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Journal of Plastic Surgery and Hand Surgery

ISSN: 2000-656X (Print) 2000-6764 (Online) Journal homepage: http://www.tandfonline.com/loi/iphs20

Isolated cleft palate requires different surgical


protocols depending on cleft type

Anna Elander, Christina Persson, Jan Lilja & Hans Mark

To cite this article: Anna Elander, Christina Persson, Jan Lilja & Hans Mark (2017) Isolated cleft
palate requires different surgical protocols depending on cleft type, Journal of Plastic Surgery and
Hand Surgery, 51:4, 228-234, DOI: 10.1080/2000656X.2016.1235579

To link to this article: https://doi.org/10.1080/2000656X.2016.1235579

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Published online: 18 Oct 2016.

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JOURNAL OF PLASTIC SURGERY AND HAND SURGERY, 2017
VOL. 51, NO. 4, 228–234
http://dx.doi.org/10.1080/2000656X.2016.1235579

ORIGINAL ARTICLE

Isolated cleft palate requires different surgical protocols depending


on cleft type
Anna Elandera, Christina Perssonb, Jan Liljaa and Hans Marka
a
Institute of Clinical Sciences, Department of Plastic Surgery, Sahlgrenska Academy, University of Gothenburg, Sweden; bInstitute of
Neuroscience and Physiology, Division of Speech-Language Pathology, Sahlgrenska Academy, University of Gothenburg, Sweden

ABSTRACT ARTICLE HISTORY


A staged protocol for isolated cleft palate (CPO), comprising the early repair of the soft palate at 6 Received 22 September 2015
months and delayed repair of the eventual cleft in the hard palate until 4 years, designed to improve Revised 25 July 2016
maxillary growth, was introduced. CPO is frequently associated with additional congenital conditions. Accepted 18 August 2016
The study evaluates this surgical protocol for clefts in the soft palate (CPS) and for clefts in the hard Published online 29 Septem-
ber 2016
and soft palate (CPH), with or without additional malformation, regarding primary and secondary surgi-
cal interventions needed for cleft closure and for correction of velopharyngeal insufficiency until 10 KEYWORDS
years of age. Of 94 consecutive children with CPO, divided into four groups with (þ) or without () Isolated cleft palate;
additional malformations (CPS þ or CPS  and CPH þ or CPH), hard palate repair was required in 53%, malformation; soft and hard
performed with small local flaps in 21% and with bilateral mucoperiosteal flaps in 32%. The total palate
incidence of soft palate re-repair was 2% and the fistula repair of the hard palate was 5%. The total
incidence of secondary velopharyngeal surgery was 17% until 10 years, varying from 0% for CPS  and
15% for CPH, to 28% for CPS þ and 30% for CPHþ. The described staged protocol for repair of CPO
is found to be safe in terms of perioperative surgical results, with comparatively low need for second-
ary interventions. Furthermore, the study indicates that the presence of a cleft in the hard palate and/
or additional conditions have a negative impact on the development of the velopharyngeal function.

Introduction morphology, and only 10% of patients had to undergo


orthognathic surgery to correct a maxillary growth aberration
The isolated cleft palate (CPO) is usually described as a
judged to be untreatable with orthodontic treatment alone
homogenous group of clefts. There are various anatomical
[7]. Furthermore, the SNA angle describing maxillary prog-
variations in size and configuration, of course, such as
nathism was closer to normal (76.8) and significantly larger
whether the cleft is in the soft palate only (CPS) or both the
than when using Wardill-Kilner (72.9) [3] and von
hard and soft palates (CPH). Moreover the isolated cleft pal-
Langenbeck (74.2) [8], while the maxillary length was also
ate is frequently associated with other additional congenital
significantly longer than when using von Langenbeck
conditions (þ) [1]. Different surgical techniques can be used
to repair the cleft, most of them aiming for a one-stage (49.5 mm vs 46.0 mm) [8].
repair. To date there has been no consensus as to the best For CPO, the Wardill-Kilner technique showed a similar
surgical protocol. negative impact regarding constricting scars in the hard pal-
In Gothenburg, the Wardill-Kilner pushback technique was ate affecting the dental arch [4,5,9,10], which was why it was
long used to repair all kinds of palatal clefts, including CPO abandoned. Instead, a similar protocol was introduced for
[2]. This technique is a one-stage closure using a pushback CPO in 1987, including the same soft palate repair as used
procedure of bilateral mucoperiosteal flaps, leaving denuded for UCLP at 6 months. Any cleft in the hard palate was left
bone areas in the anterior and lateral parts of the hard palate for closure at 4 years. An evaluation of this protocol per-
for secondary healing, with scar formation that contracts the formed by Friede et al. [5] in 2000 to judge maxillary growth
upper dental arch, which has been shown to restrict the at 5 years of age was encouraging, as a significantly better
maxillary growth with negative impact on the facial profile occlusion was found in children with CPS in comparison to
and dental occlusion for both complete and CPO clefts [3–6]. when the Wardill-Kilner technique was used. From an ortho-
Aiming for improved maxillary growth, the Wardill-Kilner dontic perspective, these results support the introduction of
technique was, therefore, abandoned for complete unilateral this strategy for CPO. The children’s speech was also eval-
cleft lip and palate (UCLP) in 1975, in favour of a two-stage uated at age 5, which showed that 35% were rated to have
procedure for palatal closure. The new protocol included an an impaired velopharyngeal function (VPI) [11]. The sub-
early soft palate repair at 6–9 months, followed by a delayed group of children with CPS  performed best (0% VPI), fol-
hard palate closure at the age of mixed dentition (7–9 years). lowed by the sub-groups with CPH (31% VPI), CPS þ (44%
This protocol showed improved results regarding facial VPI), and CPH þ (77% VPI). These results indicate that speech

CONTACT Hans Mark hans.mark@vgregion.se Department of Plastic Surgery, Sahlgrenska University Hospital, Gothenberg, Sweden
ß 2016 Acta Chirurgica Scandinavica Society
JOURNAL OF PLASTIC SURGERY AND HAND SURGERY 229

outcomes not only depended on the surgical technique, but the cleft margins, at the junction between the oral and nasal
also on the presence of a cleft in the hard palate and mucosa to the tip of the uvula (Figure 1). Small bilateral oral
whether the cleft was in conjunction with a malformation. flaps were raised from the posterior part of the hard palate
However, further investigation of older children is needed. at each side of the cleft. At the border of the soft palate the
The primary aim of the present study is to describe and blunt and sharp dissection continued, dividing the oral
evaluate a surgical protocol for soft palate repair and hard mucosa from the muscles, which were kept attached to the
palate closure for different types of CPO, with or without nasal mucosa as one layer. The incision posterior to the max-
additional conditions, until 10 years of age. illary tuberosities was widened by blunt dissection and the
hamulus was identified, but not broken. The oral flaps were
Patients and methods mobilised posteriorly and medially until they could reach the
midline without tension. The tensor insertions and the nasal
Subjects mucosa were dissected free from the posterior part of the
Between 1987–1993, 105 consecutive children with CPO are palatal shelf and released by a cut through the nasal mucosa
known to have been treated using this protocol at the going lateral then posterior, enabling the levator muscles
and the nasal layer to be rotated posteriorly and medially
Department of Plastic Surgery, Sahlgrenska University
towards the midline, to be reconstructed about 1 cm poster-
Hospital, in Gothenburg, Sweden, of whom 94 children
ior to the hard palate, leaving an anterior defect in the nasal
(57 girls and 37 boys) were retrospectively followed until
layer. Both the nasal layer, including the levator muscles, and
10 years of age. (Of the 11 children excluded from the study,
the oral layer were sutured in the midline with mattress
five had moved away from the area, five had passed away,
sutures using 4–0 Dexon or Vicryl and the uvula with 5–0.
and one had been operated on in the early period using the
For CPS, the anterior tips of the oral flaps were not pulled
Wardill-Kilner technique at the behest of the surgeon.) Of
forward, attempting to cover the exposed bone of the palatal
those 94 children, 42 (45%) had a CPS and 52 (55%) had a
CPH, where CPH was defined as all children needing repair
to the hard palate in a separate procedure. No submucous
clefts were included. In 40% of the children (38/94), the pres-
ence of other malformations could be identified for both CPS
(17/42) and CPH (21/52). The definition of malformations was
the presence of any kind of additional malformation affecting
the heart, limbs, or urogenital region or neuropsychiatric
problem, or if the cleft was part of a syndrome [12].

Surgical protocol
The soft palate repair was performed at 6 months of age,
leaving the eventual cleft in the hard palate open as a
residual cleft to be repaired in a secondary procedure at
4 years of age. General anaesthesia was used in combination
with infiltration with Xylocaine 0.25% with adrenaline 1:200
000 of surgical area. All patients had pre-operative antibiotics
with cloxacillin during both procedures. There was a total of
five surgeons performing soft palate repairs in the period in
question, all of whom had different experience of the
method—indeed, two surgeons operated on only two cases
each. During the early period all surgeons were allowed to
operate, whereas during the last period only two surgeons
performed the operations. Meanwhile, the number of sur-
geons performing the residual cleft closure was two. All chil-
dren had splints and were prevented from moving freely; the
food regime was a full liquid diet for the first 5 days, then a
soft diet for the subsequent 5 days.

Figure 1. Schematic illustration of soft palate closure in a cleft in the Soft pal-
Repair of the soft palate ate only. (a) Cleft in the soft palate. (b) Incision line posterior to the tuber of
the alveolar ridge, continues in medial-anterior direction. The incision continues
In repairing the soft palate, the incision started posterior to medial-posterior behind the major palatine vessel and turns to a medial anterior
the maxillary tuberosities, continued medially on the poster- direction until it meets the incision at the cleft border. (c) Oral mucosal flaps
ior part of the hard palate shelves, going posterior to the pal- raised and muscular insertions to the hard palate are cut, the muscles are
moved posteriorly near the Eustachian tube. (d) Muscles are sutured in a poster-
atine vessel to the lateral border of the cleft on the palatal ior position. (e) Oral layer closed covering muscles leaving raw bone surface
shelf. When reaching the cleft, the incision continued along anteriorly.
230 A. ELANDER ET AL.

shelves, but were fixed by some sutures to the posterior shelves and the V-shaped flaps were turned over into the
edges of the hard palate, achieving a pushback of 1 cm defect, facilitating the suturing and closure of the nasal layer.
(Figure 1). For CPH, the two-layer repair of the soft palate By one or two sutures brought through the nose, the nasal
bridged over the cleft from the sides without any anchorage layer could be mobilised by traction into the nasal cavity. For
in the anterior direction (Figure 2); however, a posteriorly closure of the oral layer, two different principles were used
based vomer flap was raised and turned over and sutured depending on the size of the cleft. For small clefts, the oral
between the nasal flaps if possible, which anchored the soft layer was closed with two bipedicle flaps raised from the
palate to the vomer (Figure 3). medial half of the palatal shelf and brought over the defect
and sutured; the residual cleft was, thus, closed in two layers
(Figure 4). Wider clefts required bilateral mucoperiosteal
Repair of the residual cleft flaps. Since children at the age of 4 have developed molars,
mucoperiosteal flaps were raised after dento-gingival mar-
The nasal layer was closed using the same technique for
ginal incisions from behind the tuber area anterior to the
both small and large clefts. An incision was made along the
region of the deciduous lateral incisor, where the incisions
border of the residual cleft. At the anterior and posterior went medially to the anterior part of the circumferential inci-
ends of the cleft V-shaped flaps were raised from the oral sion around the residual cleft in the palate. The flaps were
side. The nasal mucosa was dissected free from the palatal sutured with mattress sutures in the midline and could in
most instances be repositioned back into their original pos-
ition and sutured along the neck of the teeth without leaving
denuded bone for secondary healing. The closure of the cleft
defect was, thus, achieved by a hanging bridge principle
(Figure 5).

Secondary pharyngeal surgery


The indications to perform surgery were based on perceived
problems with speech according to the family and velophar-
yngeal insufficiency, perceptually assessed by an experienced
speech-language pathologist and verified by videofluoro-
scopy or nasendoscopy. Surgical correction for velopharyng-
eal insufficiency was performed using a velopharyngeal flap
in all cases but one (in that patient a secondary intravelar
veloplasty was performed instead. All velopharyngeal flaps
were superiorly based and sutured into the soft palate either
in the nasal layer or tucked in between the nasal and oral
layer through an incision along the posterior edge of the soft
palate cranially of the uvula.
Figure 2. Schematic illustration of soft palate closure in a cleft in the soft and
hard palate. (a). Cleft in the soft and hard palate. (b) Incision line posterior to
the tuber of the alveolar ridge. Then medial in a zigzag fashion behind the Statistics
major palatine vessel until it meets the incision at the cleft border. Oral mucosal
flaps are raised and muscular insertions to the hard palate are cut the muscles The mean ± SD and range are given for the different varia-
are moved posteriorly near Eustachian tube. (c) Muscles are sutured in a poster-
ior position. (d) Oral layer closed covering muscles leaving an opening anteriorly bles. Student’s t-test was used for comparison; p < .05 was
in the hard palate. considered significant.

Figure 3. Schematic illustration soft palate closure in a cleft in the soft and hard palate and a vomer flap. (a) Cleft in the soft and hard palate. Incision line posterior
to tuber of the alveolar ridge. Then medial in a zigzag fashion behind the major palatine vessel until it meets the incision at the cleft border. Oral mucosal flaps are
raised and muscular insertions to the hard palate are cut and muscles are moved posteriorly near the Eustachian tube. A posteriorly based vomer flap is raised and
introduced between the muscular bundles. (b) Muscles are sutured together and to the vomer flap in a posterior position. (c) Oral layer closed covering muscles
leaving an opening anteriorly and a raw bone surface on the vomer.
JOURNAL OF PLASTIC SURGERY AND HAND SURGERY 231

Figure 4. Schematic illustration of closure of a small residual cleft. (a) Small residual cleft in the hard palate. (b) Two bipedicular flaps are raised, nasal layer dis-
sected free and sutured. (c) Oral layer closed.

Figure 5. Schematic illustration of closure of a large residual cleft. (a) Large residual cleft in hard palate with incision lines made along the neck of the teeth in the
gingival pocket. No tissue is left near the teeth. Incision continues to the second incisor where it turns to a medial-posterior direction. It will meet the incision from
the opposite side close to the residual cleft. The incision continues around the residual cleft leaving flaps anterior and posterior. (b) The nasal layer is closed using
nasal mucosa dissected free and the anterior and posterior turnover flaps. (c) Oral layer closed. Note, minimal raw surfaces between the mucoperiosteal flaps and
the teeth.

Table 1. Data from soft palate repair and residual cleft closure. reoperation due to bleeding and no blood transfusions were
Soft palate repair Residual cleft closure given. No patient stayed for intensive care observation over-
(n ¼ 94) (n ¼ 50)
night. The hospital stay after residual cleft closure ranged
Mean age, months (min-max) 7.7 (4.5–33.5) 49 (24–83)
HSP 7.6 (4.5–27) from 3–9 days.
SPO 7.7 (5–33.5) The total incidence of fistulas needing repair was 5%. Of
Mean weight, kg 7.6 – the 50 patients operated on for residual cleft closure, postop-
HSP 7.4
SPO 7.7 erative fistulas were diagnosed in seven patients, of whom
Bleeding, ml; min-max 14 (5–70) 28 ± 27 five needed reoperation due to significant problems (10%),
Reoperation due to bleeding 4 patients – while in the other two patients the fistulas were without sig-
Postoperative intensive care 4 patients –
Hospital stay, days (min–max) 7.8 (4–11) 5 (3–9) nificance. Two of the fistulas needed repair after local flap
Postoperative dehiscience 2 partial separations 7 fistulas closure and three after closure by use of mucoperiosteal
Dehiscience needing reoperation 2 5
flaps, giving a similar rate for secondary surgery due to
impaired healing of 10% for both principles used for residual
cleft closure.
Results The total incidence of secondary velopharyngeal surgery
The demography and surgical data associated with the soft was 17% until age 10. The incidence varied according to two
and hard palate repair are given in Table 1. The mean sur- factors: the presence of a cleft in the hard palate or add-
gery time for soft palate repair was 71 minutes. In total, a itional conditions as described in Table 2. In the group with
vomer flap was raised and sutured into the nasal layer in 22 no additional conditions CPH had a significantly higher fre-
out of 94 children (24%), all CPH; four out of 94 children quency of secondary velopharyngeal surgery (15%) than SPO
needed intensive care postoperatively, one after reoperation (0%). Children with an additional condition had a significantly
due to bleeding, and three due to respiratory problems diag- higher incidence of secondary velopharyngeal surgery com-
nosed prior to surgery or during anaesthesia for surgery. The pared to the whole CPO group, and indeed to the CPS sub-
hospital stay after soft palate repair ranged from 4–11 days. group.
Partial postoperative separations of the soft palate occurred
in two children, which required reoperation (2%). No com-
Discussion
plete separations occurred.
In total, 53% needed repair of a residual cleft in the hard There are many techniques for cleft palate repair and the
palate, 21% by use of small local flaps and 32% by the use ultimate protocol is yet to be identified. For various reasons,
of bilateral mucoperiosteal flaps. No patients needed it is still the case that each surgeon advocates his or her
232 A. ELANDER ET AL.

Table 2. Velopharyngeal flap surgery performed until age 10 years in the cleft used. One possibility is that the size of the cleft is a factor
groups with malformations present or not. Level of statistical significance
[15], as also reported here. Marrinan et al. [15] have pub-
between HSP vs SPO and between no malformation vs malformation.
lished different incidences of pharyngeal flaps for CPS and
All iCP (n ¼ 94) HSP (n ¼ 52) SPO (n ¼ 42)
CPH, reporting 9% and 25%, respectively, when using von
All iCP (n ¼ 94) 16 (17%) 11 (21%) 5 (12%)
No malformation (n ¼ 56) 5 (9%) 5 (15%) 0 (0%) Langenbeck and Wardill-Kilner techniques. Nyberg et al. [16]
Malformation (n ¼ 38) 11 (29%)† 6 (30%) 5 (28%)† reported a higher number of velopharyngeal flaps at age 5
p < .05; among children with CPH (30%) compared with children with
†p < .05. CPS (3%). Andersson et al. [17] reported 10 years after pri-
mary surgery an overall incidence of pharyngeal flaps of
14%; and, the larger the cleft, the higher the incidence,
own. Many studies have been published, but to date the sci-
amounting to 42% for HSP. In this group, all children with
entific quality has been low due to the study design – includ-
additional malformations were excluded.
ing mixed groups with respect to cleft types, small numbers
One thing that speaks in favour of the present protocol is
of patients, and a mixture of clefts with or without additional
that it seems to result in a significantly better width of the
conditions – which, combined with different ages, different maxillary dental arch at the level of the first deciduous
timings of the surgical protocol, and the fact that almost all molars by 2.5 mm in CPS, as indicated by data on patients
reports are retrospective, makes valid scientific conclusions from the present cohort regarding palatal growth and dental
impossible. occlusion at 5 years, compared to when the Wardill-Kilner
In the present study, soft palate repair was timed to take technique was used [4,5]. There was, thus, less need for
place at 6 months, since early surgery was considered to orthodontic treatment. One possible explanation for the bet-
be beneficial for speech development, a hypothesis that now ter growth is less scarring of the hard palate as compared to
seems supported [13]. Earlier repair was tried at our unit, but when the hard and soft palate are moved posteriorly as one
at lower ages (3–4 months) and with other complicating con- unit, which has been reported by others too [9,10].
ditions, respiratory problems needing advanced postopera- Furthermore, when comparing children from this cohort at 5
tive care were more common. The mean age for soft palate years who had their cleft in the hard palate repaired with
repair in this study was, thus, 7.7 months, and from the peri- large mucoperiosteal flaps, to those who could be closed by
operative data it may be concluded to be safe. local flaps, the former had a significantly narrower maxillary
A positive aspect of the present protocol is that it concen- dental arch width by a mean value of 1.4 mm [5].
trates on the soft palate repair, facilitating muscle repair and Against a two-stage protocol for CPH is the fact that chil-
the posterior transition of the soft palate. Furthermore, the dren with CPH will need two surgical interventions – in the
two-stage protocol for CPH in this study enables an individu- present study, 48% of patients. However, in order to properly
alisation regarding the hard palate closure depending on the evaluate the two-stage procedure, the various outcomes – all
size and extent of the cleft. The reason for introducing a primary and secondary surgical interventions, need for ortho-
vomer flap if possible is that it helps to lengthen and close dontic treatment and speech therapy, and not least the final
the nasal layer, and anchors the soft palate to the vomer. speech result – must be analysed and compared to one-
Characteristic for the soft palate repair is that the muscles stage procedures. In the present study, we present the load
are kept undissected from the nasal mucosa, and the reposi- of surgical interventions until the age of 10. A drawback with
tioning of the muscles is achieved by dissecting and releas- a one-stage approach is that the same technique is used
ing the nasal layer from the posterior part of the hard palate, regardless of the presence of a cleft in the hard palate or not
enabling it to be closed in the midline by moving it posterior [2,10]; consequently, children with CPH were treated with
to the level of the Eustachian tube opening. The fact that unnecessary wide mucoperiosteal flaps and large undermin-
the closure is in one layer only in the anterior part was not ing, since it impacts negatively on the maxillary growth.
thought a problem, and this procedure might be beneficial Another one-stage technique for palatal closure in CPO
for the lengthening and function of the soft palate, indicated patients, presented by Sommerlad [18], combines minimal
by the rather low occurrence of velopharyngeal flaps found hard palate dissection with radical repositioning of the velar
in the sub-group CPS without additional malformations in musculature and tensor tenotomy. Fistulas occurred in 12%
this study (Table 2). Further analysis of speech outcomes is of the CPO clefts, as opposed to a fistula frequency of 5% in
needed to support this hypothesis though. the present study. However, this technique is being investi-
In the present study, 17% of the patients have had sec- gated in the TOPS (timing of primary surgery) trial, and more
ondary pharyngeal surgery at 10 years of age, and later fol- will be reported in future.
low-up may increase this figure. Our findings that CPS had a After closure of the hard palate, seven fistulas occurred, of
lower frequency of pharyngeal flaps than CPH was supported which five needed a reoperation (only 5% of the entire
by Brunnegård and Lohmander [14], who report that none of group), which is in the same low range as has been reported
the children with a cleft in the soft palate had required a by others [10,19,20]. Fistulas are common in palatal repairs
pharyngeal flap, while 8% of the children with CHP operated and vary from low numbers up to more than 50% [20]. A fre-
on with a two-stage procedure similar to ours had one at 10 quency of 50% means in reality that two procedures are
years of age. The reported incidence of secondary pharyngeal used in half of the patients – meaning a two-stage procedure
surgery in CPO when using a one-stage protocol varies, prob- – but with a more complicated situation for surgical repair,
ably depending on more factors than the surgical technique since fistulas involve more scarring. Against this, a residual
JOURNAL OF PLASTIC SURGERY AND HAND SURGERY 233

cleft has intact surrounding tissue and is subsequently easier though, due to differences in characteristics and in order to
to close, which speaks in favour of a two-stage protocol gain a better understanding of the outcome data.
for CPH. To sum up, our primary goal with this individually staged
Delayed closure of a cleft in the hard palate after early protocol for CPS and CPH – to achieve better maxillary growth,
soft palate closure has shown that the palatal shelves con- with at least the same or better speech outcomes – seems to
tinue to grow medially, reducing the residual cleft, until 3 be reached. However, follow-up until adulthood is needed to
years [21]. However, this does not seem to occur in ICP clefts. confirm this. Furthermore, the results indicate that, by closing
The palatal shelves in CPO patients have continuous growth the hard and soft palate separately, the effect on maxillary
before soft palate closure, but this growth seems to stop growth for the majority of CPO patients is reduced. Thus, large
after this surgery [21,22]. This implies that there is no residual mucoperiosteal flaps could be applied when necessary, mean-
cleft reduction to be expected. On the other hand, during ing in 30% of CPH, instead of in all CPO.
growth of the child, the whole hard palate and the dental
arch grow, giving larger dimensions of the palatal shelves in
relation to the residual cleft, making it easier to close without Conclusion
leaving denuded bone for secondary healing. In the present In conclusion, the described staged protocol for the repair of
study the residual cleft closure was performed at 4 years, CPO, including the techniques for soft and hard palate repair,
which might be too long to wait, since the speech evaluation are safe regarding perioperative surgical results. The need for
at 5 years has shown in a previous study that 46% of secondary interventions for fistula repair or speech-improving
CPH þ patients had retracted oral articulation [11]. Therefore, surgery is comparatively low. Furthermore, the study indi-
the residual cleft closure should preferably be performed at cates that the presence of a cleft in the hard palate and/or
an earlier age, but without affecting the dental arch. additional malformation or syndromes have a negative
The isolated cleft palate is one of the three main cleft sub- impact on the development of the velopharyngeal function.
groups; each having its own special characteristics, the cleft
sub-groups should be regarded as distinct entities. The inci-
dence of additional conditions varies between them, with the Disclosure statement
highest for the CPO sub-group. In a large European study
The authors report no conflicts of interest. The authors alone are respon-
based on 6 million births in 16 countries, 54.8% of clefts were sible for the content and writing of the paper.
isolated, with no other anomalies present, 18.0% were associ-
ated with multiple congenital anomalies, and 27.2% were in
recognised conditions [1]. Of the children who underwent sur- References
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