Clinical Practice Guideline On Treatment Of Patients With Clefts Of The .

Chapter 5 Lip and palate closure in patients with clefts of the lip and palateModule 1: Timing of the lip and palate closurePerform surgical closure of the lip during the first 6 months of life.Define a preferred approach within the cleft team for lip closure and palate closure,in order to advise parents in the decision-making process, but also give parents theopportunity to discuss a different approach. Check prior to the operation whether the Eurocleft checklist is complete andperform intra-oral X-rays during the procedure if necessary and documentany abnormalities in the surgical report. Close only the soft palate during the first year of life and close the hard palateat a later stage if the aim is to achieve optimum growth of the maxilla. Close the hard palate and the soft palate during the first year of life if the aimis to achieve optimum speech development.Module 2: Technique for lip and palate closureDuring palate closure, move the palate musculature to a more anatomical position(connection placed in the medial line and more posterior) to achieve a better result forspeech, for example a Furlow or Langenbeck technique with transposition of themuscles.Do not use a Furlow extension-plasty in the case of a wide palate cleft, due to theincreased risk of fistula formation.Preferably do not use the Wardill-Kilner pushback technique.Use everting (suture) techniques to achieve improved wound margin approximation.Use a (combination of) technique(s) for palate closure that the surgeon is mostexperienced in, so that the risk of complications is kept to a minimum.Use a (combination of) technique(s) for lip closure that the surgeon is most experiencedin, to ensure an optimum result with regard to function and aesthetics while the risk ofcomplications is kept to a minimum.Chapter 6 Hearing problems in patients with clefts of the lip and palateAsk for the results of the neonatal hearing screening for each child with a cleft (lip,alveolar arch alveolar archand) palate.Check the hearing of children with a cleft (lip, alveolar arch alveolar arch and) palatethoroughly. The recommendation is to perform periodic testing in an audiological centreup to the age of 3-4 years, followed by testing by the ENT physician if indicated. Theseexaminations should be tailored to the surgical protocol.Check the hearing of children with a cleft (lip, alveolar arch and) palate at least oncepost-operatively after insertion of tympanostomy tubes, in order to rule out perceptivehearing loss (see Guideline Otitis media with effusion NVKNO, 2012).

Insert tympanostomy tubes in children with a cleft (lip, alveolar arch and) palate only ifindicated (see Guideline Otitis media with effusion) and take the audiological findingsand speech-language results into consideration (and not only the presence or absence ofotitis media with effusion).Chapter 7 Hypernasality in patients with clefts of the lip and palateModule 1: Diagnosis of hypernasalityThe diagnosis of velopharyngeal insufficiency should be made in a multi-disciplinarysetting (at least a surgeon, ENT physician and speech therapist).Following primary palate closure, the diagnosis of velopharyngeal insufficiency shouldonly be made after six months of specialised speech therapy with inadequate treatmentresult, on condition that the soft palate appears to be sufficiently long and mobile(during intra-oral inspection) and the child has been able to follow instructionsadequately.The speech therapy examination (Meijer, 2003) to diagnose velopharyngeal insufficiencyshould be performed by a speech therapist who participates in the national NVSCAguideline task force for speech therapists specialising in clefts of the lip and palate.To make the imaging studies as complete as possible, preferably also perform an oralinspection, acoustic nasometry, mirror tests and nasal endoscopy to confirm thediagnosis of velopharyngeal insufficiency.Perform nasal endoscopy if the chances of success for the child are high (usually fromthree-and-a-half years).Perform video fluoroscopy as an alternative to confirm the diagnosis of velopharyngealinsufficiency (for example if nasal endoscopy is not successful) or to provide additionaldiagnostic information about the velum function.The speech therapist should preferably be present during nasal endoscopy and videofluoroscopy.Make a photo or video recording of the nasal endoscopy.One year after the speech-improving surgery, repeat all the examinations that wereperformed pre-operatively, except possibly the nasal endoscopy/video fluoroscopy, sothat the effect of the speech-improving surgery can be determined as objectively aspossible.One year after the speech-improving surgery, repeat the nasal endoscopy/videofluoroscopy if examinations performed after 6 months of specialised speech therapy stillindicate the presence of velopharyngeal insufficiency in combination with decreasedunderstandability

Do not perform an MRI as a standard part of the diagnostic process for determiningvelopharyngeal insufficiency.Module 2: Treatment of hypernasalityMake a choice for a specific surgical technique based on preoperative speech therapyexamination combined with an examination such as nasal endoscopy and/or videofluoroscopy (see module Diagnosis of hypernasality).Consider an intravelar palatoplasty with repositioning of the palate muscles beforeperforming a pharyngoplasty if the patient has persistent velopharyngeal dysfunctiondespite previously closed palate.In the case of persistent velopharyngeal insufficiency despite renewed repositioning ofthe palate muscles, consider a pharyngoplasty that is based on renewed diagnostic testssuch as nasal endoscopy and/or video fluoroscopy.For a submucous cleft palate, consider a simple palatoplasty over a combined operationconsisting of a palatoplasty with pharyngoplasty.Only use fat injection in the context of a scientific study.Chapter 8 Bone grafting procedure in patients with clefts of the lip and palateModule 1: Timing of the bone grafting procedurePreferably close the cleft in the alveolar arch by means of an early secondary bonegrafting procedure.Base the timing for the bone grafting procedure on the position and the stage of rootformation (½ - 2/3) of the cuspid tooth on the cleft side. The presence of a lateral incisorand the moment of eruption of this incisor can bring the timing forward. For certainindications, the position of the central incisor can be of importance.Decide on the timing of the bone grafting procedure in close consultation with thetreating orthodontist.Consider the tertiary bone grafting procedure only in cases where no (secondary) bonegrafting procedure was performed or when insufficient bone is present in the alveolararch at the site of the cleft for - for example - an implant at a later age. The tertiary bonegrafting procedure can also be performed in adulthood.Module 2: Technique of the bone grafting procedureReconstruct the alveolar cleft (the bone grafting procedure) using bone from the iliaccrest or the chin (possibly supplemented by a bone substitute).If a larger volume is required, use bone from the iliac crest, or opt for bone from the chinsupplemented by a bone substitute.

Based on the literature, it is not possible to make a recommendation for the choice ofthe bone substitute to be used.The use of only a bone substitute for the reconstruction of an alveolar cleft, in otherwords without an autologous bone transplant, should only be performed in a researchcontext and is as yet not suitable for use in general practice.Chapter 9 Orthodontic treatment in patients with clefts of the lip and palateModule 1 : Nasoalveolar mouldingBe reticent about the use of NAM in babies with a complete unilateral or bilateral cleftlip, alveolar arch and palate and only apply NAM in preparation of or in the context of aclinical trial.Module 2: Maxillary protractionIn general, never apply ventral traction to the maxilla using a face mask on a dentallyanchored orthodontic device in children with clefts of the lip and palate with deficientforward growth of the maxilla.Consider ventral traction with a face mask on a dentally anchored orthodontic device inthe case of:1) slight underdevelopment of the maxilla, or;2) if the patient and/or parents do not want surgical treatment, or;3) if the patient has a number of favourable characteristics. The following can serveas a guideline:- patient aged between five and nine years;- mild skeletal defect ANB -10 with SNB angle normal for age;- minimal aesthetic abnormalities of the face;- convergent growth pattern with low bottom half of face;- AP displacement of the mandible in habitual occlusion (negative overjet);- symmetrical condylar growth;- no family history of hyperplasia of the mandible; good cooperation in the orthodontic treatment is expected.If ventral traction with a face mask on a dentally anchored orthodontic device is applied:Inform the patient and parents that the effect can be limited and that it will only becomeclear at the end of the growth period whether an osteotomy of the maxilla will benecessary after all.Module 3: RetentionUse the same form of retention of the position of the teeth as for a patient without cleftsof the lip and palate.In addition, for a patient with a cleft lip, alveolar arch and palate, use a removableorthodontic retention device for the retention of the width of the upper dental arch thatis worn at night for the rest of the patient’s life.

Check the retention device at least once every two years.Module 4: Osteoomy versus distractionosteogenesisConsider a classic Le Fort 1 - either alone or in combination with a setback osteotomy ofthe mandible - in cleft patients with a sagittal discrepancy between the upper and lowerjaw, that is so large that it can no longer be resolved by orthodontic treatment alone.Consider a distraction osteogenesis only in the case of large sagittal discrepancies. Bothtreatments have specific advantages and disadvantages, which can determine the choiceper individual patient and per surgeon.Chapter 10 Rhinoplasty in patients with clefts of the lip and palateDuring primary lip closure, pay attention to the primary correction of the nostril and theposition of the caudal septum and columella of the nose.Preferably delay secondary surgery of the nose for clefts of the lip and palate untilgrowth of the mid-face has been completed and any orthognathic surgery has beencompleted, in order to limit the total number of procedures.Chapter 11 Psychosocial guidance for patients with clefts of the lip and palateScreen the child with clefts of the lip and palate and the parents for psychosocialproblems after birth, at the age of 2 to 3 years, 5 years, 10 to 11 years and 17 years.During this contact, screening should be performed for child factors (wellbeing, possiblelearning problems, fear of medical procedures and acceptance problems) andfamily/parent factors (acceptance problems, grief and parenting style).In addition to conversations, all cleft teams should use the same validated instrument(SDQ, GVL, conversation with parent and/or child).Based on the results of the screening, offer the parents and child further diagnostic testsor treatment if necessary.Appoint a behavioural expert with post-academic training (clinical psychologist or specialeducation generalist) and a social worker for less complex problems in each cleft teamfor the diagnosis of psychosocial problems of the child and family and for the treatmentof complex psychosocial problems.Chapter 13 Dentistry for patients with clefts of the lip and palateFor the cleft teamInclude a (paediatric) dentist with an affinity for paediatric dentistry in the cleft team.An initial consultation with the dentist from the cleft team should take place as soon as

the first deciduous elements have erupted (six to twelve months).Within the cleft team, check the teeth of children with clefts of the lip and palate at theage of 5 years. This should take place in addition to the periodic monitoring by thegeneral dentist.Inform the primary care dentist about the treatment pathway implemented by the cleftteam and discuss who will provide the dental care.For the general dentistContact the cleft team when treating a child with clefts of the lip and palate in yourpractice and offer opportunities for consultation with the cleft team when you deem thisnecessary.Check the teeth of children with clefts of the lip and palate at least every 6 months.

IntroductionReason for creating the guidelineClefts of the lip and palate are one of the most commonly occurring congenitalabnormalities in the Netherlands, with a prevalence of approximately 16.6 per 10,000live births per year (Luijsterburg and Vermeij-Keers, 2011). Clefts of the lip and palatediffers from many other congenital defects due to the fact that the cleft is treatable, thetreatment is generally finite and children with an isolated cleft generally have the samesocial chances and opportunities as children without a cleft.Following the example set by the United Kingdom (UK) and the Scandinavian countries,the care of orofacial clefts in the Netherlands is performed by so-called multi-disciplinarycleft teams. A cleft team generally consists of all the departments of a hospital orinstitute that are required to provide adequate treatment for a child with clefts of the lipand palate.At the moment, the care offered by a cleft team usually stretches from antenatal care orcare at birth to the age of approximately 22 years. The endpoint of the treatment isdetermined by the end of the child’s growth. Teams are expected to offer individualisedcare until the treatment, including possible osteotomies etc., has been completed. Foradults with late problems resulting from clefts of the lip and palate, there is currently nostructured (multidisciplinary) care comparable to the care offered to children with cleftsof the lip and palate available in the Netherlands.The build-up to the development of this clinical practice guideline took place at the startof the development of the clinical practice guideline “Counselling after prenataldetection of clefts of the lip and palate” in 2009. When this guideline was delivered in2011, it was decided that a follow-up guideline for the postnatal care pathway fororofacial clefts was not only logical, but also highly desirable. The prenatal guidelineproject demonstrated that the significant practice variation between cleft teams in theNetherlands in the treatment of patients with clefts of the lip and palate was consideredconfusing and judged as undesirable by patients, parents and healthcare professionals.Patients and parents do understand that some practice variation will be necessary togive each patient the individualized treatment he or she needs. Patients and parentshowever do not understand the often unexplained differences in treatment protocolsbetween the Dutch cleft teams in general. Patients and parents find these differencesfrustrating, because they cannot judge which protocol is preferable or the best for themor their child. They also find it unpleasant that it appears the cleft teams (in theNetherlands) by way of their protocols are in competition with each other. For thepatient, it is very important that the professionals should work together to determinewhat they see as the scientific foundation for their medical actions and define thestandard of care the patient and parents may expect.In summary, both patients and parents would like to see the existing and in their viewconfusing practice variation in treatment between the cleft teams in the Netherlands

clarified (based on evidence) and - if possible - reduced if the evidence gives reason todo so. In addition, there is a desire to improve on the provision of reliable andindependent information about treatment methods.Objective of the guidelineThe objective of this clinical practice guideline is to optimise the care given to patientswith clefts of the lip and palate in the Netherlands, substantiated by scientificknowledge from research where possible. This optimisation also includes obtaininginsight into the cause of the existing practice variation between the various cleft teamsand distinguishing between desirable and undesirable practice variation in thetreatment of patients with clefts of the lip and palate. This will result in proposals for amore uniform treatment insofar as this can be scientifically substantiated.Specific attention will be paid to the following topics:1.reducing undesirable/unfounded practice variation in the working methodand treatment protocol

This clinical practice guideline was primarily written for and is applicable to the treatment of patients with an isolated cleft of the lip and palate without further anomalies. Therefore, this was the starting point for the evidence synthesis on which this guideline is based. Many of these recommendations will also apply to patients with