Uitgangsvraag

Welke overwegingen (voor- en nadelen) spelen een rol bij het bepalen van het moment van het sluiten van de gehemeltespleet en de lipspleet bij kinderen met een (cheilognatho-)palatoschisis?

Aanbeveling

Sluit de lip operatief in de eerste 6 maanden van het leven.

 

Definieer een voorkeursaanpak binnen het schisisteam voor lipsluiting en palatumsluiting om ouders te adviseren in de besluitvorming, maar geef ouders ook de ruimte om een andere aanpak te bespreken.

  • Controleer voorafgaand aan de operatie of de Eurocleft checklist volledig is en vul zo nodig aan met intra-orale foto’s tijdens de ingreep en documentie van bijzonderheden in het OK-verslag.
  • Sluit alleen het palatum molle in het eerste levensjaar en het palatum durum pas later als optimale groei van de maxilla wordt nagestreefd.
  • Sluit het palatum durum en het palatum molle in het eerste levensjaar als optimale spraak wordt nagestreefd.

Inleiding

Door de palatoschisis is het voor patiënten met een schisis onmogelijk om de neusholte van de keelholte te scheiden. Dit geeft met name spraak- en voedingsklachten. Sluiting van het (zachte en/of harde) palatum heeft tot doel deze klachten op te lossen. Eerdere sluiting geeft een eerdere oplossing van de klachten, maar kan groeistoornissen van de bovenkaak geven. Een verstoorde groei van de bovenkaak kan leiden tot een onderontwikkeling van het middengezicht hetgeen een effect heeft op de voor-achterwaartse relatie van de onderkaak en de bovenkaak en op de prominentie van de neus. Ook de breedtegroei van de bovenkaak kan door operatie van het palatum belemmerd worden waardoor de kiezen niet meer goed op elkaar sluiten. Het is dan ook van belang om te weten op welke leeftijd (een deel van) het gehemelte het best gesloten kan worden.

 

Bij lipsluiting is er minder discussie over de timing. Gezien de esthetiek wordt de lip over het algemeen in het eerste jaar gesloten, veelal in de eerste levensmaanden van het leven, waarbij dan een primaire correctie van de neus uitgevoerd kan worden (zie hoofdstuk neuscorrecties bij schisis). Het is van belang om het optimale tijdstip van lipsluiting te onderbouwen met de bestaande literatuur.

Conclusies

Moderate

GRADE

There is a moderate level of evidence that delayed hard palate closure (>3 years) results in a better dental arch relationship than early palatal closure in patients with unilateral cleft lip and palate.

 

Nollet, 2005

 

Moderate

GRADE

There is a moderate level of evidence that there is no difference in speech development or risk of fistula formation in patients with complete unilateral cleft lip and palate undergoing palatal closure using the Furlow technique or von Langenbeck technique (combined with Spina or Millard lip closure) at the age of 9 to 12 months, and those undergoing one of the same procedures at the age of 15 to 18 months.

 

Williams, 2011

 

Low

GRADE

There is a low level of evidence that sequencing of cleft closure (anterior – posterior versus posterior – anterior) has no difference on facial growth in patients with unilateral complete cleft lip and palate.

 

Richard, 2006

 

Very low

Grade

There is a very low level of evidence that in patients with a unilateral complete cleft lip, alveolus and palate minimal incision palatoplasty at six months of age is related to a better speech development when compared to minimal incision palatoplasty at 12 months of age. Maxillary growth does not differ between both groups.

 

Ysunza, 1998

 

Very low

Grade

There is a very low level of evidence that when the outcomes of soft palate repair at 11 months plus hard palate repair at 49 months are compared to soft and hard palate repair at 49 months in patients with a complete cleft of the lip, alveolus and secondary palate:

    • the effects on maxillary growth are similar
    • the effects on hearing loss are similar
    • the speech impediment is greater in the late hard palate closure group
    • risk of temporary and persistent fistulae is greater in the late hard palate closure group.

 

Rohrich, 1996

 

Very low

Grade

There is a very low level of evidence that in children with cleft (lip, alveolus and) palate soft and hard palate repair at six to nine months is related to a decreased risk of fistula formation when compared to soft palate repair at six to nine months and hard palate repair (modified Langenbeck procedure) at four to seven years.

 

Landheer, 2010

 

Very low

Grade

There is a very low level of evidence that there is a lower risk of velopharyngeal insufficiency in patients with only a cleft in the soft palate only, operated who underwent palate closure before six months of age, compared to those operated who underwent palate closure after the age of six months.

 

Grobbelaar, 1994

 

Very low

Grade

There is a very low level of evidence that in children with complete unilateral cleft lip, alveolus and palate there is no difference in speech development between patients undergoing palatal closure using the modified Furlow technique before the age of seven months, and those undergoing the same procedure after seven months.

 

Kirschner, 2000

 

Very low

Grade

There is a very low level of evidence that there is a better speech development in patients with a complete unilateral or bilateral cleft lip, alveolus and palate undergoing one-stage palate repair at ten months (intravelar veloplasty combined with von Langenbeck or two-flap palatoplasty or a hybrid palatoplasty), compared to those undergoing 2-stage palate repair at 11 and 18 months (same procedures).

 

There is no difference in risk of short-term complications between these two groups.

 

Randag, 2014

 

Very low

Grade

There is a very low level of evidence that closing the hard palate in one stage at one year or less is interferesing more with the maxillary growth than closing the hard palate at a later age study in patients with complete unilateral cleft lip, alveolus and palate and alveolus.

 

Gundlach, 2013

 

Very low

Grade

It is not clear what effect one-stage versus two-stage palate repair with delayed hard palate closure has on facial growth on patients with cleft lip and palate.

 

Liao, 2005; Yang, 2010

 

Very low

Grade

There is a very low level of evidence that two-stage palatal closure (primary veloplasty at 20 month and double overlapping palatal hingeflap procedure at five years ten months) leads to a better maxillary development compared to one-stage palatal closure (mucoperiosteal palatal pushback procedure at 20 months) in patients with a unilateral cleft lip and palate.

 

Wada, 1990

 

Very low

Grade

There is a very low level of evidence that two-stage palatal closure (primary veloplasty at 20 month and double overlapping palatal hingeflap procedure at five years ten months ) leads to a comparable maxillary development as one-stage palatal closure (mucoperiosteal palatal pushback procedure at 20 months) in patients with a bilateral cleft lip and palate.

 

Wada, 1990

 

Very low

Grade

There is a very low level of evidence that in unilateral complete cleft lip and palate, children with delayed hard palate repair (velum repair at eight months, hard palate closure at 8,5 years together with bone grafting of alveolar cleft) have a better maxillomandibular development compared to children treated with early hard palate closure (vomer flap at three months together with lip closure with pushback procedure at 22 months) closing of posterior palate.

 

Friede, 2001

 

Very low

Grade

There is a very low level of evidence that in patients with unilateral cleft lip, alveolus and palate the facial growth is similar in patients that underwent one stage palatal closure (lip repair at three months by Millard, one-stage palatal closure within 12 months by Veau) and late palate repair (lip repair within six months by Tennison-Randall, soft palate repair at 12 months by intravelar velaplasty, hard palate repair at 30 months by Veau).

 

Zemann, 2011

 

Very low

Grade

There is a very low level of evidence that one-stage surgical protocol (lip and palate at six to 12 months) is associated with a more favourable palatal morphology but a less favourable dental arch relationship when compared to a three stage surgical protocl(lip, soft palate at 12 to 14 months and hard palate repair at nine to 11 years) in patients with unilateral complete cleft lip and palate.

 

Fudalej, 2011

 

Very low

Grade

There is a very low level of evidence that delaying hard palate repair and using infant orthopaedics does not affect maxillary growth in patients with complete bilateral cleft lip and palate.

 

Bartzela, 2010

Samenvatting literatuur

Description of studies

A total of 18 studies was included in this literature summary: three systematic reviews (Liao, 2006; Nollet, 2005; Yang, 2010), three randomized controlled trials (RCTs) (Richard, 2006; Wada, 1990; Williams, 2011), two prospective observational studies (Fudalej, 2011; Ysunza, 1998) and eight retrospective observational studies (Bartzela, 2010; Friede, 2001; Gundlach, 2013; Kirschner, 2000; Landheer, 2010; Randag, 2014; Rohrich, 1996) and two observational studies where it was not reported whether they were prospective or retrospective (Grobbelaar, 1994; Zemann, 2011).

 

Bartzela (2010) is a retrospective observational study in complete bilateral cleft lip/palate that compares the effect of three different center protocols on maxillary growth (dental arch relationship) in patients with bilateral cleft lip and palate. In center A (n=56) patients underwent soft palate closure (center’s own technique) at the age of 8.5 months and hard palate closure with alveolar bone grafting at the age of 8.5 years. In center B (n=37) patients underwent modified von Langenbeck soft palate closure at 14 months and von Langenbeck hard palate closure at the age of 9.9 years. In center C (n=107) the hard palate was closed on one side at 3.4 months and on the other side at five months, soft palate closure (von Langenbeck) at 19 months and bilateral alveolar bone grafting was performed at the age of 10 years. Dental casts were evaluated at the age of 6, 9 and 12 years.

 

Friede (2001) is a retrospective observational study that examines cephalometric growth in patients with unilateral complete cleft lip and palate that underwent early one-stage hard palate closure (n=29) in one center versus two-staged palate closure (n=30) in another center. In the center that performed early hard palate closure the patients underwent the vomer flap procedure at three months together with lip closure and then a pushback procedure at 22 months. In the center that performed late hard palate closure the patients underwent velum repair at eight months and then hard palate closure at 8.5 years together with bone grafting of alveolar cleft. Lateral röntgencephalograms of these patients at the age of 10 to 16 years were analysed retrospectively.

 

Fudalej (2011) is a prospective observational study that compares dental arch relationship in patients who underwent a one-stage surgical protocol including lip and palate closure in one operation (n=61) versus a three-stage surgical protocol including lip closure, soft palate closure and delayed hard palatal closure in three operations (n=97) in patients with complete unilateral cleft lip and palate. Dental casts of the children were compared at the age of 11.2 for the one-stage closure and 8.7 years for the three-stage closure.

 

Grobbelaar (1994) is an observational study that assessed the effects of timing and time of cleft palate repair on speech in patients with a cleft in only the soft palate. A total of 184 patients were included in this study who underwent a Dorrance repair (n=25), Wardill (n=41), Perko (n=19), von Langenbeck (n=79) or Furlow Z-plasty (n=20). It was the policy of the unit to repair cleft palates before the age of one year. Palates were repaired after one year of age when patients were referred at a later age or failed to attend the hospital on the booking date and thus had to be rescheduled. A total of 78 patients were operated before six months of age and 107 after six months. Articulation, intelligibility and resonance were assessed. The follow-up period was from 3 to 24 years with a mean of 9.6 years.

 

Gundlach (2013) is a retrospective observational study in patients with complete unilateral cleft lip, palate and alveolus, in which the effects of one-stage versus two-stage palatoplasty on speech development and maxillary growth are described. Plaster casts from 85 patients were evaluated (n=36 at 8 years of age, n=49 at 18 years of age). The treatment results of three centers were compared. In two centers (A and B, n=50) the palate was closed in two stages and in one center (C, n=35) in one stage. In center A and B labioplasty was performed at six months according to the waveline procedure and intravelar veloplasty by Kriens at age 15 to18 months in center B and three years at center A. In center C labioplasty was performed at three months according to the Triangular Flap Method and veloplasty at 12 to 18 months by the Two Flap technique, a modification of Tennison’s Stensil Method. Maxillary arch dimensions measured on plaster casts, taken at the age of 8 and 16 years, were studied. The speech development was not precisely described in this paper, but in the discussion an earlier publication of speech development in this population (1987) is mentioned.

 

Kirschner (2000) describes a retrospective observational study that examines speech outcome in children with complete unilateral cleft lip and palate, who underwent palate repair between three to seven months of age (n=40) or later than seven months of age (n=50). Surgical procedures were performed by two surgeons using a modified Furlow technique. Speech evaluations were conducted using the Pittsburg Weighted Values for Speech symptoms associated with velopharyngeal incompetence by two speech pathologists. Patients were followed for a mean of 7.9 ± 3.2 years.

 

Landheer (2010) is a retrospective observational study that studies the clinical outcomes of cleft (lip and) palate patients in terms of fistula rate and aims to determine whether patient or surgery characteristics determine this outcome. The medical records of 275 cleft palate patients were examined, and a multivariate analysis was performed to look for predictors of formation of fistulas. One of the examined determinants was one- stage (n=121) versus two-stage (n=154) palate repair. All patients underwent the cleft palate repair in two stages, unless the operating surgeon considered the cleft narrow enough to close in one operation. The soft palate was closed at six to nine months. The hard palate was closed by a modified von Langenbeck procedure at four to seven years; when the speech therapist felt that the open hard palate may influence speech. The majority of operations was performed by two plastic surgeons. The mean duration of follow-up was nine years.

 

Liao (2006) is a systematic review in which the effect of timing of hard palate repair on facial growth in patients with cleft palate and lip is studied, with special reference to cranial base, maxilla mandible, jaw relation and incisor relation. A total of 15 studies (n=1439), all retrospective and non-randomized, were included in this review.

 

Nollet (2005) is a systematic review and meta-analysis, that assesses determinants for treatment outcome in patients with unilateral cleft lip and palate. A total of 15 studies (n=1236) were included in this review. Patients were evaluated by the Great Ormond Street London and Oslo Yardstick (GOSLON) score, which assesses the dental arch relationship in terms of anterior-posterior, transverse and vertical discrepancies in persons with unilateral cleft lip and palate. For this score a very good dental arch relationship is scored as one and a very poor one as five.

 

Randag (2014) is a retrospective observational study that describes the effect of one-stage versus two-stage palate closure on speech and risk of postoperative complications in patients with a complete unilateral or bilateral cleft lip and palate. A total of 24 patients underwent one-stage palate closure at ten months, and 24 patients underwent two-stage closure at 11 and 18 months. Both patient groups underwent lip closure at 3-6 months. Soft palate was closed using intravelar veloplasty. Hard palate was closed using the von Langenbeck technique: a two-flap palatoplasty or a hybrid palatoplasty depending on the width of the cleft. Speech analysis was performed by analysing spontaneous speech and performing an articulation test. Patients were followed until the age of 2.5 years.

 

Richard (2006) describes an RCT in which the effects of operating the soft palate first (n=23, at follow-up 16) versus operating hard palate first (n=24, at follow-up n=19) on facial growth are studied in unilateral complete cleft lip and palate patients. The anterior operation consisted of a lip repair by Millard rotation advancement, a nasal correction using the McComb procedure and a hard palate repair by a single layer vomerine flap. The posterior operation consisted of a soft palate repair with medial von Langenbeck incisions. The two operations were undertaken three months apart with the first operation at 19 months of age. Preoperative maxillary models, speech and velopharyngeal function and ontological examinations were performed. Patients were followed until the age of four to six years.

 

Rohrich (1996) is a retrospective observational study that describes the effect of early (at an average age of 11 months, n=21) versus late (at an average age of 49 months, n=23) hard palate closure on speech and maxillary growth in patients with a complete cleft of the alveolus and secondary palate. Out of the total of 44 patients, 13 had bilateral cleft types. In all patients the alveolar cleft repair (Millard rotation-advancement) was undertaken at an average age of 3.4 months. The timing of soft palate repair was 11 months in both groups. Patients in the early closure group underwent a modified three- or four-flap Wardill-Kilner procedure. In the late closure group, the soft palate closure was performed with short Veau flaps, followed by a second-stage hard palate closure with a vomer flap. All operative procedures in both groups were performed by the same plastic surgeon. Speech analysis of specific parameters was assessed by the speech pathologist. Maxillofacial growth evaluation based on soft-tissue profile measurements, cephalometric analysis and dental impression of upper and lower arches was assessed by the orthodontist. Palatal assessment based on a detailed palatal examination and history of fistulas was assessed by the plastic surgeon. Hearing status was assessed by an otologic history, examination and audiogram by the otologist. Patients were followed until an average of 17.5 years (12.8 years of follow-up).

 

Wada (1990) is a randomized trial that describes the effects of one-stage palatal closure (unilateral cleft n=14, bilateral n=8) versus two-stage palatal closure (unilateral n=16, bilateral n=7) in patients with uni- and bilateral cleft lip and palate. Also, the maxillary growth was compared with 11 healthy controls. Lip repair was performed at five months of age. One-stage repair was performed at 20 months using mucoperiosteal palatal pushback procedure. Two-stage repair was performed with primary veloplasty at 20 month and double overlapping palatal hingeflap procedure at five years ten months. Maxillofacial cast models were examined. The children are followed until the age of ten years.

 

Williams (2011) describes a RCT in which different surgical techniques and different timings of surgery for cleft palate were compared in terms of speech outcome and risk of palatal fistulae in patients with a complete unilateral cleft lip and palate. A 2x2x2 factorial clinical trial was used in which each subject was randomly assigned to one of eight groups: one of two different lip repairs (Spina versus Millard), one of two different palatal repairs (von Langenbeck versus Furlow) and one of two different ages at time of palatal surgery (9 to 12 months versus 15 to 18 months). All surgeries were performed by the same 4 surgeons. A total of 181 patients were operated at 9 to 12 months (Spina - Furlow = 35, Millard – Furlow = 43, Spina – Langenbeck = 51, Millard – Langenbeck = 52) and 195 at 15-18 months (Spina - Furlow = 48, Millard – Furlow = 47, Spina – Langenbeck = 46, Millard – Langenbeck = 54). Children were followed for at least the age of four years.

 

Yang (2010) describes a systematic review that evaluates the effect of one-stage versus two-stage palate repair with delayed hard palate closure in patients with cleft lip and palate. A total of nine studies (n=812), all retrospective and non-randomized, are included.

 

Ysunza (1998) describes a prospective observational study in which closure of the primary and secondary palate at six months of age (n=35) was compared to the same procedure at 12 months of age (n=41) in terms of speech outcome in patients with unilateral complete cleft of primary and secondary palate. Minimal incision palatopharyngoplasty was used in all cases. Operations were performed by two surgeons. All patients were followed until they were four years of age and underwent a speech evaluation and maxillofacial assessment.

Zemann (2011) is an observational study that describes the effect of one-stage (n=22) in one center versus two-stage (n=32) palatal repair on facial growth in another center in patients with unilateral cleft lip, alveolus and palate. In the center that performed one stage palate closure the patients underwent lip repair at three months by Millard and one-stage palatal closure within 12 months by Veau. In the center that performed two-stage palate closure the patients lip repair was performed within six months by Tennison-Randall, soft palate repair at 12 months by intravelar velaplasty and hard palate repair at 30 months by Veau. Lateral röntgencephalograms of these patients at the age of 6-10 years were analysed retrospectively.

 

Results

Maxillary growth

Bartzela (2010) describes that maxillary growth was similar in all three centers at the age of 9 and 12 years. Apparently delaying hard palate closure or employing maxillary orthopaedics did not affect maxillary growth with long follow-up.

 

Friede (2001) reports that children that had undergone early hard palate closure generally had more retrognathic faces (more maxillary retrusion and thus less facial convexity) than those who underwent late hard palate closure. Furthermore, the ANB angle only became negative in the patients who underwent early hard-palate closure and only in the older (14 to 16 years) age groups. The anterioir vertical development of the maxilla was most satisfactory in patients who underwent delayed closure of the hard palate. Overall, the midfacial development was more favourable in the delayed hard palate closure group (exact data not reported, p-values not reported).

 

Fudalej (2011) describes that the dental arch relationship was better in the group that underwent the two-stage palatal closure. However, the palatal morphology was more favourable in the one-stage palatal closure group, when compared to the two-stage closure.

 

Liao (2006) describes that the studies included in this systematic review show conflicting results. A total of 13 studies describe the effect of timing of hard palate repair on maxillary growth. Three studies concluded that the variation in timing of the hard palate repair does not affect the length of the maxilla significantly, whereas one study opposes this view. One study reports that the effect of timing depends on the type of cleft and another that the results depend on the age at time of assessment. Seven studies conclude that variation in timing of hard palate repair does not affect protrusion of the maxilla, whereas another two oppose this view and one more study states that this depends on the age at assessment. Methodological deficiencies and heterogeneity of the studies prevented pooling of results and possibility to draw major conclusions.

 

Gundlach (2013) describes that in center C (one-stage palatoplasty) the patients had more constricted palates than in the centers with two-stage palatoplasty. In center A (two-stage palatoplasty at three years) the prevalence of anterior cross-bite was lowest.

 

Nollet (2005) describes in a meta-analysis that patients whose hard and soft palate was closed before the age of three (early) presented significantly poorer GOSLON scores (mean 2.9±0.4) than patients whose palate was closed at a later age (mean 2.3±0.2, p=0.002). The percentage of patients with a GOSLON score of four and five (poor or very poor relationship) was 29% in the early palate closure group and 26% in the late palate closure group (p=0.002). However, Nollet 2005 also notes that well-designed randomized clinical trials are required for further investigation of the optimal timing for palatal closure, since the methodological quality of the included studies is low and heterogeneity between studies is high.

 

Richard (2006) describes that there was no significant difference in overall facial growth between the different types of palatal closure sequencing.

 

Rohrich (1996) describes that there was no significant difference in maxillary arch width between the late and early closure groups. Both groups demonstrated anterior maxillary collapse and a relatively normal arch relationship at the molar level without maxillary collapse; with no significant difference between the groups. Both groups also demonstrated a degree of maxillary and midfacial hypoplasia, again with no significant difference between the groups.

 

Wada (1990) reports that in unilateral cleft palate patients maxillary growth after two-stage palatal closure was comparable to those of non-cleft controls regarding depth and height of the maxilla, while after one-stage closure aberrant maxillary development was observed. For patients with bilateral clefts, maxillary growth was similar in the one-stage and two-stage palatal closure groups.

 

Yang (2010) reports that the results of the included studies are conflicting. Two studies evaluate the effect of stage of palate repair on the length of the maxilla. One study concludes that the stage of palate repair does not affect the length of the maxilla, whereas the other study opposes this view. Eight studies evaluate the effect of stage of palate repair on the protrusion of the maxilla. Seven studies conclude that the stage of palate repair does not affect the protrusion of the maxilla, whereas one study opposes this view. The difference in the palate repair timing between the stages varies considerably between studies (1-96 months). Pooling of results is not possible due to the heterogeneity of included studies. Furthermore, definitive conclusions about maxillary growth cannot be drawn based on the results of current literature.

 

Ysunza (1998) reports that there was no significant difference in orthodontic parameters (intercanine and intermolar width for both arches, maxillary length) between patients operated at SIX months and at 12 months. Both groups showed anterior maxillary collapse and a relatively normal interarch relationship at the molar level.

 

Zemann (2011) reports that there was no statistically significant difference in cephalometric parameters between patients undergoing one-stage versus two-stage palate closure. However, in the one-stage closure center there was considerably normal sagittal facial growth, with tendency to forward growth of the mandible. In the two-stage closure group there was a slight decrease in sagittal maxillary growth and mandibular growth with unchanged jaw relationship.

Speech

Grobbelaar (1994) describes that there was significantly (p<0.05) more velopharyngeal incompetence in the patients undergoing palatalum closure after six months (9/107), compared to those before 6 months of age (5/73).

Gundlach (2013) mentions in the discussion that an earlier publication noted no significant difference in speech success rates between center C where the hard palate was closed in one stage at 12 to 18 months (74%) and center A where the palate was closed in two stages at three years (70%).

 

Kirschner (2000) describes that there were no differences observed between patients who underwent palate closure before and after seven months of age in terms of resonance, articulation and velopharyngeal function (exact data not reported, shown in figures).

 

Randag (2014) reports that the one-stage palate closure group had a significantly higher number of correctly produced initial consonants (9.7±4.5 versus 7.0±3.8, p=0.03). There was no difference between the groups in frequency of inadequate language production, resonance problems, nasal air emission and intelligibility.

 

Richard (2006) reports that there was hypernasal resonance significant enough to warrant surgery in five patients in the posterioir-anterior group and four in the anterior-posterior group. This difference was not statistically significant.

 

Rohrich (1996) describes that 10% of the patients in the late closure group had articulation errors versus 5% in the early closure group (p-value not reported). Furthermore, nasal resonance was normal in 30% of the late closure group versus 81% in the early closure group (p<0.001). There was more hypernasality in the late closure group (p<0.01, data not reported). Speech intelligibility was impaired in 35% of the patients in the late closure group versus 5% in the early closure group (p<0.02). The number of subjects with a normal articulation pattern was similar in both groups (data not reported).

 

Williams (2011) reports that of the patients operated early (9 to 12 months) 78% had hypernasality and 57% had nasal air emission versus 74% and 55% operated late (15 to 18 months) respectively. The odds ratio for hypernasality was 1.46 (95% CI: -0.84 to 2.54, p=0.12) and for nasal air emission 1.16 (95% CI: 0.72 to 1.85, p=0.49) for patients operated early versus patients operated late.

 

Ysunza (1998) reports that articulation scales (as measured by the BELE articulation scale) were significantly better in patients operated at six months (mean 5.4 ± 1.0) compared to patients operated at 12 months (mean 9.0 ± 1.1).

 

Hearing Status

Richard (2006) reports that there was no significant difference in hearing status between the patients in the posterior-anterior group and the anterior-posterior group.

 

Rohrich (1996) describes that there was no statistically significant difference in the prevalence of severe hearing loss between the late and early closure group as evaluated audiometrically (data not reported).

 

Esthetic results

No studies were identified that met the inclusion criteria and reported esthetic results as an outcome measure.

 

Early complications

Landheer (2010) describes that out of the 154 patients with 2-stage repair 42 (25 hard palate, six soft palate, 11 combined) developed fistulas and out of 121 patients with 1-stage repair 58 (31 hard palate, 11 soft palate, 17 combined) developed fistulas. Most fistulas occurred in patients with Veau IV cleft types: 2-stage closure 12/48 (25%) and 1-stage closure 1/8 (13%). Odds ratio of fistula rate was 2.3 (95% CI: 1.2 – 4.3) for 2-stage repair versus 1-stage repair.

 

Randag (2014) describes that there was no significant difference between the one-stage (4/24) and two-stage (1/24) group in postoperative haemorrhage. No differences were found in other short-term postoperative complications (data not reported).

 

Richard (2006) reports that there were 10 symptomatic fistulae in the anterior-posterior group and six in the posterior-anterior group (p>0.05).

 

Rohrich (1996) describes that the overall fistula rate was six (29%) in the early closure group versus 14 (61%) in the late closure group (p<0.05).

 

Williams (2011) describes that 44/181 patients operated early (9 to 12 months) developed a fistula, versus 37/195 in the late (15 to 18 months) operation group. The odds ratio for fistula formation in the early versus late group was 1.37 (95% CI: 0.84 to 2.22, p=0.21).

 

Late complications

Landheer (2010) describes that the majority of recurrent fistulas occurred in the one-stage repair group. However, this difference was not statistically significant (data not reported).

 

Rohrich (1996) describes that the persistent fistula rate at time of examination was 1(5%) in the early closure group versus 8 (35%) in the late closure group (p<0.05). No pharyngeal flaps were performed in any of the studied patients.

 

Grading the evidence

Due to the large variation in study design, type of intervention, measurement of outcome and length of follow-up it was not possible to pool the results.

 

Most studies were graded as very low level of evidence due to study design type and imprecision.

Zoeken en selecteren

Om de uitgangsvraag te kunnen beantwoorden is er een systematische literatuuranalyse verricht op basis van de volgende PICO:

P: patiënt met minimaal een palatoschisis;

I: palatumsluiting voor de leeftijd van 18 maanden / lipsluiting voor de leeftijd drie maanden;

C: palatumsluiting na de leeftijd van 18 maanden/ lipsluiting na de leeftijd van drie maanden;

O: kaakgroei/vorm van de kaak, spraak, gehoor, esthetisch resultaat, vroege complicaties (fistula), late complicaties.

 

Relevante uitkomstmaten

De werkgroep achtte de vorm van de lip, groei van de maxilla en velopharyngeale insufficiëntie voor de besluitvorming kritieke uitkomstmaten.

 

Search and selection (Method)

A systematic search was performed in the databases of Medline (through OVID), Embase and the Cochrane Library between 1980 and December 3rd 2014. This search was aimed to identify systematic reviews, RCTs, CCTs, and observational studies.

Studies that investigated patients with cleft lip and palate were selected if they compared stage palatal closure before 18 months of age / lip closure before three months of age to stage palatal closure palate after 18 months / lip closure after three months of age. Data on effect sizes or primary data had to be available for at least one of the outcomes of interest: early complications (fistula), late complications (maxillary growth), speech, aesthetic results. For the outcomes speech and hearing, a follow-up until at least the age of four years, and a follow-up length of at least one year was deemed sufficient. For the outcome maxillary growth, a follow-up time until at least the age of nine years was deemed sufficient. When papers reported a shorter follow-up time they were excluded.

 

The initial search identified 516 references of which 40 were assessed on full text. After assessment of full text 22 studies were excluded and 18 studies were included.

Referenties

  1. Bartzela T, Katsaros C, Shaw WC, Ronning E, Rizell S, Bronkhorst E, et al. A longitudinal three-center study of dental arch relationship in patients with bilateral cleft lip and palate. Cleft Palate-Craniofac J 2010;47(2):167-74.
  2. Friede H, Enemark H. Long-term evidence for favorable midfacial growth after delayed hard palate repair in UCLP patients. Cleft Palate-Craniofac J 2001 Jul;38(4):323-9.
  3. Fudalej P, Katsaros C, Bongaarts C, Dudkiewicz Z, Kuijpers-Jagtman AM. Dental arch relationship in children with complete unilateral cleft lip and palate following one-stage and three-stage surgical protocols. Clinical Oral Investigations 2011 Aug;15(4):503-10.
  4. Grobbelaar AO, Hudson DA, Fernandes DB, Lentin R. Speech results after repair of the cleft soft palate. Plast Reconstr Surg 1995;95(7):1150-4.
  5. Gundlach KK, Bardach J, Filippow D, Stahl-de CF, Lenz JH. Two-stage palatoplasty, is it still a valuable treatment protocol for patients with a cleft of lip, alveolus, and palate? Journal of Cranio-Maxillo-Facial Surgery 2013 Jan;41(1):62-70.
  6. Kirschner RE, Randall P, Wang P, Jawad AF, Duran M, Huang K, et al. Cleft palate repair at 3 to 7 months of age. Plast Reconstr Surg 2000;105(6):2127-32.
  7. Landheer JA, Breugem CC, van der Molen AB. Fistula incidence and predictors of fistula occurrence after cleft palate repair: two-stage closure versus one-stage closure. Cleft Palate-Craniofac J 2010 Nov;47(6):623-30.
  8. Liao YF, Mars M. Hard palate repair timing and facial growth in cleft lip and palate: a systematic review. [Review] [27 refs]. Cleft Palate-Craniofac J 2006 Sep;43(5):563-70.
  9. Nollet PJ, Katsaros C, Van't Hof MA, Kuijpers-Jagtman AM. Treatment outcome in unilateral cleft lip and palate evaluated with the GOSLON yardstick: a meta-analysis of 1236 patients. Plastic & Reconstructive Surgery 2005 Oct;116(5):1255-62.
  10. Randag AC, Dreise MM, Ruettermann M. Surgical impact and speech outcome at 2.5 years after one- or two-stage cleft palate closure. Int J Pediatr Otorhinolaryngol 2014.
  11. Richard B, Russell J, McMahon S, Pigott R. Results of randomized controlled trial of soft palate first versus hard palate first repair in unilateral complete cleft lip and palate. Cleft Palate-Craniofac J 2006 May;43(3):329-38.
  12. Rohrich RJ, Rowsell AR, Johns DF, Drury MA, Grieg G, Watson DJ, et al. Timing of hard palatal closure: a critical long-term analysis. Plastic & Reconstructive Surgery 1996 Aug;98(2):236-46.
  13. Wada T, Tachimura T, Satoh K, Hara H, Hatano M, Sayan NB, et al. Maxillary growth after two-stage palatal closure in complete (unilateral and bilateral) clefts of the lip and palate from infancy until 10 years of age. Journal of the Osaka University Dental School 1990 Dec;30:53-63.
  14. Williams WN, Seagle MB, Pegoraro-Krook MI, Souza TV, Garla L, Silva ML, et al. Prospective clinical trial comparing outcome measures between Furlow and von Langenbeck Palatoplasties for UCLP. Ann Plast Surg 2011 Feb;66(2):154-63.
  15. Yang IY, Liao YF. The effect of 1-stage versus 2-stage palate repair on facial growth in patients with cleft lip and palate: a review. [Review]. International Journal of Oral & Maxillofacial Surgery 2010 Oct;39(10):945-50.
  16. Ysunza A, Pamplona MC, Mendoza M, Garcia-Velasco M, Aguilar MP, Guerrero ME. Speech outcome and maxillary growth in patients with unilateral complete cleft lip/palate operated on at 6 versus 12 months of age. Plastic & Reconstructive Surgery 1998 Sep;102(3):675-9.
  17. Zemann W, Karcher H, Drevensek M, Kozelj V. Sagittal maxillary growth in children with unilateral cleft of the lip, alveolus and palate at the age of 10 years: an intercentre comparison. Journal of Cranio-Maxillo-Facial Surgery 2011 Oct;39(7):469-74.

Evidence tabellen

Table of excluded studies (exclusion after assessment of full text).

Author and year of publication

Reason for exclusion

Berkowitz, 2005

Does not meet selection criteria

Da Silva Filho, 2000

Does not meet selection criteria (follow-up too short)

Deluke 1997

Does not meet selection criteria (case serie)

Farronato 2014

Does not meet selection criteria

Friede, 2001

Included in systematic review Liao, 2006

Fudalej, 2010

Does not meet selection criteria

Gerke 2014

Does not meet selection criteria (congress abstract)

Goodacre, 2003

Does not meet inclusion criteria (lip closure)

Hudson 1994

Does not meet selection criteria (review, not systematic)

Klintö, 2014

Does not meet selection criteria (follow-up too short)

Latham 2007

Does not meet selection criteria

Lohmander, 2011

Does not meet selection criteria

O’Gara, 1994

Does not meet selection criteria (follow-up too short)

Prasad, 2000

Does not meet selection criteria

Rohrich, 1996

Included in systematic review Yang, 2010 and Liao, 2005.

Rohrich 2000

Does not meet selection criteria (review, not systematic)

Silva, 2001

Included in systematic review Yang, 2010

Tanino, 1997

Does not meet selection criteria

Wada, 1990

Does not meet selection criteria

Willadsen, 2012

Does not meet selection criteria

Williams, 2011

Does not meet selection criteria

Ysunza, 2010

Does not meet selection criteria

 

Evidence table for systematic review of RCTs and observational studies (intervention studies)

Study reference

Study characteristics

Patient characteristics

Intervention (I)

Comparison / control (C)

 

Follow-up

Outcome measures and effect size

Comments

Liao 2006

 

[individual study characteristics deduced from [1st author,

year of publication

]]

 

PS., study characteristics and results are extracted from the SR (unless stated otherwise)

SR and meta-analysis of RCTs / cohort / case-control studies

 

Literature search up to December 2004

 

A: Robertson and Jolleys 1974

B: Hotz 1978

C: Schweckendiek 1978

D: Hotz and Gnoinsky 1979

E: Blijdorp and Egyedi 1984

F: Bardach 1984

G: Ross 1987

H: Friede 1987

I: Noverraz 1993

J: Smahel 1994

K: Rohrich 1996

L: Friede 1999

M: Nandlal 2000

N: Friede and Enemark 2001

O: Swennen 2002

 

Study design: RCT [parallel / cross-over], cohort [prospective / retrospective], case-control

A: cross-sectional

B: cross-sectional

C: cross-sectional

D: longitudinal

E: cross-sectional

F: cross-sectional

G: longitudinal

H: cross-sectional

I:mixed longitudinal

J: cross-sectional

K: cross-sectional

L: mixed longitudinal

M: cross-sectional

N: longitudinal

O: cross-sectional

 

 

Setting and Country: United Kingdom

 

Source of funding:

Not reported

 

Inclusion criteria SR:

1) studies evaluating the effect of timing of hard palate repair on facial growth in patients with cleft lip and palate

2) studies in English

 

Exclusion criteria SR:

1) case reports

2) case series (<10 patients)

3) studies with no comparison or control in sample

 

15 studies included

 

 

Important patient characteristics at baseline:

 

Number of patients

A: 40

B: 53

C: 266

D: 46

E: 105

F: 78

G: 450

H: 49

I: 88

J: 24

K: 44

L: 37

M: 50

N: 60

O: 62

 

Sex:

A: NR

B: NR

C: NR

D: NR

E: NR

F: 59% Male

G: 100% Male

H: 42% Male

I: NR

J: 100% Male

K: 59% Male

L: 42% Male

M: NR

N: 80% Male
O: 65% Male

 

Groups comparable at baseline? Unclear

Describe intervention:

 

A: L+A+SP (3)

B: L (6) à SP (18)

C: SO (6-8) à L (7-9) à HP (144 – 168)

D: L(6) à SP (18)

E: L + HP(3) à SP (66-78) (Veau)

F: SO (8) à L (8.6) à HP (158.4)

G: 1) SO à HP (58-84)

2) HP, SP (21-33)

3) HP, SP (12-20)

4) HP, SP (<11)

H: 1) L (2.5) --: SP (8.8) à L/N (18.1)

2) L+HP (2.1) à SP (7.6) à L/N (19.1)

3) L(2.9)à SP (10.4) à L/N (19.8)

I:1) L9^) à SP (13) à HP + A (113)

2) L(6) à SP (13) à HP (55)

3) L(6) à SP (13) à HP (18)

(all modified vL)

J: à HP (72) (Push-back)

K: L (3.4) à SP (11.4) à HP (48.6) (VMF)

L: L(2.1) à SP (8.1) à L/N (17.2) à HP + A (102.5)

M: 1) L à HP + SP (36-78)

2) L à HP + SP (24-36)

N: L (2) à SP (8) à L/N (18) à HP + A (104)

O: L (5.8) à HP (29.1) à SP (32.3)

 

Number is age in months

L=lip

L/N: lip/nose

A= alveolus

(H) (S) P = (hard) (soft) palate

vL = von Langenbeck

(P) (V) MF = (palatal) (vomerine) mucoperiosteal flap

Describe control:

 

A: L + SP (3) à HP 12-15 (PMF)

B: L (3) à HP + SP (30-36) (PMF)

C: -

D: -

E: L+HP (3) à SP (28-42) (Veau)

F: -

G: SP à HP

H: L+HP (1.5) à HP (4.1) à SP (9.5) à L/N (20.5)--

I: L(6) à SP (13)

J: à HP (48) (Push-back)

K: L (3.4) à HP + SP(10.8) (Push-back)

L: L/N (7.8) à SP (20.2) à HP (61.9) à A (114.8)

M: L à HP + SP (8-24)

N: L+HP (3) à SP (22) à A (119)

O: SP (3) à L + HP (6.2)

 

 

 

Number is age in months

L=lip

L/N: lip/nose

A= alveolus

(H) (S) P = (hard) (soft) palate

vL = von Langenbeck

(P) (V) MF = (palatal) (vomerine) mucoperiosteal flap

End-point of follow-up:

Until the age of… (in years)

 

A: 3 (lateral cephalograms); 4.5 (cast)

B: 4-6

C: adult

D: 5

E: adult

F: 17.2 (range 12-24)

G: 11 – 19.6

H: 7

I: 17.1

J: adult

K: 18

L: 16

M: 10 (range 6-14)

N: 15.9

O: 10 (range 8.8 – 11.2)

 

 

For how many participants were no complete outcome data available?

(intervention/control)

Not reported in all studies (however, since the studies were cross-sectional or retrospective in nature, this is not applicable)

 

Maxillary growth:

 

A total of 13 studies describes the effect of timing of hard palate repair on maxillary growth. Three studies concluded that the variation in timing of the hard palate repair do not affect the length of the maxilla significantly, whereas one study opposes this view. One study reports that the length of the effect of timing depends on the type of cleft and another that the results depend on the age at time of assessment. Seven studies conclude that variation in timing of hard palate repair does not affect protrusion of the maxilla, whereas another two oppose this view and one more study states that this depends on the age at assessment. Methodological deficiencies and heterogeneity of the studies prevented pooling of results and possibility to draw major conclusions.

 

 

 

 

 

 

Facultative:

 

Methodological deficiencies and heterogeneity of the studies prevented pooling of results and possibility to draw major conclusions.

 

Level of evidence: GRADE very Low

 

 

Nollet 2005

 

[individual study characteristics deduced from [1st author,

year of publication

]]

 

PS., study characteristics and results are extracted from the SR (unless stated otherwise)

SR and meta-analysis of [RCTs / cohort / case-control studies]

 

Literature search up to December 2003

 

A: Mars 1987

B: Mars 1992

C: Noverraz 1993

D: Hathorn 1996

E: Atack 1998

F: Leonard 1998

G: Johnson 2000

H: Morris 2000

I:Williams 2001

J: Williams 2001

K: DiBiase 2002

L: Pigott 2002

M: Chan 2003

N: Choudhary 2003

O: Bongaarts 2004

 

Study design: RCT [parallel / cross-over], cohort [prospective / retrospective], case-control

 

Setting and Country: the Netherlands

 

Source of funding:

Not reported

 

Inclusion criteria SR:

 

Exclusion criteria SR:

 

XX studies included

 

 

Important patient characteristics at baseline:

Number of patients; characteristics important to the research question and/or for statistical adjustment (confounding in cohort studies); for example, age, sex, bmi, ...

 

N, mean age

A: 115

B: 149

C: 68

D: 32

E: 46

F: 70

G: 54

H: 35

I: 452

J: 18

K: 44

L: 38

M: 40

N: 25

O: 41

 

 

 

Sex:NR

 

 Groups comparable at baseline? Unclear

 

Describe intervention:

 

Early palatal closure (soft and hard) <3 years

(E)

 

Not reported per study

Describe control:

 

Late palatal closure (soft and hard) >3 years

(L)

 

Not reported per study

End-point of follow-up:

 

NR

 

 

For how many participants were no complete outcome data available?

(intervention/control)

NR

 

 

 

GOSLON score

 

(1 very good, 5 very poor)

 

GOSLON score:

E: 2.9±0.4

L: 2.3 ± 0.2

P=0.002

 

% GOSLON score 4 and 5:

E: 29 ± 14

L: 4 ± 4

P=0.002

 

 

 

 

 

Facultative:

 

Brief description of author’s conclusion:

well-designed randomized clinical trials are required for further investigation of the optimal timing for palatal closure, since the methodological quality of the included studies is low and heterogeneity between studies is high.

 

 

Level of evidence: GRADE low

 

 

Yang 2010

 

[individual study characteristics deduced from [1st author,

year of publication

]]

 

PS., study characteristics and results are extracted from the SR (unless stated otherwise)

SR and meta-analysis of [RCTs / cohort / case-control studies]

 

Literature search up to November 2007

 

A: Ross 1987

B: Molsted 1992

C: Smahel 1993

D: Rohrich 1996

E: Silva Filho 2001

F: Silvera 2003

G: Corbo 2005

H: Stein 2007

I:Holland 2007

 

Study design: RCT A: cross-sectional

B: cross-sectional

C: cross-sectional

D: cross-sectional

E: cross-sectional

F: longitudinal

G: longitudinal

H: longitudinal

I: cross-sectional

 

 

Setting and Country: Taiwan

 

Source of funding:

No funding

 

Inclusion criteria SR:

 

Exclusion criteria SR:

 

XX studies included

 

 

Important patient characteristics at baseline:

Number of patients; characteristics important to the research question and/or for statistical adjustment (confounding in cohort studies); for example, age, sex, bmi, ...

 

Number of patients:

A: 380

B: 95

C: 51

D: 44

E:75

F: 21

G: 21

H: 43

I: 82

….

 

Sex:

A:100 % Male

B: 58% Male

C: 86% Male

D: 59% Male

E: 60% Male

F: 62% Male

G: 57% Male

H: 67% Male

I: 59% Male

….

 

Groups comparable at baseline? Unclear

Describe intervention:

 

A: L (?) à SP + HP (<20)

B: L (3) à SO (9) à HP (108) (V) (P)MF

C: 1) L(6) à SP + HP (12)

2) L (6) à SP + HP (<24)

D: L (3) à SP + HP (11)

E: L (9) à SP + HP (19)

F: L (3-11) à SP + HP (18)

G: SP (3) à L + HP (6) à A (120)

H: L (4) à SP (23) à HP (86) à A (127)

I: L (?) à SP (12) à HP (84)

 

Number is age in months

L=lip

L/N: lip/nose

A= alveolus

(H) (S) P = (hard) (soft) palate

vL = von Langenbeck

(P) (V) MF = (palatal) (vomerine) mucoperiosteal flap

? = unclear

Describe control:

 

A: L (>) à SP (?) à HP (<11-108)

B: -

C: L (5) à SP + HP (12)

D: L (3) à SP (11) à HP (49)

E: L + SP (5.5) à HP (20)

F: L (5&8) à SP (18) à HP (72)

G: L (4) à SP + HP (23) à A (150)

H: L (?) à SP + HP (12)

I: -

 

Number is age in months

L=lip

L/N: lip/nose

A= alveolus

(H) (S) P = (hard) (soft) palate

vL = von Langenbeck

(P) (V) MF = (palatal) (vomerine) mucoperiosteal flap

? = unclear

End-point of follow-up:

(in years)

 

A: 15

B: 8-10

C: 10

D: 17-18

E: 7

F: 12

G: 12

H: 18

I: adults

….

 

 

For how many participants were no complete outcome data available?

(intervention/control)

Not reported in all studies (however, since the studies were cross-sectional or retrospective in nature, this is not applicable)

 

 

 

Maxillary growth

 

Two studies evaluate the effect of stage of palate repair on the length of the maxilla. One study concludes that the stage of palate repair does not affect the length of the maxilla, whereas the other study opposes this view.

 

Eight studies evaluate the effect of stage of palate repair on the protrusion of the maxilla. Seven studies conclude that the stage of palate repair does not affect the protrusion of the maxilla, whereas one study opposes this view.

 

The difference in the palate repair timing between the stages varies considerably between studies (1-96 months).

 

 

 

 

Facultative:

 

Pooling of results is not possible due to the heterogeneity of included studies.

 

Furthermore, definitive conclusions about maxillary growth connot be drawn based on the results of current literature.

 

Level of evidence: GRADE (very low

 

 

 


 

Evidence table for intervention studies (randomized controlled trials and non-randomized observational studies [cohort studies, case-control studies, case series]

Study reference

Study characteristics

Patient characteristics 2

Intervention (I)

Comparison / control (C) 3

 

Follow-up

Outcome measures and effect size 4

Comments

Bartzela, 2010

Type of study: retrospective observational

 

Setting: outpatients

 

Country: the Netherlands

 

Source of funding: not reported

Inclusion criteria:

1) patients with bilateral cleft lip and palate who underwent surgery in one of the participating centers

2) Caucasian ethnic background

3) no associated congenital malformations, syndromes or mental retardation

4) treatment from birth onward in the same center

5) born before 1996

 

Exclusion criteria:

-

 

N total at baseline:

A: 56

B: 37

C: 107

 

Important prognostic factors2: -

 

Groups comparable at baseline? unclear

 

Describe intervention (treatment/procedure/test):

 

 

Center A:

Birth: infant orthopaedics (1.5 yrs), nose plugs (2.5 yrs)

3 months: bilateral lip adhesion

6 months: soft palate closure (center’s own technique)

18 months: definitive bilateral lip and nose repair (center’s own technique)

8 years: one-side alveolar bone grafting

8.5 years: hard palate closure with alveolar bone grafting of second side

 

Center B:

Birth: infant orthopaedics with extraoral strapping (9.2 months)

7 months: one-stage lip closure (modified Manchester)

14 months: Von Langenbeck soft palate closure

10 years: hard palate closure and bilateral alveolar bone grafting and osteotomy of the premaxilla

 

Center C:

3 months: straight-line lip closure and hard palate closure on one side

5 months: straight-line lip closure and hard palate closure on the other side

19 months: Von Langenbeck soft palate closure

10 years: bilateral alveolar bone grafting

 

Describe control (treatment/procedure/test):

 

 

See Intervention column, 3 different center protocols were compared

Length of follow-up:

Until at least 11 years of age

 

Loss-to-follow-up:

23/204 (11%)

at 12 years

 

Incomplete outcome data:

23/204 (11%)

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Bauru-BCLP yardstick score: assesses dental arch relationship

 

Linear regression analysis for increments at different age intervals.

Center C is reference category

 

6-12 years

Center B: 10% (95% CI: -0.4% - 214%), p=0.041

Center A: 4% (95% CI: -7% - 15%) p=0.53

 

9-12 years

Center B: 13% (95% CI: 3% - 27%) p=0.009

Center A: 6% (95% CI: -3% - 16%) p=0.21

 

6 – 9 years

Center B: -4% (95% CI: -11% - 3%)

Center A: -.2% (95% CI: -7% - 8%) p=0.95

 

Author’s conclusion: Despite different treatment protocols, dental arch relationships in final scores at age 9 and 12 years. Delaying hard palate closure and employing infant orthopaedics did not appear to be disadvantageous in the long run. Premaxillary osteotomy employed in center B appeared to be associated with less favourable development of the dental arch relationship between 9 and 12 years.

Friede 2001

Type of study: retrospective observational

 

Setting: outpatients (2 centers)

 

Country: Sweden and Denmark

 

Source of funding: not reported

Inclusion criteria:

1) nonsyndromic patients with unilateral cleft palate treated in one of the two participating centers

 

Exclusion criteria:

-

 

N total at baseline:

Early closure (E): 29

Delayed closure (D): 30

 

Important prognostic factors2:

 

Sex:

E: 83% M

C: 77% M

 

Groups comparable at baseline? unclear

 

Describe intervention (treatment/procedure/test):

 

 

early hard palate closure:

vomer flap procedure at 3 months together with lip closure

 

and then a pushback procedure at 22 months

 

 

Describe control (treatment/procedure/test):

 

 

late hard palate closure:

 velum repair at 8 months

 and then hard palate closure at 8.5 years together with bone grafting of alveolar cleft

Length of follow-up:

Until 16 years of age

 

Loss-to-follow-up:

Not reported

 

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Maxillary growth:

 

(Lateral röntgencephalograms)

 

Children that had undergone early hard palate closure generally had more retrognathic faces (more maxillary retrusion and thus less facial convexity) than those who underwent late hard palate closure.

 

The ANB angle only became negative in the patients who underwent late hard-palate closure and only in the older (14-16 years) age groups.

 

The anterior vertical development of the maxilla was most satisfactory in patients who underwent delayed closure of the hard palate.

 

Overall, the midfacial development was more favourable in the delayed hard palate closure group.

 

 

All E surgeries performed in one center and all D surgeries perfomed in another center.

Fudalej, 2011

Type of study: prospective observational

 

Setting: outpatients

 

Country: Poland, Czech Republic, the Netherlands

 

Source of funding: non-commercial

Inclusion criteria:

1) children with non-syndromic complete unilateral cleft lip and palate with and without simonart’s bands treated at the participating hospitals in Warsaw and Nijmegen

 

Exclusion criteria:

-

 

N total at baseline:

Intervention: 61

Control: 97

 

Important prognostic factors2:

For example

age ± SD:

I: 11.2 years

C: 8.7 years

 

Sex:

I: 67% M

C: 77% M

 

Groups comparable at baseline?

yes

Describe intervention (treatment/procedure/test):

 

Warsaw protocol:

 

6-12 months: one-stage lip, soft and hard palate closure

9-11 years: bone grafting

Describe control (treatment/procedure/test):

 

Nijmegen protocol:

 

0-6 months: infant orthopaedics

6-12 months: lip closure (Millard)

12-14 months: soft palate closure; Modified von Langenbeck

9-11 years: bone grafting and hard palate closure

Length of follow-up:

Until 11 years

 

Loss-to-follow-up:

unclear

 

Incomplete outcome data:

unclear

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

EUROCRAN index used to grade dental arch relationship and palatal morphology of dental casts

 

Dental arch: grade 1 (best) to 4 (worst)

I: 2.58 (95% CI: 2.52 – 2.82)

C: 1.97 (95% CI: 1.79 – 2.15)

P<0.001

 

Palatal morphology: grade 1 (good) to 3 (worst)

I: 1.79 (95% CI: 1.68 – 1.90)

C: 1.96 (95% CI: 1.85 – 2.07)

P=0.047

 

 

 

Author’s conclusion: The dental arch relationship following one-stage repair was less favourable than the outcome of three-stage repair. The palatal morphology following one-stage repair, however, was more favourable than the outcome of three-stage repair

Goodacre 2003

Type of study: prospective observational

 

Setting: outpatients (4 centers)

 

Country: United Kingdom

 

Source of funding: not reported

Inclusion criteria:

1) patients born in one of the participating hospitals with cleft palate or healthy control patient born around the same time

 

Exclusion criteria:

1) multiple births

2) gestational age <36 months

3) birth weight <2.25kg

4) presence of any syndrome

 

N total at baseline:

Early lip closure: 34 (42 in experiment 3)

Late lip closure: 29 (43 in experiment 3)

Healthy controls: 80

 

Important prognostic factors2:

Not reported

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

 

Early lip closure

At median 4.5 days (range 0-19)

 

Describe control (treatment/procedure/test):

 

 

Late lip closure

At median 18 days (range 2-152)

 

Length of follow-up:

12 months

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Static attractiveness as rated by surgeons postoperatively

(experiment 1)

Mean difference (early – late): -0.10 (95% CI: -2.3 – 0.44, p=0.54)

 

Static attractiveness as rated by laypersons postoperatively

(experiment 2)

Mean difference (early – late): -0.11 (95% CI: -0.42 – 0.11, p=0.47)

 

Dynamic attractiveness as rated by laypersons at 12 months

(experiment 3)

Mean difference (early – late): 0.08 (95% CI: -0.11 – 0.28, p=0.41)

 

Grobbelaar 1994

Type of study: prospective observational

 

Setting: outpatients (1 center)

 

Country: South Africa

 

Source of funding: not reported

Inclusion criteria:

1) children with a cleft palate registered at the participating clinic

 

Exclusion criteria:

 

N total at baseline:

Early closure: 77

Late closure: 107

 

Important prognostic factors2:

Not reported

 

Groups comparable at baseline?

 

Describe intervention (treatment/procedure/test):

 

 

Palate closure perfomed before 6 months

 

 

Dorrance: 13

Wardill: 14

Perko: 13

Furlow: 11

Von Langenbeck: 26

 

 

Describe control (treatment/procedure/test):

 

 

Palate closure perfomed after 6 months

 

Dorrance: 12

Wardill: 27

Perko: 6

Furlow: 9

Von Langenbeck: 47

 

Length of follow-up:

Median 9.6 years

(range 3-24)

 

Loss-to-follow-up:

290 registered at clinic

37 never returned for surgery

50 never returned after their operation

19 attended only once

 

Incomplete outcome data:

Intervention:

N (%)

Reasons (describe)

 

Control:

N (%)

Reasons (describe)

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Velopharyngeal insufficiency:

 

Early closure: 4/77

Late closure: 9/107

P<0.05

 

 

 

 

Other speech results except for velopharyngeal insufficiency not reported stratified by timing of operation.

Gundlach 2013

Type of study: retrospective observational

 

Setting: outpatients (3 centers)

 

Country: Germany / United States of America

 

Source of funding: no funding

Inclusion criteria:

1) non-syndromal patients with complete unilateral clefts of lip, alveolus and palate

 

Exclusion criteria:

-

 

N total at baseline:

One-stage closure (O): 35 (center A and B)

Two stage closure (T): 50 (center C)

 

Important prognostic factors2:

O: 7-9 year olds: 18

15-17 year olds: 32

T: 7-9 year olds: 18

15-17 year olds: 17

 

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

 

Labioplasty at 3 months (Triangular Flap Method, modification on Tennison’s Stencil Method)

 

Veloplasty at 12-18 months

(Two Flap Technique, modification of Veau’s two-pedicled palatal flaps)

 

Describe control (treatment/procedure/test):

 

 

Labioplasty at 6 months

(Waveline procedure)

 

Veloplasty

At 15-18 months in center B (n=24)

At 3 years in center A (n=26)

(Intravelar Veloplasty according to Kriens)

Length of follow-up:

Until the age of 7-9 years n=36

Until the age of 15-17 years n=49

 

 

Loss-to-follow-up:

Not described

 

Incomplete outcome data:

Not described

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Maxillary growth:

 

Anterior cross-bites:

Least often in center A

At 7-9 years

P<0.05

 

Posterior cross-bites:

Most often in center B

At 15-17 years

P<0.05

 

Constricted palates (or dental arches):

Highest rate in center C

At 7-9 years

P<0.05

 

 

Speech:

 

Success rates:

Center A: 70%

Center C: 74%

p>0.05

 

Speech results published earlier (1987) and briefly mentioned in Discussion section

Landheer 2010

Type of study: retrospective observational

 

Setting: outpatients (one center)

 

Country: Netherlands

 

Source of funding: not reported

Inclusion criteria:

1) all consecutive children treated for cleft (lip and) palate, born between January 1 1988 and December 31 1997, who received cleft palate repair at the Utrecht Medical Center

 

Exclusion criteria:

-

 

N total at baseline:

Early closure of hard palate(1-stage): 121

Late closure of hard palate (2-stage): 154

 

Important prognostic factors2:

Only reported for group as a whole:

 

Age at last follow-up: 12.5 ± 7.3 years

 

Sex: 58%M

 

Syndrome: 56 (20%)

 

Cleft type (Veau): I : 6%

II: 34%

III: 40%

IV: 56%

 

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

 

Soft and hard palate closure at 6-9 months

 

(1-stage repair)

 

Describe control (treatment/procedure/test):

 

 

Soft and hard palate closure at 6-9 months

 

Hard palate closure by a modified von Langenbeck procedure at 4-7 years; when the speech therapist felt that the open hard palate may influence speech.

 

(2-stage repair)

Length of follow-up:

Median 9.3 years,

IQR: 7.2 years

 

Loss-to-follow-up:

Not described

 

Incomplete outcome data:

Two subanalyses were performed due to incomplete data for some of the 275 patients (DNR):

-among patients with cleft repair in 2 stages

-among patients with recorded preoperative cleft width

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Fistula:

 

Odds ratio of fistula formation in 2-stage repair versus 1-stage repair:

2.3 (95% CI: 1.2 – 4.3)

 

 

 

 

All patients underwent the cleft palate repair in 2 stages, unless the operating surgeon considered the cleft narrow enough to close in 1 operation.

Thus the patients undergoing 1-stage repair had a narrower cleft to start with à selection bias.

Kirschner 2000

Type of study: retrospective observational

 

Setting: oupatients (1 center)

 

Country: United States of Amerika

 

Source of funding: not reported

Inclusion criteria:

1) Veau class III unilateral cleft lip and palate who underwent palatoplasty at the participating center

 

Exclusion criteria:

1) moderate or severe hearing loss

2) associated major craniofacial malformations

3) associated syndromes

4) palate repair included primary posterior pharyngeal flap

5) unintentional oronasal fistulas

 

N total at baseline:

Early repair: 40

Late repair: 50

 

Important prognostic factors2:

For example

age ± SD:

E: 8.3 ± 3.2

L: 8.7 ± 3.0

 

Sex: not reported

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

 

 

Palate closure at 3-7 months

Modified Furlow technique

Describe control (treatment/procedure/test):

 

 

Palate closure after 7 months

Modified Furlow technique

Length of follow-up:

 

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

No significant difference between the groups in terms of :

Resonance

Nasal air emission

Velopharyngeal function

 

Klintö 2014

Type of study: prospective observational

 

Setting: outatients (2 centers)

 

Country: Sweden

 

Source of funding: not reported

Inclusion criteria:

1) patients with complete unilateral cleft palate patients

2) monolingual speakers of Swedish

 

Exclusion criteria:

1) known malformations or syndromes

 

N total at baseline:

Two-stage palate closed (TSC): 9

Two-stage open (TSO): 9

One stage closed (OSC): 10

 

Important prognostic factors2:

Not reported

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

1) TSC:

Early soft palate repair with lip closure at 4.6 months

(Scandleft soft palate repair)

Hard palate closure at 12 months

(cranially based vomer flap)

 

2) TSO:

Early soft palate repair with lip closure at 4.6 months

(Scandleft soft palate repair)

Hard palate closure at 12 months

(cranially based vomer flap)

 

 

 

Describe control (treatment/procedure/test):

 

OSC

Lip repair or adhesion at 3-6 months

One stage palate repair at 14 months

(intravelar veloplasty)

Length of follow-up:

Until the age of 3 years

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Speech:

 

Correct consonats, adjusted for age:

Median (range)

TSC: 76% (14-91%)

TSO: 59% (3-92%)

OSC: 86% (41-97%)

P=0.06

 

Active cleft speech characteristics:

Median (range)

TSC: 16% (3-51%)

TSO: 29% (4-64%)

OSC: 6% (0-26%)

P=0.015

 

Total number of phonological processes

Median (range)

TSC: 21 (6-72)

TSO: 35 (9-76)

OSC: 13 (4-44)

P=0.033

 

Number of different types of phonological processes:

Median (range)

TSC: 8 (3-12)

TSO: 9 (4-16)

OSC: 6 (3-13)

P=0.18

 

Hypernasality: lowest prevalence in OSC group, highest in TSO group (p=0.013)

 

No significant differences in hyponasality or nasal air leakage between groups.

 

Audiometry:

 

Not reported

 

In two-stage palate closure all patients were operated in one center and randomized to late or early hard palate closure. One-stage palate closure patients operated in another center.

 

None of the patients received speech therapy

O’Gara 1994

Type of study: prospective observational

 

Setting: outpatients (1 center)

 

Country: United States of Amerika

 

Source of funding: not reported

Inclusion criteria:

1) babies born with non-syndromic complete unilateral cleft palate

 

Exclusion criteria:

-

 

N total at baseline:

Early closure (E): 11

Late closure (L): 12

 

Important prognostic factors2:

For example

age ± SD:

not reported

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

Palatoplasty before 12 months

 

Techniques: von Langenbeck (55%), Wardill, V-Y pushback (27%), with or without levator reconstruction

 

Describe control (treatment/procedure/test):

 

Palatoplasty after 12 months

 

Techniques: von Langenbeck (67%), Wardill (17%), V-Y pushback , with or without levator reconstruction

 

Length of follow-up:

Babies followed from 5 – 35 months

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Speech:

 

Frequency of oral stops significantly higher in the early closure group after 11 months (p=0.02):

 

14-18 months

E: 45±8

L: 15 ± 4

 

30-35 months:

E: 62 ± 5

L: 34 ± 6

 

Frequencies of other speech features do not differ significantly between groups.

Each baby’s readiness for palatoplasty was evaluated on an individual basis by the plastic surgeon of the team.

The goal was to operate as early as possible, but with the greates chance of success.

Randag 2014

 

Type of study: retrospective observational

 

Setting: outpatients

 

Country: the Netherlands

 

Source of funding: not reported (no financial conflict of interest of authors reported either)

Inclusion criteria:

 

Exclusion criteria:

 

N total at baseline:

One-stage closure (O): 24

Two-stage closure (T): 24

 

Important prognostic factors2:

Not reported

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

one-stage palate closure at 10 months

 

 

lip closure at 3-6 months, soft palate was closed using intravelar veloplasty.

Hard palate was closed using the von Langenbeck technique: a two-flap palatoplasty or a hybrid palatoplasty depending on the width of the cleft.

 

 

Describe control (treatment/procedure/test):

 

two-stage palate closure at 11 and 18 months

 

lip closure at 3-6 months, soft palate was closed using intravelar veloplasty.

Hard palatum was closed using the von Langenbeck technique: a two-flap palatoplasty or a hybrid palatoplasty depending on the width of the cleft

Length of follow-up:

Until 2.5 years of age

 

Loss-to-follow-up:

Only reported for one-stage closure group: 27/51

13: cleft of soft palate only

3 lost to follow-up

9 different mother tongue

15 insufficient language comprehension

 

Incomplete outcome data:

Not described

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Speech:

 

Articulation: number of correctly pronounced initial consonants:

O: 9.7±4.5

T: 7.0 ± 3.8

P=0.03

 

Inadequate language production:

O: 1/24 (4%)

T: 3/24 (13%)

P=0.61

 

Resonance problems:

O: 6/24 (26%)

T: 8/24 (33%)

P=0.75

 

Nasal air emissions:

O: 12/24 (53%)

T: 15/24 (63%)

P=0.56

 

Good intelligibility:

O: 10 (44%)

T: 3 (19%)

P=0.13

 

 

 Short term complications:

 

Postoperative haemorrhage

O: 1/24

T: 4/24

p>0.05

 

No difference in other short term complications (DNR)

 

Richard 2006

Type of study: randomized controlled trial

 

Setting: outpatients

 

Country: United Kingdom

 

Source of funding: non-commercial

Inclusion criteria:

1) children with non-syndromic unilateral cleft palate

2) age 3-60 months

 

Exclusion criteria:

-

 

N total at baseline:

I: Posterior-anterior (P-A): 23

C: Anterior-posterior (A-P): 24

 

Important prognostic factors2:

For example

age

I: 18.7 months

C: 18.8 months

 

Sex:

I: 67% M

C: 46% M

 

Groups comparable at baseline? Yes

 

Describe intervention (treatment/procedure/test):

 

 

Posterior – anterior order of palate closure

 

(The anterior operation consisted of a lip repair by Millard rotation advancement, a nasal correction using the McComb procedure and a hard palate repair by a single layer vomerine flap. The posterior operation consisted of a soft palate repair with medial von Langenbeck incisions. The two operations were undertaken 3 months apart with the first operation at 19 months of age.)

Describe control (treatment/procedure/test):

 

 

Anterior-posterior order of palate closure:

 

(The anterior operation consisted of a lip repair by Millard rotation advancement, a nasal correction using the McComb procedure and a hard palate repair by a single layer vomerine flap. The posterior operation consisted of a soft palate repair with medial von Langenbeck incisions. The two operations were undertaken 3 months apart with the first operation at 19 months of age.)

Length of follow-up:

Until the age of 4-6 years

 

Loss-to-follow-up:

I: 6/23 (26%)

1 died, 5 lost contact

 

C: 4/24 (17%)

1 syndromic, 3 lost contact

 

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Maxillary growth:

 

No significant differences in cephalometric measurements between groups

 

 

Speech:

 

No significant differences in speech between groups.

 

Hearing loss:

 

No significant differences in hearing status between groups.

 

Fistula formation:

 

No significant differences in prevalence of symptomatic fistulae between groups.

 

No power analysis for sample size is presented. Bias due to imprecision?

Rohrich 1996

Type of study: retrospective observational

 

Setting: outpatients (1 center study)

 

Country: United States of America

 

Source of funding: not reported

Inclusion criteria:

1) patients with unilateral and bilateral cleft of the alveolus and secondary palate born during the perion 1960 – 1969 and treated at the Oxfor Plastic Surgery Department

 

Exclusion criteria:

-

 

N total at baseline:

Early closure (E): 21

Late closure (L): 23

 

Important prognostic factors2:

For example

age (range):

E: 17 (15-19)

L: 18 (14-21)

 

Sex:

E: 57% M

L: 61 % M

 

Bilateral cleft type:

E: 29%

L: 30%

 

Groups comparable at baseline? Yes

 

Describe intervention (treatment/procedure/test):

 

 

Cleft repair (Miljard rotation-advancement) at 3.4 months.

 

Soft palate repair plus hard palate repair at 11 months using the modified three- or four-flap Wardill-Kilner procedure.

Describe control (treatment/procedure/test):

 

 

Cleft repair (Miljard rotation-advancement) at 3.4 months.

 

Soft palate repair at 11 months using short Veau flaps

 

Second-stage hard palate closure with a vomer flap at 49 months.

Length of follow-up:

Average 12.8 years

 

Loss-to-follow-up:

91 patient operated between 1960 – 1969.

 

In 1996 68 completed and returned questionnaire.

 

44 were randomly selected for interview and examination.

 

Incomplete outcome data:

Not described

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Speech:

 

Articulation errors:

E: 5%

L: 10%

P not reported

 

Nasal resonance:

E: 30%

L: 81%

P<0.001

 

Hyponasality:

More in L group (p<0.01, DNR)

 

Nasal emission:

No difference between groups (DNR)

 

Speech intelligibility impaired:

E: 5%

L: 35%

P<0.02

 

Normal articulation patterns: no difference between groups (DNR)

 

Maxillary growth:

 

arch width: no difference between groups (DNR)

 

anterior maxillary collapse: no difference between groups (DNR)

 

posteriorly a relatively normal relationship at the molar level without maxillary collapse: no difference between groups (DNR)

 

Maxillary hypoplasia: no difference between groups (DNR)

 

Midfacial hypoplasia: no difference between groups (DNR)

 

Hearing:

 

No significant difference between E and L

Tendency for severe hearing loss in L (DNR, p<0.50)

 

Complications:

 

Overall fistulas

E: 29%

L: 61%

P<0.05

 

Persistent fistula

E: 5%

L: 35%

p<0.05

 

Overall palatal fistulas in unilateral cleft types:

E: 27%

L: 69%

P<0.05

 

All operative procedures in both groups were performed by the same plastic surgeon.

Da Silva Filho 2000

Type of study: retrospective observational

 

Setting: outpatients (2 centers)

 

Country: Brazil

 

Source of funding: not reported

Inclusion criteria:

1) patients with unilateral cleft palate treated in one of the participating centers

 

Exclusion criteria:

-

 

N total at baseline:

Early palate closure (E): 22

Delayed palate closure (D): 53

 

Important prognostic factors2:

 

Sex:

I: 55% M

D: 62% M

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

early palate closure
lip repair at 3-27 months and

 

palate repair at 12-44 months.

 

(The exact type of surgery was not reported.)

 

 

Describe control (treatment/procedure/test):

 

late hard palate closure:

lip and soft palate repair at 5.5 months

 

hard palate repair at 20 months

 

(The exact type of surgery was not reported.)

 

Length of follow-up:

Age 4-7 years

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Maxillary growth:

 

(lateral röntgencephalograms)

 

No significant difference between groups in any of the cephalometric measurements

All E surgeries performed in one center and all D surgeries perfomed in another center.

Trotman 1996

Type of study: retrospective observational

 

Setting: outpatients

 

Country: United States of America

 

Source of funding: not reported

Inclusion criteria:

1) unilateral cleft palate patients treated in one of the two participating centers

 

Exclusion criteria:

1) other congenital abnormalities

2) previous orthodontic treatment

 

N total at baseline:

One-stage (O): 43

Two-stage (T): 43

 

Important prognostic factors2:

 

Sex:

O: 56% M

T: 56% M

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

primary alveolar bone grafting with definite lip repair at 4-6 months

 

and hard and soft palate repair at 6-12 months.

 

 

Describe control (treatment/procedure/test):

 

triangular flap lip repair at 3 months

 

and staged surgeries of soft and hard palate completed by 18 months with no alveolar bone grafting. were studied.

 

Length of follow-up:

Until the age of 10 years

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Maxillary growth:

 

(Lateral cephalometric radiographs)

 

Patients in the center that used one-stage palatal closure had faces that were on average less maxillary protrusive compared with the faces of the non-grafted patients (intervention) other center.

However in the two-stage center the mandible compensated for the maxillary protrusion by downward and backward rotation.

 

Overall, a similar jaw relationship was noted in both groups, although in the one-stage closure group the anterior facial height increased.

 

All O surgeries performed in one center and all T surgeries performed in another center.

Wada 1990

Type of study: randomized controlled trial

 

Setting: outpatients

 

Country: Japan

 

Source of funding: no funding

Inclusion criteria:

1) patients with unilateral or bilateral cleft palate

 

Exclusion criteria:

 

N total at baseline:

One stage closure:

Unilateral cleft: 14

Bilateral cleft: 8

 

Two stage closure:

Unilateral cleft: 16

Bilateral cleft: 7

 

Important prognostic factors2:

Not reported

 

Groups comparable at baseline?

Unclear

Describe intervention (treatment/procedure/test):

 

 

Lip repair at 5 months by Tennison’s procedure

 

Mucoperiosteal palatal pushback procedure at 20 months

(one-stage closure)

Describe control (treatment/procedure/test):

 

 

Lip repair at 5 months by Tennison’s procedure

 

Primary veloplasty at 20 months

 

Double overlapping palatal hingeflap procedure at 5 years 10 months

 

(two-stage closure)

Length of follow-up:

Until the age of 10 years

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not described

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Maxillary growth:

In the unilateral cleft palate patients the maxillary growth of the patients who underwent the two-stage closure was comparable to those of the non-cleft controls regarding depth and height of the maxilla, while the one-stage closure patients had aberrant maxillary development.

 

For the patients with bilateral clefts, the maxillary growth was similar in the one-stage and two-stage palatal closure groups.

 

 

Williams 2009

Type of study: RCT

 

Setting: outpatients 91 center)

 

Country: United States of Amerika and Brazil

 

Source of funding: non-commercial

Inclusion criteria:

1) patients with cleft lip at palate, of age and in good health for surgery

 

Exclusion criteria:

1) family and patient did not show up for scheduled surgery date

2) condition that could interfere with speech development

 

 

N total at baseline:

Early surgery (E): 181

Late surgery (L): 195

 

Important prognostic factors2:

Not reported

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

Palate closure at 9-12 months of age

 

Spina – Furlow=35

Millard – Furlow= 43

Spina – von Langenbeck= 51

Millard – von Langenbeck= 52

 

 

Describe control (treatment/procedure/test):

 

Palate closure at 15-18 months of age

 

Spina – Furlow=48

Millard – Furlow= 47

Spina – von Langenbeck= 46

Millard – von Langenbeck= 54

 

Length of follow-up:

Until at least the age of 4 years

 

Loss-to-follow-up:

After surgery 31/498 (6%)

Reasons not described

 

Incomplete outcome data:

Not described

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Odds ratio (OR)

 

Speech:

 

Hypernasality:

E: 79%

L: 73%

OR (E versus L): 1.46 (95% CI: 0.84 – 2.54, p=0.12)

 

Nasal air emission:

E: 57%

L: 54%

OR (E versus L): 1.16 (95% CI: 0.72 – 1.85, p=0.49)

 

Fistula formation:

 

E: 44/181

L: 37 / 195

OR (E versus L): 1.37 (95% CI: 0.84 – 12.22, p=0.21)

 

Method of randomization and (presence or absence of) blinding unclear.

 

Statistical analyses presented very adequately.

Ysunza 1998

Type of study: prospective observational

 

Setting: outpatients

 

Country: Mexico

 

Source of funding: not reported

Inclusion criteria:

1) unilateral complete cleft of primary or secondary palate, patient normal in all other respects

2) cleft palate width I or II

3) patients were in adequate conditions for surgical repair at 6 months

4) surgery performed by standard surgical routine of clinic

5) speech evaluation performed at 48 months

6) underwent videopharyngoscopy and multiview videofluorocopy

7) linguistic development within normal limits

8) normal hearing as demonstrated by pure tone audiometry

9) maxillofacial growth evaluation performed at 48 months of age

 

Exclusion criteria:

1) patients with postoperative fistulae

 

N total at baseline:

Early closure (E): 41

Late closure (L): 35

 

Important prognostic factors2:

 

Sex:

I: 46% M

C: 51% M

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

 

 

Minimal incision palatopharyngoplasty at 6 months of age

Describe control (treatment/procedure/test):

 

 

 

Minimal incision palatopharyngoplasty at 12 months of age

Length of follow-up:

Until the age of 4 years

 

Loss-to-follow-up:

580 cleft palate cases reviewed

76 patients met all the inclusion criteria

 

Incomplete outcome data:

Not decribed

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Speech:

 

Articulation development below normal limits for patient’s age in all cases. No significant difference between groups (DNR)

 

 

Maxillary growth:

 

No significant differences in maxillary or cephalometric parameters between the groups (DNR)

 

Both group had anterior maxillary collapse but normal development of the posterior maxilla (DNR)

Only included patients with cleft palate width I or II.

 

Patients with postoperative fistulae excluded from study group.

Zemann 2011

Type of study: retrospective observational

 

Setting: outpatients (2 centers)

 

Country: Austria, Slovenia

 

Source of funding: not reported

Inclusion criteria:

1) children with unilateral cleft palate treated in one of the participating centers

 

Exclusion criteria:

-

 

N total at baseline:

One-stage closure (O): 22

Two-stage closure (T): 32

 

Important prognostic factors2:

 

Sex:

O: 59% M

C: 66% M

 

Groups comparable at baseline? Unclear

 

Describe intervention (treatment/procedure/test):

 

one stage palate closure:

 

lip repair at 3 months by Millard

 

one-stage palatal closure within 12 months by Veau

Describe control (treatment/procedure/test):

 

two-stage palate closure:

 

lip repair within 6 months by Tennison-Randall,

 

soft palate repair at 12 months by intravelar velaplasty

 

 hard palate repair at 30 months by Veau

Length of follow-up:

Age of 6-10 years

 

Loss-to-follow-up:

Not reported

 

Incomplete outcome data:

Not reported

 

 

Outcome measures and effect size (include 95%CI and p-value if available):

 

Maxillary growth:

 

(Lateral röntgencephalograms)

 

no statistically significant difference in cephalometric parameters between the patients undergoing one-stage versus two-stage palate closure.

 

However, in the one-stage closure center there was considerably normal saggital facial growth, with tendency to forward growth of the mandible.

 

In the two-stage closure group there was a slight decrease in sagittal maxillary growth and mandibular growth with unchanged jaw relation.

 

All O surgeries performed in one center and all T surgeries perfomed in another center.

 

 


 

Table of quality assessment for systematic reviews of RCTs and observational studies

Study

 

 

 

 

First author, year

Appropriate and clearly focused question?1

 

 

 

Yes/no/unclear

Comprehensive and systematic literature search?2

 

 

 

Yes/no/unclear

Description of included and excluded studies?3

 

 

 

Yes/no/unclear

Description of relevant characteristics of included studies?4

 

 

Yes/no/unclear

Appropriate adjustment for potential confounders in observational studies?5

 

 

Yes/no/unclear/notapplicable

Assessment of scientific quality of included studies?6

 

 

Yes/no/unclear

Enough similarities between studies to make combining them reasonable?7

 

Yes/no/unclear

Potential risk of publication bias taken into account?8

 

 

Yes/no/unclear

Potential conflicts of interest reported?9

 

 

 

Yes/no/unclear

Liao 2006

Yes

Yes

No

Yes

Not applicable

Yes

No

No

No

Nollet 2005

Yes

Yes

No

Yes

Unclear

No

Unclear

No

No

Yang 2010

`Yes

Yes

No

Yes

Not applicable

Yes

No

No

No

 

  1. Research question (PICO) and inclusion criteria should be appropriate and predefined
  2. Search period and strategy should be described; at least Medline searched; for pharmacological questions at least Medline + EMBASE searched
  3. Potentially relevant studies that are excluded at final selection (after reading the full text) should be referenced with reasons
  4. Characteristics of individual studies relevant to research question (PICO), including potential confounders, should be reported
  5. Results should be adequately controlled for potential confounders by multivariate analysis (not applicable for RCTs)
  6. Quality of individual studies should be assessed using a quality scoring tool or checklist (Jadad score, Newcastle-Ottawa scale, risk of bias table etc.)
  7. Clinical and statistical heterogeneity should be assessed; clinical: enough similarities in patient characteristics, intervention and definition of outcome measure to allow pooling? For pooled data: assessment of statistical heterogeneity using appropriate statistical tests (e.g. Chi-square, I2)?
  8. An assessment of publication bias should include a combination of graphical aids (e.g., funnel plot, other available tests) and/or statistical tests (e.g., Egger regression test, Hedges-Olken). Note: If no test values or funnel plot included, score “no”. Score “yes” if mentions that publication bias could not be assessed because there were fewer than 10 included studies.
  9. Sources of support (including commercial co-authorship) should be reported in both the systematic review and the included studies. Note: To get a “yes,” source of funding or support must be indicated for the systematic review AND for each of the included studies.

 

 

 

Risk of bias table for intervention studies (randomized controlled trials)

Study reference

 

(first author, publication year)

Describe method of randomisation1

Bias due to inadequate concealment of allocation?2

 

 

(unlikely/likely/unclear)

Bias due to inadequate blinding of participants to treatment allocation?3

 

(unlikely/likely/unclear)

Bias due to inadequate blinding of care providers to treatment allocation?3

 

(unlikely/likely/unclear)

Bias due to inadequate blinding of outcome assessors to treatment allocation?3

 

(unlikely/likely/unclear)

Bias due to selective outcome reporting on basis of the results?4

 

 

(unlikely/likely/unclear)

Bias due to loss to follow-up?5

 

 

 

(unlikely/likely/unclear)

Bias due to violation of

intention to treat analysis?6

 

 

(unlikely/likely/unclear)

Richard 2006

“randomly allocated, stratified by block randomization”

Unclear

Unclear

Unlikely

Unclear

Unlikely

Unclear

Unclear

Wada 1990

“randomly assigned”???

Unclear

Unclear

Unlikely

Unclear

Unlikely

Unclear

Unclear

Williams 2009

“block randomization plan” ???

Unclear

Unclear

Unlikely

Unclear

Unlikely

Unclear

Unclear

 

  1. Randomisation: generation of allocation sequences have to be unpredictable, for example computer generated random-numbers or drawing lots or envelopes. Examples of inadequate procedures are generation of allocation sequences by alternation, according to case record number, date of birth or date of admission.
  2. Allocation concealment: refers to the protection (blinding) of the randomisation process. Concealment of allocation sequences is adequate if patients and enrolling investigators cannot foresee assignment, for example central randomisation (performed at a site remote from trial location) or sequentially numbered, sealed, opaque envelopes. Inadequate procedures are all procedures based on inadequate randomisation procedures or open allocation schedules..
  3. Blinding: neither the patient nor the care provider (attending physician) knows which patient is getting the special treatment. Blinding is sometimes impossible, for example when comparing surgical with non-surgical treatments. The outcome assessor records the study results. Blinding of those assessing outcomes prevents that the knowledge of patient assignment influences the process of outcome assessment (detection or information bias). If a study has hard (objective) outcome measures, like death, blinding of outcome assessment is not necessary. If a study has “soft” (subjective) outcome measures, like the assessment of an X-ray, blinding of outcome assessment is necessary.
  4. Results of all predefined outcome measures should be reported; if the protocol is available, then outcomes in the protocol and published report can be compared; if not, then outcomes listed in the methods section of an article can be compared with those whose results are reported.
  5. If the percentage of patients lost to follow-up is large, or differs between treatment groups, or the reasons for loss to follow-up differ between treatment groups, bias is likely. If the number of patients lost to follow-up, or the reasons why, are not reported, the risk of bias is unclear
  6. Participants included in the analysis are exactly those who were randomized into the trial. If the numbers randomized into each intervention group are not clearly reported, the risk of bias is unclear; an ITT analysis implies that (a) participants are kept in the intervention groups to which they were randomized, regardless of the intervention they actually received, (b) outcome data are measured on all participants, and (c) all randomized participants are included in the analysis.

 

 

Risk of bias table for intervention studies (observational: non-randomized clinical trials, cohort and case-control studies)

Study reference

 

(first author, year of publication)

Bias due to a non-representative or ill-defined sample of patients?1

 

 

 

(unlikely/likely/unclear)

Bias due to insufficiently long, or  incomplete follow-up, or differences in follow-up between treatment groups?2

 

 

(unlikely/likely/unclear)

 

Bias due to ill-defined or inadequately measured outcome ?3

 

 

 

(unlikely/likely/unclear)

Bias due to inadequate adjustment for all important prognostic factors?4

 

 

 

(unlikely/likely/unclear)

Bartzela, 2009

Unlikely

Unlikely

Unlikely

Unclear

Friede 2000

Unlikely

Unlikely

Unlikely

Unclear

Fudalej, 2011

Unlikely

Unlikely

Unlikely

Unclear

Goodacre 2003

Unlikely

Unlikely

Unlikely

Unclear

Grobbelaar 1994

Unlikely

Unlikely

Unlikely

Unclear

Gundlach 2013

Unlikely

Unlikely

Unlikely

Unclear

Kirschner 1999

Unlikely

Unlikely

Unlikely

Unclear

Klintö 2014

Unlikely

Likely

Unlikely

Unclear

Landheer 2010

Likely

Unlikely

Unlikely

Likely

O’Gara 1994

Likely

Unlikely

Unlikely

Likely

Randag 2014

Unlikely

Likely

Unlikely

Unclear

Rohrich 1996

Unlikely

Unlikely

Unlikely

Unclear

Da Silva Filho 2000

Unlikely

Likely

Unlikely

Unclear

Trotman 1996

Unlikely

Unlikely

Unlikely

Unclear

Ysunza 1998

Unlikely

Likely

Unlikely

Unclear

Zemann 2011

Unlikely

Likely

Unlikely

Unclear

Overwegingen

Er is veel onderzoek gedaan naar de timing van het operatief sluiten van het palatum durum met veelal weinig bewijslast. Uit de beschikbare literatuur komt naar voren dat er geen bewijs is voor een eenduidig sluitend protocol betreffende het vroeg of laat sluiten van het palatum durum dat zowel beter voor de spraak is en niet slechter voor de groei van de maxilla. Naast timing zijn er nog diverse – veelal niet te kwantificeren – overige onbekende invloeden op de uitgroei van de maxilla die meespelen zoals; de techniek van palatumsluiting en de bijkomende littekens (kunst en kunde) en genetische predispositie (familiaire belasting en raciale kenmerken). Er bestaat geen literatuur naar de effecten van bovenstaande factoren in combinatie met timing en techniek van palatumsluiting.

 

Er zijn geen studies bekend die aantonen dat het vroeg sluiten van het palatum durum leidt tot een betere uitgroei van het gelaat op volwassen leeftijd dan een latere sluiting van het palatum durum. Er wordt daarentegen in enkele studies wel geconcludeerd dat de groei van de bovenkaak verstoord wordt door vroege sluiting. Inhaalgroei van de bovenkaak wordt niet beschreven en wordt dan ook niet verwacht. Er zijn ook diverse studies die concluderen dat vroege sluiting van het palatum durum geen groeistoornissen geeft. De follow-up tijd voor beide behandelprincipes is echter vaak kort en in geval van een vroegtijdige sluiting is het niet uit te sluiten dat een groeivertraging ook later kan optreden, met name tijdens de groeispurt in de puberteit. De werkgroep is dan ook van mening dat het palatum durum pas later gesloten moet worden als optimale groei van de maxilla wordt nagestreefd.

 

Er wordt in enkele studies geconcludeerd dat het laat sluiten van het palatum durum spraakproblemen geeft. Andere studies concluderen dat het later sluiten van het palatum durum (uiteindelijk) geen spraakproblemen geeft. Er zijn geen studies bekend, die aantonen dat het laat sluiten van het palatum durum een betere spraak geeft dan het sluiten in het eerste levensjaar. Er zijn geen vergelijkende studies verricht naar de spraakontwikkeling op een latere leeftijd waarbij zowel de patiënten met een laat gesloten palatum durum vergeleken worden met kinderen waarbij het palatum durum vroeg gesloten is. Hierdoor is het niet te onderzoeken of een inhaalslag wordt gemaakt door kinderen met een laat gesloten palatum durum. De werkgroep is van mening dat het palatum durum net als het palatum molle vroegtijdig gesloten moet worden als optimale spraakontwikkeling wordt nagestreefd. Sluiting voor 12 maanden past bij een vroege behandeling met als doel optimale spraakontwikkeling .

 

Gezien het gebrek aan wetenschappelijke bewijs kan er ook logischerwijs geen behandelprotocol bestaan dat zowel voor de groei van de bovenkaak als voor de spraak optimaal is. De werkgroep is dan ook van mening dat dit dilemma met ouders besproken dient te worden. Wel adviseert de werkgroep om een voorkeursaanpak binnen het schisisteam te definiëren (standaard zorgpad/behandelprotocol).

Wij vonden geen bewijs voor een optimaal tijdstip voor sluiting van de lip. De werkgroep adviseert een operatieve sluiting van de lip in de eerste 6 maanden van het leven.

 

Timing in Nederland:

Op de website van de NVSCA waren medio 2015 de schisisprocollen van de 13 Schisisteams in Nederland samengevat:

Lipsluiting:

  • enkelzijdige schisis: 12 van de 13 teams sluiten de lip op ongeveer drie maanden, één team sluit de lip op zes maanden;
  • dubbelzijdige schisis: in ieder geval beschrijven twee teams een ander protocol ten opzichte van de enkelzijdige schisis, waarbij bij een sterke tilt van de premaxilla op drie maanden een lipadhaesie wordt uitgevoerd, waarna op negen maanden de lip definitief gesloten wordt.

Conclusie: in Nederland sluiten alle teams de lip in het eerste levensjaar, waarbij de grote meerderheid de sluiting rond de drie maanden uitvoert.

 

Gehemeltesluiting: palatum molle

  • enkelzijdige schisis: 11 van de 13 teams sluiten het palatum molle rond de leeftijd van negen maanden, waarvan één team sluit op de leeftijd tussen zes en 12 maanden; één team sluit het palatum molle op de leeftijd van zes maanden en één team op de leeftijd van drie maanden;
  • dubbelzijdige schisis: zelfde protocol;
  • geïsoleerde palatoschisis: zelfde protocol.

Conclusie: In Nederland sluiten alle teams het palatum molle in het eerste levensjaar; de grote meerderheid sluit rond de negen maanden.

 

Gehemeltesluiting: palatum durum

  • enkelzijdige schisis: er is een grote variatie met drie maanden en 12 jaar als uitersten, waarbij vrijwel elk team een eigen protocol heeft; één team sluit het palatum durum volledig op drie maanden, twee teams verrichten een vomerlap op drie maanden gevolgd door sluiting van de orale laag op negen tot 24 maanden; één team sluit op zes tot 12 maanden, drie teams op negen maanden, één team op anderhalf jaar, twee teams op drie jaar, één team op drie tot zeven jaar, één team op zeven tot negen jaar en één team op negen tot 12 jaar;
  • dubbelzijdige schisis: overeenkomstig protocol als enkelzijdige schisis, alleen twee teams verrichten reeds een enkelzijdige vomerlap op drie maanden;
  • geïsoleerde gehemeltespleet: acht teams hanteren hetzelfde protocol; één team sluit op negen maanden in plaats van negen tot 12 jaar bij complete schisis, één team op zes tot 12 maanden in plaats van zes maanden bij complete schisis, één team sluit bij een geisoleerde gehemeltespleet op 12 jaar in plaats van drie tot zeven jaar en twee teams sluiten op negen tot 24 maanden zonder vomerlappen op drie maanden.

Conclusie: Er is een grote variatie in het moment van sluiting van het palatum durum (met drie maanden en 12 jaar als uitersten), met name tussen de teams, maar ook tussen de typen schisis.

 

Combinatie behandelingen:

  • enkelzijdige schisis: drie teams combineren een lipsluiting op drie maanden met een (partiële) palatum durum sluiting (vomer lap), één team combineert een lipsluiting op zes maanden met een palatum molle sluiting; drie teams combineren altijd sluiting van het harde en palatum molle op negen maanden, twee teams combineren deze ingreep alleen op indicatie op negen maanden (anders uitstellen durumsluiting tot maximaal 24 maanden);
  • dubbelzijdige schisis: hetzelfde als bij de enkelzijdige schisis, waarbij bij twee teams in geval van de definitieve lipsluiting na lipadhaesie de uiteindelijke lipsluiting combineren met de palatumsluiting;
  • geïsoleerde gehemeltespleet: één team combineert sluiting van het zachte en palatum durum op negen maanden (vier totaal).

Conclusie: tien van de 13 teams voert op enig tijdsstip binnen het eigen behandeltraject een combinatie van ingrepen uit van lip, zachte of palatum durum.

 

De literatuur laat zien dat de bewijslast voor veel onderdelen van de schisisbehandeling over het algemeen mager is. Er zijn verschillende filosofieën over diagnostiek en behandeling, maar overtuigend bewijs voor een bepaalde strategie ontbreekt. Het gebrek aan consensus is het meest duidelijk bij de palatum durum sluiting. Enige bescheidenheid is dus op zijn plaats in het verdedigen van een protocol. Daarin past niet dat een protocol van een ander team als onvolwaardig bestempeld wordt zonder dat hier een hoge graad van wetenschappelijk bewijs tegenover staat. Wel moet een schisisteam aan patiënten en ouders duidelijk maken waarom voor een bepaald protocol gekozen is. Natuurlijk mag daarin benoemd worden om welke redenen afgezien wordt van een ander protocol, als maar duidelijk gemaakt wordt dat elk protocol zijn voor- en nadelen kent en dat het ideale protocol helaas (nog) niet bestaat. Met eerlijkheid en bescheidenheid, gedrevenheid en samenwerking en het delen van resultaten, bieden de schisisteams aan patiënten en ouders de beste basis voor optimale zorg.

Autorisatiedatum en geldigheid

Laatst beoordeeld : 01-09-2018

Laatst geautoriseerd : 01-09-2018

De richtlijnwerkgroep heeft als doel de richtlijn periodiek (digitaal) van updates te voorzien.

 

De NVPC is als houder van deze richtlijn de eerstverantwoordelijke voor de actualiteit van deze richtlijn. De andere aan deze richtlijn deelnemende wetenschappelijke verenigingen of gebruikers van de richtlijn delen de verantwoordelijkheid en informeren de eerstverantwoordelijke over relevante ontwikkelingen binnen hun vakgebied.

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 Plastische Chirurgie
  • Vereniging Klinische Genetica Nederland
  • Nederlandse Vereniging voor Logopedie en Foniatrie
  • Nederlandse Vereniging voor Mond- Kaak- en Aangezichtschirurgie
  • Nederlandse Verenging voor Schisis en Cranio Faciale Afwijkingen
  • Nederlandse Vereniging van Orthodontisten

Algemene gegevens

In samenwerking met:

Nederlandse Vereniging voor Keel-Neus-Oorheelkunde en Heelkunde van het Hoofd-Halsgebied (NVKNO)

Nederlandse Vereniging van Orthodontisten (NVvO)

Nederlandse Vereniging voor Mondziekten, Kaak- en Aangezichtschirurgie (NVMKA)

Nederlandse Vereniging voor Tandheelkunde (NVT)

Vereniging Klinische Genetica Nederland (VKGN)

Nederlands Instituut van Psychologen (NIP)

Nederlandse Vereniging voor Logopedie en Foniatrie (NVLF)

Nederlandse Vereniging voor Schisis en Cranio-Faciale Afwijkingen (NVSCA)

Nederlandse Vereniging van Pedagogen en Onderwijskundigen (NVO)

 

Met ondersteuning van:

Kennisinstituut van de Federatie Medisch Specialisten

 

Financiering

De richtlijnontwikkeling werd gefinancierd uit de Kwaliteitsgelden Medisch Specialisten (SKMS) met cofinanciering van de NVKNO, NVvO, NvT en NVMKA.

Doel en doelgroep

Doel

Doel van de richtlijn is het optimaliseren van de zorg voor patiënten met een schisis in Nederland, voor zover mogelijk onderbouwd met wetenschappelijke kennis uit onderzoek. Onder optimaliseren wordt ook verstaan het inzichtelijk maken van de oorzaak van de bestaande praktijkvariatie tussen de verschillende schisisteams en hierbij onderscheid te maken tussen gewenste en ongewenste praktijkvariatie in de behandeling van patiënten met een schisis. Dit resulteert in voorstellen voor een meer uniforme behandeling voor zover dit wetenschappelijk onderbouwd kan worden.

 

Specifieke aandacht zal worden gegeven aan de volgende onderwerpen:

    1. terugdringen van ongewenste/ongefundeerde praktijkvariatie in de werkwijze en behandelprotocollen van de Nederlandse schisisteams, zonder daarbij maatwerk, innovatie of onderzoek in de weg te staan;
    2. het beschikbaar stellen en toegankelijk maken van objectieve / evidence based informatie over de behandeling van schisis aan zorgverleners, patiënten, ouders en andere partijen;
    3. te bezien in hoeverre de bestaande organisatie van zorg aanpassing behoeft om te kunnen voldoen aan de eisen ten aanzien van “state of the art” behandeling van een kind of volwassene met een schisis en de controle daarop.

 

De richtlijn biedt op deze wijze een handvat voor een meer uniforme zorg op het gebied van de postnatale behandeling van een kind met een schisis en de implementatie van deze zorg in Nederland.

 

Doelgroep

De richtlijn is primair bedoeld voor alle zorgverleners die betrokken zijn bij de zorg van een kind met een schisis: huisartsen, verloskundigen, gynaecologen, kinderartsen, keel-, neus- en oorartsen, plastische chirurgen, mond-, kaak- en aangezichtschirurgen, orthodontisten, klinisch genetici, gespecialiseerde verpleegkundigen, logopedisten, (kinder)tandartsen, medisch psychologen, orthopedagogen en maatschappelijk werkers. De secundaire doelgroep betreft de ouders en hun omgeving.

Samenstelling werkgroep

Voor het ontwikkelen van de richtlijn is in 2013 een multidisciplinair samengestelde werkgroep ingesteld, bestaande uit vertegenwoordigers van alle relevante specialismen die betrokken zijn bij de zorg voor patiënten met een schisis en de NVSCA (zie hiervoor de samenstelling van de werkgroep). De werkgroepleden zijn door hun wetenschappelijke verenigingen en de NVSCA gemandateerd voor deelname. De werkgroep werkte gedurende twee jaar aan de totstandkoming van de richtlijn. De werkgroep is verantwoordelijk voor de integrale tekst van deze richtlijn.

 

Samenstelling van de werkgroep:

  • dr. A.B. Mink van der Molen (voorzitter), plastisch chirurg, Universitair Medisch Centrum Utrecht
  • dr. R.J.C. Admiraal, KNO-arts, Radboudumc Nijmegen
  • dr. L.N.A. van Adrichem, plastisch chirurg, Erasmus MC Rotterdam
  • drs. F.Bierenbroodspot, Mond-, Kaak- en Aangezichtschirurg, Isala, Zwolle
  • drs. D. Bitterman, tandarts, Universitair Medisch Centrum Utrecht
  • dr. M.J.H. van den Boogaard, Klinisch Geneticus, Universitair Medisch Centrum Utrecht
  • drs. J.M. Dijkstra-Putkamer MPA, logopedist, Medisch Centrum Leeuwarden
  • dr. M.C.M. van Gemert-Schriks, tandarts-pedodontoloog, Academisch Centrum Tandheelkunde Amsterdam
  • Prof.dr. A.M. Kuijpers-Jagtman, orthodontist, Radboudumc Nijmegen
  • dr.C.M.Moues-Vink, plastisch chirurg, Medisch Centrum Leeuwarden Friesland
  • dr. H.F.N. Swanenburg de Veye, gezondheidszorgpsycholoog, Universitair Medisch Centrum Utrecht/Wilhelmina Kinderziekenhuis
  • drs. N. van Tol – Verbeek, orthopedagoog-generalist; Koninklijke Kentalis Deventer / Almelo
  • dr. C. Vermeij – Keers, registratieleider, Nederlandse Vereniging voor Schisis en Craniofaciale Afwijkingen
  • drs. H. de Wilde, logopedist, Universitair Medisch Centrum Utrecht

 

Met ondersteuning van:

  • P.H. Broos MSc, senior adviseur, Kennisinstituut van Medisch Specialisten
  • dr. I.M. Mostovaya, adviseur, Kennisinstituut van Medisch Specialisten

 

Met dank aan:

  • drs. N.G. Janssen, Mond-, kaak- en aangezichtschirurg, Universitair Medisch Centrum Utrecht

Belangenverklaringen

De KNMG-Code ter voorkoming van oneigenlijke beïnvloeding door belangenverstrengeling is gevolgd. Alle werkgroepleden hebben schriftelijk verklaard of zij in de laatste drie jaar directe financiële belangen (betrekking bij een commercieel bedrijf, persoonlijke financiële belangen, onderzoeksfinanciering) of indirecte belangen (persoonlijke relaties, reputatie management, kennisvalorisatie) hebben gehad. Op de richtlijnendatabase in de richtlijn behandeling van patiënten met een schisis vindt u een overzicht van de belangen van alle werkgroepleden en het oordeel over het omgaan met eventuele belangen.

 

In 2017/2018 hebben we de belangen opnieuw uitgevraagd van de mensen die meegewerkt hebben aan de aanvullende modules: (1) Le Fort I vs. distractie-osteogense en (2) NAM. Een overzicht van de belangen van deze werkgroepleden is zichtbaar in onderstaande tabel.  

 

De ondertekende belangenverklaringen zijn op te vragen bij het secretariaat van het Kennisinstituut van de Federatie Medisch Specialisten.

 

Werkgroeplid

Functie

Nevenfuncties

Persoonlijke financiële belangen

Persoonlijke relaties

Reputatiemanagement

Extern gefinancierd onderzoek

Kennisvalorisatie

Overige belangen

Admiraal

Arts

geen

nee

nee

nee

nee

nee

nee

Bierenbroodspot

lid werkgroep vanuit de
NVMKA

nee

nee

nee

nee

nee

nee

nee

Bitterman

kinderarts in WKZ Utrecht. Op dit moment hoofd poli kindertandheelkunde van WKZ Utrecht

werkzaam als tandarts in algemene praktijk

nee

nee

nee

nee

nee

nee

De Wilde

logopedist WKZ Utrecht

Post HBO cursus Schisis

nee

nee

nee

nee

nee

nee

Dijkstra-Putkamer

logopedist schisisteam (8 uur p/w)
physician assistant KNO (32 uur p/w)

voorzitter werkgroep
logopedisten schisisteams
Nederland, onbetaald. Bestuurslid stichting Schisis Friesland, onbetaald. Bestuurslid NVSCA, onbetaald

nee

nee

nee

nee

nee

nee

Kuipers-Jagtman

Hoogleraar orthodontie; omvang 1,0 fte tot 01-03-2014; daarna 0,6 fte

Editor-in-Chief Orthodontics and Craniofacial Research, hoofdredacteur scientific impact-factor journal op het gebied van orthodontisch en craniofaciaal onderzoek; 0,2 fte, betaald
Deputy Professor, Universitas Indonesia, Jakarta (Indonesia), onderwijs en onderzoek op het gebied van orthodontie en schisis; onbetaald
Member Cochrane Oral Health Expert Group, beoordeling en priorering van onderwerpen voor Cochrane Systematic Reviews, beoordeling van Chochrane protocollen op het gebied van Oral Health, waaronder ook schisis valt; ontbetaald
Councillor World Federation of Orthodfontics, professional world organisation to advance the art and science of orthodontics throughout the world; onbetaald
Voorzitter Stichting Sumbing Bibir voor behandeling van en onderzoek naar schisis in Indonesia; onbetaald

nee

nee

Ik was tot 01-01-2014 hoofd van het Centrum voor Schisis en Craniofaciale Afwijkingen van het Radboudumc.

nee

nee

nee

Mink van der Molen

50% UMCU, 50% St.
Antonius Ziekenhuis, Nieuwegein

nee

nee

nee

nee

nee

nee

nee

Moues

Werkgroep lid
Plastisch Chirurg te Leeuwarden

nee

nee

nee

nee

nee

nee

nee

Swanenburg de Veye

Gezondheidszorgpsycholoog

geen

nee

nee

nee

nee

nee

nee

Van Adrichem

Plastisch Chirurg, Erasmus MC Rotterdam (Sophia Kinderziekenhuis), sectorhoofd
Plastisch Chirurg, Velthuiskliniek (via Maatschap plastische chirurgie AZR/SFG)
Opleider Plastische Chirurgie Erasmus MC / SFG; plv afdelingshoofd Erasmus MC plch

Adviseur Medirisk/VVAA (betaald via Maatschap)
Vice-voorzitter kinderchirurgische groep (onbetaald, SKZ)
Secretaris concilium plastico chirurgicum (onbetaald, nvpc)
BBC NVPC adviseur op afroep (onbetaald)
Klankbordgroep Nieuwbouw Erasmus MC (onbetaald)
Projectgroep structuur thema Dijkzigt (onbetaald)
Voorzitter schisisteam Erasmus MC (onbetaald)
Voorzitter landelijke "Kenniscentra Schedelvormafwijkingen"

nee

nee

nee

nee

nee

nee

Van den Boogaard

klinisch geneticus

nee

nee

nee

nee

nee

nee

nee

Van Gemert – Schriks

Tandarts-pedodontoloog
- Coördinator post-initiële opleiding kindertandheelkunde (ACTA)
- Tandarts pedodontoloog CBT Rijnmond

 

geen

geen

geen

nvt

nvt

nee

Van Tol-Verbeek

behandelcoördinator Kentalis
vroeg-behandeling - 0,8 FTE

lid netwerk LZMG
en van de NVO (onbetaald)

nee

nee

nee

nee

nee

nee

Vermeij – Keers

registratieleider NVSCA schisis
en craniofaciale afwijkingen (onbetaald)

wetenschappelijk onderzoeker
Erasmus MC; gepensioneerd
met gastvrijheidsovereenkomst
(onbetaald)

nee

nee

nee

nee

nee

nee

Inbreng patiëntenperspectief

Er werd aandacht besteed aan het patiëntenperspectief door middel van een focusgroepbijeenkomst. Een geanonimiseerd verslag is als bijlage bij de richtlijn gevoegd. De resultaten van de focusgroepbijeenkomst zijn waar mogelijk verwerkt in de richtlijn. Daarnaast heeft de BOSK de conceptrichtlijn beoordeeld tijdens de commentaarfase en suggesties voor verbetering van de richtlijn gegeven.

Methode ontwikkeling

Evidence based

Implementatie

In de verschillende fasen van de richtlijnontwikkeling is rekening gehouden met de implementatie van de richtlijn en de praktische uitvoerbaarheid van de aanbevelingen. Daarbij is uitdrukkelijk gelet op factoren die de invoering van de richtlijn in de praktijk kunnen bevorderen of belemmeren.

 

De richtlijn wordt opgenomen in de richtlijnendatabase (www.richtlijnendatabase.nl) waarmee hij toegankelijk is voor alle relevante beroepsgroepen en patiënten. Daarnaast wordt er een samenvatting van de richtlijn gepubliceerd in tijdschriften van de deelnemende wetenschappelijke verenigingen. Ook is de richtlijn te downloaden vanaf de website van de Kwaliteitskoepel: www.kwaliteitskoepel.nl en websites van betrokken wetenschappelijke verenigingen en de NVSCA.

 

De werkgroep heeft echter besloten geen nieuwe indicatoren te ontwikkelen bij de huidige richtlijn. De werkgroep beveelt de betrokken wetenschappelijke verenigingen aan om pas over een aantal jaren uitkomstindicatoren te ontwikkelen als de kwaliteitsregistratie van de NVSCA en PROMS ─ volgens bijvoorbeeld ICHOM ─ volledig operationeel zijn. Om dit te bereiken dienen de schisisteams eerst de tijd te krijgen toe te werken naar een uniforme werkwijze zoals beschreven in module Organisatie van zorg en het implementeren van PROMS.

Werkwijze

AGREE II

Deze richtlijn is opgesteld conform de eisen in het rapport ‘Richtlijnen 2.0’ van de adviescommissie Richtlijnen van de Raad kwaliteit van de Orde van Medisch Specialisten. Dit rapport is gebaseerd op het AGREE II instrument (Appraisal of Guidelines for Research & Evaluation II) (www.agreetrust.org), dat een internationaal breed geaccepteerd instrument is voor de beoordeling van de kwaliteit van richtlijnen.

 

Knelpuntenanalyse

Tijdens de voorbereidende fase inventariseerden de voorzitter van de werkgroep en de adviseur de knelpunten. Tevens zijn in een invitational conference knelpunten besproken waarbij, naast vertegenwoordigers van de partijen in de werkgroep, vertegenwoordigers van de Nederlands Huisartsen Genootschap (NHG), de Bosk en Achmea aanwezig waren. Een verslag hiervan kunt u vinden in de bijlage bij de richtlijn.

 

Uitgangsvragen en uitkomstmaten

Op basis van de uitkomsten van de knelpuntenanalyse zijn door de voorzitter en de adviseur concept-uitgangsvragen opgesteld. Deze zijn vervolgens verder uitgewerkt door de leden van de werkgroep en tijdens de werkgroepvergadering vastgesteld. Vervolgens inventariseerde de werkgroep per uitgangsvraag welke uitkomstmaten voor de patiënt relevant zijn, waarbij zowel naar gewenste als ongewenste effecten werd gekeken. De werkgroep waardeerde deze uitkomstmaten volgens hun relatieve belang als kritiek, belangrijk en onbelangrijk. Tevens definieerde de werkgroep, voor zover mogelijk, wat zij voor een bepaalde uitkomstmaat een klinisch relevant verschil vond, dat wil zeggen wanneer de verbetering in uitkomst een verbetering voor de patiënt is.

 

Strategie voor zoeken en selecteren van literatuur

Er werd eerst oriënterend gezocht naar bestaande buitenlandse richtlijnen en naar systematische reviews in Medline (OVID) en Cochrane Library. Vervolgens werd voor de afzonderlijke uitgangsvragen aan de hand van specifieke zoektermen gezocht naar gepubliceerde wetenschappelijke studies in (verschillende) elektronische databases. Tevens werd aanvullend gezocht naar studies aan de hand van de literatuurlijsten van de geselecteerde artikelen. In eerste instantie werd gezocht naar studies met de hoogste mate van bewijs. De werkgroepleden selecteerden de via de zoekactie gevonden artikelen op basis van vooraf opgestelde selectiecriteria. De geselecteerde artikelen werden gebruikt om de uitgangsvraag te beantwoorden. De databases waarin is gezocht, de zoekactie of gebruikte trefwoorden van de zoekactie en de gehanteerde selectiecriteria zijn te vinden in de module van de desbetreffende uitgangsvraag. De gedetailleerde zoekstrategieën zijn vermeld in een bijlage bij de richtijn.

 

Kwaliteitsbeoordeling individuele studies

Individuele studies werden systematisch beoordeeld, op basis van op voorhand opgestelde methodologische kwaliteitscriteria, om zo het risico op vertekende studieresultaten (bias) te kunnen inschatten. Deze beoordelingen kunt u vinden in de kolom ‘Beoordeling kwaliteit studie’ van een evidencetabel.

 

Samenvatten van de literatuur

De relevante onderzoeksgegevens van alle geselecteerde artikelen werden overzichtelijk weergegeven in evidencetabellen. De belangrijkste bevindingen uit de literatuur werden beschreven in de samenvatting van de literatuur.

 

Beoordelen van de kracht van het wetenschappelijke bewijs

A) Voor interventievragen

De kracht van het wetenschappelijke bewijs werd bepaald volgens de GRADE-methode. GRADE staat voor ‘Grading Recommendations Assessment, Development and Evaluation’ (zie http://www.gradeworkinggroup.org/handbook).

 

B) Voor vragen over de waarde van diagnostische tests, schade of bijwerkingen, etiologie en prognose

Bij dit type vraagstelling kan GRADE (nog) niet gebruikt worden. De bewijskracht van de conclusie is bepaald volgens de gebruikelijke EBRO-methode (Van Everdingen, 2004).

 

GRADE 

Definitie

Hoog

­      het is zeer onwaarschijnlijk dat de literatuurconclusie verandert als er verder onderzoek wordt gedaan;

­      er is veel vertrouwen dat het ware effect van behandeling dichtbij het geschatte effect van behandeling ligt zoals vermeld in de literatuurconclusie;

Matig

­      het is mogelijk dat de conclusie verandert als er verder onderzoek wordt gedaan;

­      er is matig vertrouwen dat het ware effect van behandeling dichtbij het geschatte effect van behandeling ligt zoals vermeld in de literatuurconclusie.

Laag

­      het is waarschijnlijk dat de conclusie verandert als er verder onderzoek wordt gedaan;

­      er is beperkt vertrouwen dat het ware effect van behandeling dichtbij het geschatte effect van behandeling ligt zoals vermeld in de literatuurconclusie.

Zeer laag

­      de conclusie is zeer onzeker;

­      er is weinig vertrouwen dat het ware effect van behandeling dichtbij het geschatte effect van behandeling ligt zoals vermeld in de literatuurconclusie.

 

Formuleren van de conclusies

Bij interventievragen verwijst de conclusie niet naar een of meer artikelen, maar wordt getrokken op basis van alle studies samen (body of evidence). Hierbij maakten de werkgroepleden de balans op voor elke interventie. Bij het opmaken van de balans werden door de werkgroep de gunstige en ongunstige effecten voor de patiënt afgewogen.

 

Voor vragen over de waarde van diagnostische tests, schade of bijwerkingen, etiologie en prognose is het wetenschappelijke bewijs samengevat in een of meerdere conclusie(s), waarbij het niveau van het meest relevante bewijs is weergegeven. Omwille van de homogeniteit is het niveau van bewijs voor alle conclusies weergegeven als hoog/matig/laag/zeer laag, waarbij EBRO niveau 1 is vertaald naar hoog, 2 naar matig, 3 naar laag en 4 naar zeer laag.

 

Wanneer er voor een uitgangsvraag geen systematisch literatuuronderzoek werd verricht, is de literatuur niet beoordeeld op kwaliteit en is de kracht van het bewijs niet bepaald.

 

Overwegingen

Voor een aanbeveling zijn naast het wetenschappelijke bewijs ook andere aspecten belangrijk, zoals de expertise van de werkgroepleden, patiëntenvoorkeuren, kosten, beschikbaarheid van voorzieningen of organisatorische zaken. Deze aspecten worden, voor zover niet wetenschappelijk onderzocht, vermeld onder het kopje ‘Overwegingen’.

 

Formuleren van aanbevelingen

De aanbevelingen geven een antwoord op de uitgangsvraag en zijn gebaseerd op het beschikbare wetenschappelijke bewijs en de belangrijkste overwegingen. Bij het formuleren en graderen van de aanbeveling worden minimaal de volgende vier factoren in ogenschouw genomen: algehele kwaliteit van het wetenschappelijke bewijs; balans tussen voor- en nadelen van de interventie; waarden en voorkeuren van professional en patiënten; en beschikbare middelen.

 

Kennislacunes

Tijdens de ontwikkeling van deze richtlijn is systematisch gezocht naar onderzoek waarvan de resultaten bijdragen aan een antwoord op de uitgangsvragen. Bij elke uitgangsvraag is door de werkgroep nagegaan of er (aanvullend) wetenschappelijk onderzoek gewenst is.

 

Commentaar- en autorisatiefase

De conceptrichtlijn werd aan de betrokken (wetenschappelijke) verenigingen voorgelegd voor commentaar. De commentaren werden verzameld en besproken met de werkgroep. Naar aanleiding van de commentaren werd de conceptrichtlijn aangepast en definitief vastgesteld door de werkgroep. De definitieve richtlijn werd aan de betrokken (wetenschappelijke) verenigingen voorgelegd voor autorisatie en door hen geautoriseerd.

Zoekverantwoording

Zoekacties zijn opvraagbaar. Neem hiervoor contact op met de Richtlijnendatabase.