research where possible. ‘Improving’ also means providing insight in the differences in practices between cleft teams and discriminating between wanted and unwanted (i.e. scientifically based or non-scientifically based) practice variation. This resulted in recommentations for a more uniform treatment. However, the lack of high-quality studies and evidence remains a serious limiting factor and forced the working group to define some conclusions in a more generalized way than was wished for at the start.

Specific attention will be given to the following aspects:

reducing undesirable/unfounded practice variation in the working method and treatment protocols of the Dutch cleft teams, without hampering custom work, innovation or research;
making objective / evidence-based information about the treatment of CLA/Ps available and accessible to healthcare providers, patients, parents and other parties;
determine to what extent the existing organisation of care needs to be changed in order to meet the requirements regarding “state of the art” treatment of a child or adult with CLA/Psand the follow-up to this treatment.

In this manner, the guideline offers a tool to create more uniform care in the field of the prenatal and postnatal treatment of a child with CLA/Ps and the implementation of this care in the Netherlands.

Delineation of the guideline

The guideline focuses primarily on the treatment of patients with isolated CLA/Ps, including the following categories: a cleft lip with or without a cleft alveolus (cheilognathoschisis; cheiloschisis; CL±A), cleft lip, alveolus and palate (cheilognathopalatoschisis; CLAP) and a cleft palate (palatoschisis; CP), without other anomalies (as seen on ultrasound screening during pregnancy) in the age range from 0 to 22 years. The literature that was consulted for this guideline was limited to these categories of cleft.

The last 12 years of the Dutch national cleft registry by the Nederlandse Vereniging voor Schisis en Craniofaciale Afwijkingen (NVSCA) have yielded on average n = 323 new unoperated patients registered per year (2016). These patients can be divided as follows across the three categories of orofacial clefts: cleft lip with or without cleft alveolus (25%), cleft lip, alveolus and palate (40%) and cleft palate (35%).

Many of these recommendations will also apply to patients with clefts of the lip and/or palate in combination with other anomalies - whether they form part of a syndrome or not - though the recommendations may need to be adjusted per patient according to the underlying disease or situation.

A wide range of interventions and treatments related to CLA/Ps take place in the period from birth to the age of 22 years. The guideline task force was forced to prioritise and to select in order to fit the amount of work within the available financial resources. The following factors were taken into consideration in the selection of the topics for this guideline:

The wishes from the patients’ perspective regarding the need for insight into and - where possible - reduction of undesirable practice variation between cleft teams (for example, variations in feeding, the time of closure of the soft palate or the best technique to close the cleft in the alveolar arch, postoperative management).
The wishes from the treating physician’s perspective regarding variations in treatment protocols; particularly creating insight into the differences between teams leading to an improved understanding.
Relevance of problems for children and parents (for example, feeding as a primary necessity of life after birth).
Rapid technological developments that need to be acted upon (for example, advancements in clinical genetic diagnostics).

The following list of topics will be discussed in this guideline (version 2021): prenatal diagnostics and counselling (update 2021), genetic diagnostics (update 2021), feeding (after birth and after surgery) (new module 2021), naso-alveolar moulding (NAM), lip and/or palate closure (timing and technique) (update 2021), dentistry, hearing problems, hypernasality (diagnostics and treatment), bone grafting procedure (timing and technique), orthodontics (ventral traction and retention), nose corrections, surgical corrections of the maxilla (orthognathic surgery), and psychosocial care. In addition, a module has been included in the guideline about the organisation of care for CLA/Ps in the Netherlands.

Intended users of the guideline

The guideline is primarily intended for all healthcare professionals who are involved in caring for a child with CLA/Ps: general practitioners, midwives, gynaecologists, paediatricians, ENT physicians, plastic surgeons, maxillofacial surgeons, orthodontists, clinical geneticists, specialised nurses, speech therapists, (paediatric) dentists, medical psychologists, remedial educationalists and social workers. The secondary target group involves patients, parents and their surroundings.

Literature

Luijsterburg AJ, Vermeij-Keers C. Ten years recording common oral clefts with a new descriptive system. Cleft Palate Craniofac J. 2011;48:173–182.