Medische deel counseling bij prenatale schisis

Beoordeeld: 26-11-2021

Uitgangsvraag

Welke medische onderwerpen zouden er aan de orde moeten komen tijdens de prenatale counseling aan ouders die een kind met een schisis verwachten (volgens ouders en professionals)?
Welke hulpmiddelen van zorgverleners zijn nuttig voor ouders in het proces van prenatale counseling van ouders die een kind met een schisis verwachten?

Aanbeveling

Bied ouders die zwanger zijn van een kind met een schisis prenatale counseling aan en laat de medische informatie over de schisis en de behandeling ervan gegeven worden door een of meerdere behandelaren (waaronder een chirurg en iemand met psychosociale expertise, zoals gedragsdeskundige of medisch maatschappelijk werker), die lid is van een schisisteam en die inzicht heeft in de problematiek en aantoonbare ervaring met de behandeling van kinderen met een schisis.

Informeer ouders over medische onderwerpen met betrekking tot het kind met schisis in de prenatale counseling zoals:

Wat is een schisis.
Hoe vaak komt het voor.
Ontstaanswijze.
Vormen van schisis.
Kenmerken.
Bijkomende anomalieën en genetische aandoeningen (bespreek indien nodig verwijzing klinische genetica).
Functionele gevolgen.
Behandeling door een team van verschillende specialisten.
Plaats van bevalling.
(Borst)voeding en verzorging.
Behandeling.
Behandelprotocol.
Operaties.
Pijn en ongemak voor het kind.
Controles bij het schisisteam.
Gehoor.
Spraak.
Gebit en kaken.
Tijdpad behandeling.
Eindresultaat (ook beperkingen zoals littekens).

Zorg dat de zorgverlener de mondelinge informatie ondersteunt met schriftelijk en digitaal informatiemateriaal, waaronder:

Informatieboekje van het schisisteam.
Pre- and postoperatief beeldmateriaal.
Ander beeldmateriaal dat de mondelinge informatie ondersteunt, bijvoorbeeld in de vorm van een PowerPoint presentatie.
Hulpmiddelen bij de voeding, zoals typen zuigfles en speen.

Wijs de ouders op websites die objectieve informatie verstrekken.

Overwegingen

Advantages and disadvantages of counselling

Unfortunately no studies were found in this search which evaluated the effectivity of counselling. It was therefore impossible to answer the PICO question. Nevertheless, a few studies were found that provided useful information about the medical and psychological themes and tools that should be mentioned during the counselling of parents expecting a child with CLA/P (Greives, 2017; Stocka, 2019; Stockb, 2019).

1. What medical information should be addressed during prenatal counselling to parents who are expecting a child with a cleft lip, alveolus and/or palate (according to parents and professionals)?

Several studies were found in which parents gave their opinion on the topics to be addressed during counselling for prenatal diagnosed clefts of the lip and/or palate. The study of Berk (1999) showed that 87% (84/97) of the parents indicated a need for medical information about the disorder and its treatment. Other topics that parents need in counselling are aetiology, chance of additional anomalies, expected medical problems after birth, such as feeding, and treatment (Maes, 1998; Hager, 2002; Jones, 2002; Nusbaum, 2008; Matthews, 1998). The online survey study of Greives, 2017 asked parents (n=112) about the topics that are the most helpful to be discussed during the prenatal visit. The top five consisted of 1. surgical treatments; 2. new-born feeding techniques; 3. team care; 4. genetics; and 5. speech/hearing. Jones (2002) states that, in addition to providing a general overview of treatment, counselling should mainly focus on issues that play a role in the first year of life such as feeding, surgical interventions, ear problems and pain.

The Royal College of Obstetricians and Gynaecologists (RCOG) guideline about prenatal information state that all practitioners performing fetal anomaly screening should be trained to impart information, and that they or another health professional should be available to provide immediate support to both parents (RCOG, 2010). Further, that all staff involved should adopt a non-directive, non-judgmental approach, and should not assume, even in the presence of a potentially fatal condition, that the parents will choose to terminate (RCOG, 2010).

Prospective parents could therefore benefit from a comprehensive description of the aetiology, likely treatment pathway and prognosis, without health professionals introducing their own values and judgements and/or exceeding their own professional capacity. A referral to cleft specialist should be made soon after prenatal diagnosis, and prospective parents should be directed to a reliable parents/patient organization as soon as possible; Schisis Nederland (https://schisisnederland.nl) in the Netherlands.

Referrals to cleft specialists would provide prospective parents with further information, emotional support, and realistic personal accounts of what life is like with a child who was born with CLA/P, so that they can decide whether further testing and/or termination of pregnancy (TOP) is something they want to pursue. Supplementary written information and trusted website addresses (see below) could also be provided, particularly given that prospective parents may be emotionally distressed and less able to process complex information in the moment.

Trusted websites (May 2021):

Parent/patient organization: www.schisisnederland.nl

Information about cleft lip, alveolus and/or palate and the Dutch cleft teams: https://www.schisis.nl/nvsca2/

Thuisarts – patient information: www.thuisarts.nl

Information about genetic testing: http://www.erfelijkheid.nl

Stock (2019a) analysed data from 217 parents of children born with cleft lip (with or without cleft palate) using a mixed-methods online survey. If a cleft lip, alveolus and/or palate is detected, the way the diagnosis is communicated can considerably influence prospective parents' decision making regarding further testing and TOP. Due to unsatisfactory information and a perceived lack of empathy from health professionals, respondents reported feeling upset, offended and/or anxious following the diagnosis. Some respondents had felt under pressure to decide quickly, with a minority having regretted undergoing amniocentesis, and / or experiencing significant distress at having come close to TOP unnecessarily. Unfortunately, the legal limit of a certain gestational age to terminate a pregnancy, might feel parents under pressure to make decisions quickly (Stock, 2019a).

We need to consider that the above-mentioned survey was only shared with parents who are part of CLAPA’s community (Cleft Lip and Palate Association UK). While CLAPA’s community is considerable, it cannot be assumed that this group, nor the subgroup who responded to the survey, are representative of the CLA/P population in the Netherlands or as a whole. Since all participants were parents of children with CLA/P, none of the survey respondents had opted for TOP. It is therefore not possible to gain an understanding of the experiences of those who opted to end their pregnancy on the basis of a CLA/P (with or without associated conditions).

The quality of the information received at the time of diagnosis can considerably influence parents’ long-term well-being, as well as their attitudes toward the diagnosis, further antenatal testing, and even TOP (Stock, 2019a).

Stock (2019a) demonstrated their findings emphasize the importance of providing accurate and individualized information to prospective parents, in a sensitive manner, so they can adjust to their child’s diagnosis, consider further genetic testing and TOP or prepare for the birth appropriately (Stock, 2019a). Given that antenatal screening for cleft lip is becoming more fully integrated into routine practice in the Netherlands more training for health-care professionals, improved access to reliable information in a variety of formats, and stronger links between local midwifes doing the primary 20-week structural anomaly scan (SEO) and specialist from cleft team may be needed (Stock, 2019a based on the British situation).

2. What resources from caregivers are useful in the process of prenatal

counselling for parents expecting a child with cleft lip, alveolus and/or palate (resources such as information leaflets and images, but also practical tools like teats and other aids)?

Four studies were found that provided information on the use of aids in counseling parents expecting a child with CLA/P. In the study of Rey-Bellet (2004) 93% (27/29) of parents were happy to see pictures of children with clefts before and after surgery. Pictures were also an aid to help family and friends prepare for the arrival of a child with CLA/P. In the study of Matthews (1998) 1/9 parents considered the pre- and postoperative pictures of a child with clefts to be too severe.

The following medical topics related to the child with CLA/P should be discussed with the parents in prenatal counseling:

What is a cleft lip, alveolus and/or palate.
How often does it occur.
How does it develop.
Types of cleft lip, alveolus and/or palate.
Characteristics.
Additional anomalies.
Functional consequences.
Treatment by a team of different specialists.
Nutrition and care.
Treatment and treatment protocol.
Operations /surgery.
Pain and discomfort for the child.
Out-patient visits to the cleft team.
Possible hearing problems.
Possible speech problems.
Teeth and jaw outgrouth problems.
Timeline of treatment.
End result of treatment (including limitations such as scars).

Parents' opinions about the internet as a source of information vary. Rey-Bellet (2004) found that 17% (5/29) of parents thought it was a good way to find information. However, the pictures published on the internet are perceived by some as scary and because people search at home, this source does not offer the possibility of direct feedback from a healthcare provider (Jones, 2002; Nusbaum, 2008). It is also customary to provide written information.

Values and preferences of patients' parents

It is clear that parents have a great need for information. The literature does not indicate what the professional background of the care provider who provides the information should be. It seems justified from studies from Rey-Bellet (2004) that persons experienced in surgical aspects are important, however the healthcare provider(s) in question should have knowledge and expertise in the whole field of treatment of CLA/Ps. The topics that should be addressed in prenatal counseling previously summarized do not seem to differ from those that are routinely used by the majority of (unprepared) parents in the immediate postnatal phase. The recommendations regarding the topics are based on this experience. It seemed that parents were more interested in short-term issues for their child rather than long-term goals for overall cleft care (Rey-Bellet, 2004).

Costs (resources required)

The ultrasound (including a consultation with a gynecologist) is reimbursed under the basic insurance. Prenatal counseling is however not covered under the basic insurance in the Dutch Health Care system and there may be additional - non-reimbursable - costs for parents due to prenatal consultation with a cleft team. We feel this should not be a reason not to refer patients to a cleft team, however, for some patients this could be a consideration not to start counseling until after birth (in the Netherlands).

Acceptability, feasibility and implementation

Clinical experience has shown that both parents and caregivers seem satisfied with prenatal counseling. It gives parents the space to make choices based on good information. There are no practical restrictions in planning an antenatal consultation with a cleft team. This is available in all locations in the Netherlands. Prenatal counseling by the cleft team has already been implemented in all academic centers.

Rationale

The working group would like to emphasize the importance of providing accurate and individualized information to prospective parents. This should be done in a sensitive manner, so the parents can adjust to their child’s diagnosis, and consider further genetic testing and TOP or prepare for the birth appropriately. It is considered important that every parent should speak to a delegation from a cleft team before a possible TOP will take place. Given that antenatal screening for cleft lip is becoming more fully integrated into routine practice in the Netherlands, access to reliable and accurate information in a variety of formats, should be freely available. The working group recommends that prenatal counseling for clefts of the lip and/or palate should always be performed by one or two representatives of a cleft team, preferably including a surgeon and a psychologist or social worker. A close work relation with the obstetric caretaker is an important factor, so that the counselling can be individualized and based on the ultrasound findings. From September 2021 pregnant woman in the Netherlands are offered a 13-week scan as part of research. Woman can only opt to have the 13-week scan if they participate in the scientific IMITAS study. This study is investigating the advantages and disadvantages of the 13-week scan. Possible influence of this opportunity of this scan on the prenatal counseling is not included in the considerations and recommendations yet.

Finally, it might be considered to provide parents with an unborn child with a cleft on ultrasound with the opportunity to speak to other parents that have a child with CLA/P. In this way parents could get firsthand information, but it may also introduce possible bias. It is possible that the patients’ parents’ organizations could play an important role in providing peer support. Parents who are willing to act as peer support should receive special training for this purpose.

Onderbouwing

Achtergrond

Since the introduction of the structural anomaly scan (SAS second trimester) as a routine screenings tool for all pregnant women in the Netherlands in 2007 and the ongoing technological improvement of the ultrasound machines, the prenatal detection of cleft lip, alveolus and/or palate (CLA/P) has increased over the years (Ensing, 2014). From September 2021 pregnant woman in the Netherlands have the opportunity to do a 13-week scan (in a research setting) in addition to the 20-week scan. Prenatal detection allows parents to receive detailed information about the short- and long-term implications of the diagnosis before birth. The goal of the prenatal counselling is to give parents accurate, uniform, and unambiguous information about the possible consequences of the diagnosis of a cleft of the lip and/or palate and future opportunities for their child and to prepare them for birth and the time until the first visit to a cleft clinic. The counselling should include medical as well as psychological information, and tools to prepare parents and their families for the birth of a child with CLA/P. If diagnosed before 24 weeks of gestation, termination of pregnancy (TOP) is an option that should be mentioned in the Netherlands and should be discussed in more detail in case parents wish to do so. Questions regarding a possible underlying genetic cause and prenatal genetic diagnostic testing are usually directed to a medical geneticist in the Dutch health care system (referral by the obstetrician) and can be of influence for possible TOP.

In the Netherlands, the care for patients diagnosed with CLA/Ps is concentrated in multidisciplinary cleft teams. There is however variation in both prenatal counselling and postnatal care between individual teams.

Zoeken en selecteren

A systematic review of the literature was performed to answer the following question:

What is the effect of prenatal medical counselling on parents of patients with cleft lip, alveolus and/or palate?

P: parents of patients with cleft lip, alveolus and/or palate;

I: prenatal medical counselling;

C: no counselling;

O: satisfaction, anxiety/concerns, informed choice, termination of pregnancy.

Relevant outcome measures

The working group considered satisfaction as a critical outcome measure for decision making; and anxiety, informed choice, termination of pregnancy as an important outcome measure for decision making.

A priori, the working group did not define the outcome measures listed above but used the definitions used in the studies.

The working group defined the criteria for minimal clinically (patient) important difference for the dichotomous outcome measures; RR < 0.80 of > 1.25) or Standardized mean difference (SMD=0.2 (small); SMD=0.5 (moderate); SMD=0.8 (large).

Search and select (Methods)

The databases Medline (via OVID), Embase (via Embase.com), and Psychinfo were searched with relevant search terms until 14^th of April 2020. The detailed search strategy is depicted under the tab Methods. The systematic literature search resulted in 317 hits (16 SR, 21 RCTs, 113 observational studies, and 167 others). Studies were selected based on the following criteria: systematic reviews, RCTs and other comparative studies focusing on medical and/or psychological counselling for parents of patients with CLA/P. A total of 16 studies were initially selected based on title and abstract screening. After reading the full text, all 16 studies were excluded (see the table with reasons for exclusion under the tab Methods), thus no comparative studies were selected for the literature summary.

Results

No comparative studies were found that answered the question: What is the effect of counselling on parents of patients with cleft lip, alveolus and/or palate?

Referenties

Berk NW, Marazita ML, Cooper ME. (1999). Medical genetics on the cleft palate-craniofacial team: understanding parental preference. Cleft Palate Craniofac J; 36(1):30-35.
Ensing S, Kleinrouweler CE, Maas SM, Bilardo CM, Van der Horst CM, Pajkrt E. (2014). Influence of the 20-week anomaly scan on prenatal diagnosis and management of fetal facial clefts. Ultrasound Obstet Gynecol;44(2):154-9. doi: 10.1002/uog.13291. PMID: 24375841.
Greives MR, Anderson CL, Dean RA et al. (2017). Survey of Parent Experiences in Prenatal Visits for Infants With Cleft Lip and Palate. Cleft Palate Craniofac J;54(6):668-673.
Hager C. (2002). Termination of pregnancy with a prenatal diagnosis of cleft lip: cultural differences and ethical analysis. Plast Surg Nurs;22(1):24-28.
Jones MC. (2002). Prenatal diagnosis of cleft lip and palate: detection rates, accuracy of ultrasonography, associated anomalies, and strategies for counseling. Cleft Palate Craniofac J;39(2):169-173.
Maes S, Demey A, Appelboom-Fondu J. (1998). Impact of ultrasound for facial cleft on mother-child relationships. Ann N Y Acad Sci; 847:249-251.
Matthews MS, Cohen M, Viglione M, Brown AS. (1998). Prenatal counseling for cleft lip and palate. Plast Reconstr Surg;101(1):1-5.
Nusbaum R, Grubs RE, Losee JE, Weidman C, Ford MD, Marazita ML. (2008). A qualitative description of receiving a diagnosis of clefting in the prenatal or postnatal period. J Genet Couns;17(4):336- 350.
Rey-Bellet C, Hohlfeld J. (2004). Prenatal diagnosis of facial clefts: evaluation of a specialised counselling. Swiss Med Wkly;134(43-44):640-644.
Royal College of Obstetricians and Gynaecologists (RCOG). (2010). Pregnancy for Fetal Abnormality in England, Scotland and Wales. Report of a working party. https://www.rcog.org.uk/globalassets/documents/guidelines/terminationpregnancyreport18may2010.pdf.
Stock NM, Costa B, Williams J, Martindale A, At The Centre For Appearance Research TVFRT. (2019a). Parental views of antenatal testing and termination following a diagnosis of cleft lip. Psychol Health Med;24(4):456-469.
Stock NM, Costa B, Williams JR, Martindale A; VTCT Foundation Research Team at the Centre for Appearance Research. (2019b). Breaking the News: Parents' Experiences of Receiving an Antenatal Diagnosis of Cleft Lip. Cleft Palate Craniofac J;56(9):1149-1156.

Evidence tabellen

Table of excluded studies

Author and year	Reason for exclusion
Aslan, 2018	About the importance of counselling (variables affecting family functions and life quality of parents), not about counselling itself
Dabadie, 2016	Prospective study comparison of MRI and ultrasound in diagnosing CLA/P
De Cuyper, 2019	Cross sectional study about the effect of CLA/P on quality of life of parents supported by a multidisciplinary cleft team
Greives, 2017	Survey study about factors influencing the choice of parents of their cleft team
James, 2016	Descriptive study about the importance of cleft teams
Laifer-Narin, 2019	Retrospective study of a fetal MRI database examining the accuracy of fetal MRI for prenatal diagnosis
Loozen, 2015	Retrospective cohort study to the accuracy of prenatal transabdominal ultrasound
Maarse, 2018	Survey study about psychosocial and moral considerations of prospective parents
Nidey, 2016	Study about predictors of psychosocial wellbeing and compared fathers and mothers
Shibui, 2016	Description of the counselling protocol in Japan
Sreejith, 2018	Descriptive review about psychological aspects of prenatal diagnosis
Steinberg, 2015	Comprehensive review about what have been changed in the last 30 years in counselling
Stock, 2019a	Mixed method online survey to explore the decision-making process from the parents’ perspective
Tang, 2016	Centre's experience in providing one-stop multidisciplinary antenatal counselling service to parents of fetus with cleft lip/palate deformity on termination.
Zeytinoglu, 2015	Thesis
Zeytinoglu, 2017	Descriptive qualitative interview study about CLA/P diagnosis on couples’ relational adjustment and coping

Verantwoording

Autorisatiedatum en geldigheid

Laatst beoordeeld : 26-11-2021

Laatst geautoriseerd : 26-11-2021

Geplande herbeoordeling : 01-01-2027

The Board of the Dutch Society for Plastic and Reconstructive Surgery (NVPC) will assess whether this guideline module is still up-to-date in 2026 at the latest. If necessary, a new working groupwill be appointed to revise the guideline module. The validity of the guideline or modules of the guideline may lapse earlier when new developments arise. As the holder of this guideline, the NVPC is chiefly responsible for keeping the guideline up to date.

Module¹

Responsible party²

Year of autorisation

Next assessment of actuality guideline³

Frequency of assessement of actuality⁴

Supervisor of actuality⁵

Relevant factors for changes in recommendations⁶

Prenatal medical

counselling

NVPC

2021

2026

every 5 years

NVPC

None

^[1] Name of module

² Responsible party for the module

³ maximum of 5 years

⁴ half a year, every (other,..) year

⁵ supervising party or parties

⁶ Current reseach, changes in organizations/restitions, new available rescourses

Other scientific organizations participating in the guideline or users of the guideline share the responsibility to inform the chiefly responsible party (NVPC) about relevant developments within their fields.

Initiatief en autorisatie

Initiatief:

Nederlandse Vereniging voor Plastische Chirurgie

Geautoriseerd door:

Nederlandse Vereniging voor Keel-Neus-Oorheelkunde en Heelkunde van het Hoofd-Halsgebied
Nederlandse Vereniging voor Kindergeneeskunde
Nederlandse Vereniging voor Obstetrie en Gynaecologie
Nederlandse Vereniging voor Plastische Chirurgie
Vereniging Klinische Genetica Nederland
Nederlands Instituut van Psychologen
Nederlandse Vereniging voor Mond- Kaak- en Aangezichtschirurgie
Nederlandse Verenging voor Schisis en Cranio Faciale Afwijkingen
Nederlandse Vereniging van Orthodontisten

Algemene gegevens

The revision of this guideline module was supported by Knowledge Institute Federation of Medical Specialists (https://www.demedischspecialist.nl/kennisinstituut) and was financed by the Quality Foundation of the Dutch Medical Specialists (SKMS). The funding organization did not have any influence on the content of the guideline in any way.

Doel en doelgroep

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.

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.

Samenstelling werkgroep

A multidisciplinary working group was appointed by the Dutch Society for Plastic and Reconstructive Surgery in October 2019 to update the existing guidelines for clefts of the lip and palate. The original guidelines were initiated by the Dutch Society for Plastic and Reconstructive Surgery and this Society remains responsible for the revisions. The working group subsequently updated both the guideline for prenatal counsellling for clefts of the lip, alveolus, and/or palate (Counseling na prenataal vastgestelde schisis, 2011) and postnatal treatment (Behandeling van patiënten met een schisis, 2018). The working group consisted of representatives from all relevant specialties involved in the care for patients with cleft lip, alveolus and/or palate. Members were mandated by their professional organizations. The working group consisted of a mix of new members and members, who worked on previous editions as well. The group worked on the update of the guideline for two years. The working group is responsible for the full text of this guideline.

Working group

Dr. A.B. Mink van der Molen, MD, plastic surgeon, Universitair Medisch Centrum Utrecht, (chairman), NVPC
Dr. M.F. van Dooren, clinical geneticist, Erasmus MC Rotterdam, VKGN
Dr. M.J.H. van den Boogaard, clinical geneticist, Universitair Medisch Centrum Utrecht, VKGN
Dr. L.N.A. van Adrichem, MD, plastic surgeon, Universitair Medisch Centrum Utrecht, NVPC
Dr. H.F.N. Swanenburg de Veye, psychologist, Universitair Medisch Centrum Utrecht/Wilhelmina Kinderziekenhuis, Utrecht, NIP
Dr. C.J. Bax, MD, gynaecologist, Amsterdam UMC, NVOG
Prof. dr. C.C. Breugem, MD, plastic surgeon, Amsterdam Medical Center and Meander Medical Center, NVSCA
Drs. F. Bierenbroodspot, MD, Oral and maxillofacial surgeon, Isala, Zwolle, NVMKA
Drs. M. Haasnoot, MD, paediatrician, Wilhelmina Kinderziekenhuis, Utrecht, NVK
Drs. H.H.W. de Gier, MD, otolaryngologist, Erasmus MC Rotterdam, NVKNO
Dr. M.A.R Kuijpers, orthodontist, Radboud University Medical Center, NVvO
Dr. M.E.L. Nienhuijs, MD, Oral and maxillofacial surgeon, Radboud University Medical Center, NVSCA
Dr. D. de Haan, patient representative, Schisis Nederland

Advisory board

Drs. B. Spaan, dentist, CBT Vogellanden Zwolle, NVvK
I. Noureldin - Hop, orthopedagogue, NSDSK, NVO
M. J. Coerts, speech therapist, Amsterdam UMC, NVLF

With methodological support of

Drs. A.A. Lamberts, senior advisor, Knowledge Institute Federation of Medical Specialists
Dr. M. den Ouden - Vierwind, advisor, Knowledge Institute Federation of Medical Specialists

Belangenverklaringen

According to the KNMG-code, all members of the working group have declared in writing if, in the last five years, they have held a financially supported position with commercial businesses, organisations or institutions that may have a connection with the subject of the guidelines. Enquiries have also been made into personal financial interests, interests pertaining to personal relationships, interests pertaining to reputation management, interests pertaining to externally financed research, and interests pertaining to valorisation of knowledge. These Declarations of Interest can be requested from the secretariat of the Knowledge Institute of Medical Specialists. See below for an overview.

Member	Profession	Side jobs	Declared conflicting interests	Actions
Dr. A.B. Mink van der Molen	plastic surgeon,	None	None	No actions
Dr. M.F. van Dooren	clinical geneticist	Co-chair VKGN	None	No actions
Dr. M.J.H. van den Boogaard	clinical geneticist	None	None	No actions
Dr. L.N.A. van Adrichem	plastic surgeon	DGA van Adrichem Medical B.V. Chairman Concilium plastico chirurgicum Member Raad Opleiding Member BBC NVPC Advisor Hoofdmaatje Chairman Medical Council Equipe Zorgbedrijven Member Medicatie Commissie Equipe Zorgbedrijven Member stuurgroep STW project TU-Twente	None	No actions
Dr. H.F.N. Swanenburg de Veye	psychologist	None	None	No actions
Dr. C.J. Bax	gynaecologist	Volunteer hospice Member NIPT consortium Member committee quality documents NVOG Secretary committee Otterlo NVOG Treasurer working group infectious diseases NVOG	None	No actions
Dr. C.C. Breugem	plastic surgeon	None	None	No actions
Drs. F. Bierenbroodspot	Oral and maxillofacial surgeon	Working Group Esthetische Aangezichtschirurgie	None	No actions
Drs. M. Haasnoot	paediatrician	None	None	No actions
Dr. J. de Gier	otolaryngologist	Board member NVSCA	None	No actions
Dr. M. Kuijpers	orthodontist	Guideline committee Mondzorg voor jeugdigen preventie diagnostiek behandeling	None	No actions
Dr. M. Nienhuijs	Oral and maxillofacial surgeon	Boardmember NVSCA	None	No actions
Dr. D. de Haan	patient representative,	Teacher/ education advisor HU-PABO, Hogeschool Utrecht	None	No actions

Inbreng patiëntenperspectief

Patients were represented by Schisis Nederland. Schisis Nederland is an independent organization representing patients with CLA/Psand their parents in the Netherlands. Representatives from Schisis Nederland participated in the working group. The concept guideline module was presented to Schisis Nederland for their comments.

Qualitative estimation of possible financial impact under the Wkkgz / Kwalitatieve raming van mogelijke financiële gevolgen in het kader van de Wkkgz

In accordance with the Wet kwaliteit, klachten en geschillen zorg (Wkkgz), a qualitative estimation has been made whether the recommendations in the guideline may lead to substantial financial consequences. In carrying out this assessment, guideline modules were tested in various domains (see the flow chart).

The qualitative estimate shows that there is probably no substantial financial impact, see table below.

Module	Results qualitative estimation	Elucidation
Medische deel counseling bij prenatale schisis	Likely no substantial financial impact	The recommendation(s) are not widely applicable (<5,000 patients) and are therefore expected to have no substantial financial consequences for public expenditure.

Methode ontwikkeling

Evidence based

Implementatie

Guideline implementation and practical applicability of the recommendations was taken into consideration during various stages of guideline development. Factors that may promote or hinder implementation of the guideline in daily practice were given specific attention. The guideline is distributed digitally among all relevant professional groups. The guideline can also be downloaded from the Dutch Society for Plastic and Reconstructive Surgery website: www.nvpc.nl, and the guideline website: www.richtlijnendatabase.nl. The implementation table can be found in the related products.

Werkwijze

AGREE

The guideline has been drafted in accordance with the requirements outlined in the ‘Guidelines 2.0’ report of the Guideline Advisory Committee of the Council on Science, Education and Quality (WOK). This report is based on the AGREE II instrument (Appraisal of Guidelines for Research & Evaluation II) (Brouwers, 2010), an instrument designed to assess the quality of guidelines with broad international support (Brouwers, 2010). The development of a evidence-based guideline module is described step-by-step in “Ontwikkeling van Medisch Specialistische Richtlijnen” of Knowledge Institute for Medical Specialists.

Inventory of the problem areas

During the preparation phase the working group used an inventory to find the problem areas. A report of this inventory can be found in the related products.

Primary questions and outcome measures

Based on the outcomes of the bottleneck analysis, the president and advisor formulated draft primary questions. These were discussed and defined together with the working group. Subsequently, the working groupdetermined which outcome measures were relevant for the patient for each primary question, examining both desired and undesirable effects. The working groupvaluated these outcomes based on their relative importance as crucial, important and unimportant.

Literature search and selection strategy

Specific search terms were used to identify published scientific studies related to each individual primary question in electronic databases like Medline, Cochrane, and Embase. Additionally, the references of the selected articles were screened for additional relevant studies. Studies offering the highest level of evidence were sought out first. Members of the working group selected articles identified by the search based on predetermined criteria. Theselected articles were used to answer the primary question. The searched databases, the search string or terms used during the search and selection criteria applied are listed in the module for each individual primary question.

Quality assessment of individual studies

Individual studies were assessed systematically based on predefined methodological quality criteria in order to assess the risk of biased study results. These assessments may be found in the column ‘Study quality assessment’ in an evidence table.

AMSTAR - for systematic reviews.
Cochrane - for randomized controlled trials.
ACROBAT-NRS - for observational studies.
QUADAS II - for diagnostic studies.

Summary of the literature

The relevant study results from all selected articles were presented clearly in evidence tables. The key findings from the literature are described in the literature summary. If studies were sufficiently similar in design, data were also summarized quantitatively (meta-analysis) using Review Manager 5.

Assessment of the level of scientific evidence

With regard to intervention questions, the level of scientific evidence was determined using the GRADE method. GRADE is short for ‘Grading Recommendations Assessment, Development and Evaluation’ (see http://www.gradeworkinggroup.org/)

GRADE distinguishes four grades of quality of evidence, i.e. high, moderate, low and very low. These grades indicate the degree of confidence in the conclusions in the literature ((Schünemann, 2013; Hultcrantz, 2017).

GRADE	Definition
High	There is a high degree of confidence that the true effect of treatment is very close to the estimated effect of treatment as reported in the conclusion in the literature. It is very unlikely that the conclusion drawn in the literature will change if further research is done.
Moderate	There is a moderate degree of confidence that the true effect of treatment is very close to the estimated effect of treatment as reported in the conclusion in the literature. It is possible that the conclusion drawn in the literature will change if further research is done.
Low	There is a limited degree of confidence that the true effect of treatment is very close to the estimated effect of treatment as reported in the conclusion in the literature. It is probable that the conclusion drawn in the literature will change if further research is done.
Very low	There is little confidence that the true effect of treatment is very close to the estimated effect of treatment as reported in the conclusion in the literature. The conclusion is very uncertain

According to the GRADE methodology the clinical decision threshold should play an important role in assessing the level of evidence (grading) in guidelines (Hultcrantz, 2017). To set the threshold all critical outcomes, and the considerations should be determined. The clinical decision threshold is not exactly the same as the Minimal Clinically Important Difference (MCID). In situations in which an intervention has no important disadvantages and low costs, the clinical decision threshold with regard to the efficiency of an intervention can be lower (closer to zero/ no effect) than MCID (Hultcrantz, 2017).

Formulation of conclusions

For interventions, the conclusion does not refer to one or more articles, but is drawn based on the body of evidence. The working group looked at the net benefits of each intervention. This was done by determining the balance between favourable and unfavourable effects for the patient.

With regard to questions about the value of diagnostic tests, harm or adverse effects, aetiology and prognosis, the scientific evidence is summarized in one or more conclusions, listing the level of evidence for the most relevant data.

Considerations

When making recommendations, scientific evidence was considered together with other key aspects, such as expertise of the group members, patient preferences, costs, availability of facilities and/or organizational aspects. Insofar as they are not part of the systematic literature review, these aspects are listed under ‘Considerations’. The considerations are written using a structured format based on the evidence-to-decision framework of the international GRADE Working Group, and part of the GRADE methodology (Alonso-Coello, 2016a; Alonso-Coello 2016b).

Formulation of recommendations

Recommendations provide an answer to the primary question and are based on the best scientific evidence available and the most important considerations. The level of scientific evidence and the importance given to considerations by the working group jointly determine the strength of the recommendation. In accordance with the GRADE method, a low level of evidence for conclusions in the systematic literature review does not rule out a strong recommendation, while a high level of evidence may be accompanied by weak recommendations (Agoritsas, 2017; Neumann, 2016). The strength of the recommendation is always determined by weighing all relevant arguments.

Preconditions (Organisation of care)

In the analysis of problem areas, the organisation of care (all those aspects that are preconditions for the provision of care) were explicitly taken into account. These aspects include coordination, communication, materials, financial means, work force and infrastructure. Preconditions that are relevant to the answering of a specific clinical question are part of the considerations related to that specific question.

Knowledge gaps

During the development of this guideline, systematic searches were conducted for research contributing to answering the primary questions. For each primary question, the working group determined whether (additional) scientific research is desirable.

Commentary and authorization phase

The draft guideline was submitted to the (scientific) organizations involved for comment. The guideline was also submitted to the following organizations for comment: Dutch College of General Practitioners (NHG), Healthcare Insurers Netherlands (ZN), The Dutch Healthcare Authority (NZA), the National Health Care Institute (ZINL), the Health Care Inspectorate (IGJ), Dutch Organisation of Hospitals (NVZ), Dutch Federation of Academic Hospitals (NFU), Dutch Organisation of Independent Clinics (ZKN), the Netherlands Patients Federation, Dutch Organisation of nurses and caregivers (V&VN), Dutch Association of Physician Assistants, and Collaborating Top Clinical Training Hospitals (STZ). Comments were collected and discussed with the working group. The draft guideline was updated and finalized by the working group based on the comments. The final guideline was submitted for authorization to the (scientific) organizations involved and authorized or approved by them.

Literature

Agoritsas T, Merglen A, Heen AF, Kristiansen A, Neumann I, Brito JP, Brignardello-Petersen R, Alexander PE, Rind DM, Vandvik PO, Guyatt GH. UpToDate adherence to GRADE criteria for strong recommendations: an analytical survey. BMJ Open. 2017 Nov 16;7(11):e018593. doi: 10.1136/bmjopen-2017-018593. PubMed PMID: 29150475; PubMed Central PMCID: PMC5701989.

Alonso-Coello P, Schünemann HJ, Moberg J, Brignardello-Petersen R, Akl EA, Davoli M, Treweek S, Mustafa RA, Rada G, Rosenbaum S, Morelli A, Guyatt GH, Oxman AD; GRADE Working Group. GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 1: Introduction. BMJ. 2016 Jun 28;353:i2016. doi: 10.1136/bmj.i2016. PubMed PMID: 27353417.

Alonso-Coello P, Oxman AD, Moberg J, Brignardello-Petersen R, Akl EA, Davoli M, Treweek S, Mustafa RA, Vandvik PO, Meerpohl J, Guyatt GH, Schünemann HJ; GRADE Working Group. GRADE Evidence to Decision (EtD) frameworks: a systematic and transparent approach to making well informed healthcare choices. 2: Clinical practice guidelines. BMJ. 2016 Jun 30;353:i2089. doi: 10.1136/bmj.i2089. PubMed PMID: 27365494.

Brouwers MC, Kho ME, Browman GP, et al. AGREE Next Steps Consortium. AGREE II: advancing guideline development, reporting and evaluation in health care. CMAJ. 2010;182(18):E839-42. doi: 10.1503/cmaj.090449. Epub 2010 Jul 5. Review. PubMed PMID: 20603348.

Hultcrantz M, Rind D, Akl EA, et al. The GRADE Working Group clarifies the construct of certainty of evidence. J Clin Epidemiol. 2017 Jul;87:4-13. doi: 10.1016/j.jclinepi.2017.05.006. Epub 2017 May 18. PubMed PMID: 28529184.

Medisch Specialistische Richtlijnen 2.0 (2012). Adviescommissie Richtlijnen van de Raad Kwalitieit. http://richtlijnendatabase.nl/over_deze_site/over_richtlijnontwikkeling.html.

Schünemann H, Brożek J, Guyatt G, et al. GRADE handbook for grading quality of evidence and strength of recommendations. Updated October 2013. The GRADE Working Group, 2013. Available from http://gdt.guidelinedevelopment.org/central_prod/_design/client/handbook/handbook.html.

Schünemann HJ, Oxman AD, Brozek J, et al. Grading quality of evidence and strength of recommendations for diagnostic tests and strategies. BMJ. 2008;336(7653):1106-10. doi: 10.1136/bmj.39500.677199.AE. Erratum in: BMJ. 2008;336(7654). doi: 10.1136/bmj.a139. PubMed PMID: 18483053.

Wessels M, Hielkema L, van der Weijden T. How to identify existing literature on patients' knowledge, views, and values: the development of a validated search filter. J Med Libr Assoc. 2016 Oct;104(4):320-324. PubMed PMID: 27822157; PubMed Central PMCID: PMC5079497.

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