You are on page 1of 11

68 

Technique for Cleft Palate Repair


Brian Sommerlad, Serryth Colbert

KEY POINTS
• The main debates over indications in cleft palate repair are • The Veau-Wardill-Kilner pushback procedure should be
about timing and whether closure of the hard palate consigned to history.
should be delayed. • The Sommerlad intravelar veloplasty has the least negative
• The author’s technique of cleft palate repair involves a impact on maxillary growth and is associated with good
single layer vomerine flap closure of the hard palate at lip speech outcomes.
repair to repair the hard palate and nasal floor.
• Twenty percent of cleft palate cases require lateral releasing
incisions—the von Langenbeck technique.

INTRODUCTION In a complete bilateral cleft lip and palate (BCLP), the vomer
passes back from the premaxilla and is not attached to the
Embryology palatal shelves (see Chapter 67, Figure 67-15).
The palate is formed by the palatal processes of the maxilla. The In an isolated cleft palate, the vomer is usually midline
central part of the lip (prolabium) and the premaxilla arise and vertical and attached to the hard palate as far back as the
from the medial nasal prominences of the frontonasal process anterior extent of the cleft (see Chapter 67, Figure 67-21).
and the lateral alveolar segments from the lateral processes of In clefts of the soft palate alone, the vomer is not involved.
the maxilla. The primary palate, embryologically, is that part of Isolated clefts of the palate are sometimes divided into V-shaped
the anterior palate, alveolus, and lip in front of the incisive clefts and U-shaped clefts, although the distinction is often
foramen. This fuses from behind forward in the fifth to sixth not clear.
week of intrauterine life. The secondary palate fuses from the The classical SMCP shows the features of:
incisive foramen in the seventh to eighth week of intrauterine • Bifid uvula
life. Each of these processes may be arrested at any point, and • Notch of the hard palate
this accounts for the varying types of clefts of the lip and palate • Diastasis of the soft palate (velar) musculature
(see Chapter 67, Figures 67-2 and 67-3). Kaplan described the occult SMCP, where these typical fea-
tures did not exist.2 Sommerlad and colleagues argued that
Types there was a spectrum of severity in submucous clefting and
Clefts of the palate may occur alone or be associated with uni- described a scoring technique where the degree of abnormality
lateral or bilateral cleft lip. They may be complete or incomplete of the triad was scored according to severity.3 Although usually
and if incomplete may involve soft palate alone or soft and part regarded as a separate entity from isolated cleft palate, SMCP is
of the hard palate. probably just part of the spectrum. Functionally, the effects of
Submucous cleft palate (SMCP) is a condition where the a submucous cleft on feeding and speech may be just as severe
mucosa is largely intact (apart, classically, from a bifid uvula) as those of a complete cleft.
but the musculature is similar to that seen in a complete cleft
palate. Incidence
One method of describing palatal type is the “striped-Y” The incidence of cleft lip and palate in Caucasians is about 1
technique (see Chapter 67, Figure 67-4).1 per 1000 and of isolated cleft palate about 1 per 1500. The
incidence of SMCP has been given as 1 in 2000, but this depends
Morphology on a diagnosis that is less accurate. Clefts of the lip and palate
In a unilateral cleft lip and palate (UCLP), the vomer is attached are more common in Asians (approximately 1.8 per 1000) and
to the palate on the non-cleft side. The cranial portion of the less common in Afro-Caribbeans (about 0.4 per 1000). They are
vomer is almost vertical, and the caudal part is angulated (often also more common in males than in females. In contrast, iso-
at almost 90 degrees) toward the palatal shelf to which it is lated clefts of the palate demonstrate little difference in either
attached (see Chapter 67, Figure 67-21). sex or racial incidence.

972
CHAPTER 68  Technique for Cleft Palate Repair 973

hamulus (to which it is also partially attached) and then fans out
Etiology to form the palatal aponeurosis (the anterior third of the velum).
There are several hypotheses regarding the pathogenesis of cleft:
• Hypoplasia: The palatal shelves are simply too small to meet Hearing
at the critical time. There is an increased incidence of serous otitis media (glue ear)
• Dysplasia: The palatal shelves may be prevented from joining in babies with clefts of the palate. This is probably due to the
by the tongue or, rarely, hamartomas or other space- abnormal anatomy of the palatal muscles. Both the levator
occupying lesions. palati and the tensor palati muscles have a role in eustachian
• Breakdown: In some situations, there may be a tenuous function.
union that breaks down.
Different mechanisms may be responsible. Associated Syndromes
In cleft palates associated with cleft lip (UCLP and BCLP),
Etiological Factors associated syndromes occur in probably less than 10% of cases.
The current view is that the etiology of cleft palate is multifacto- However, in isolated cleft palate associated syndromes are
rial. However, some relevant factors are: described in up to 50%. The most common syndromes are:
• Genetic: Cleft palate associated with cleft lip may have a • Pierre Robin syndrome: Better described as a sequence,
genetic basis in that the incidence in a first-degree relative because it is a descriptive term to describe a cleft palate
(offspring or sibling) is approximately 40 times as common (usually U-shaped) associated with a small jaw (microgna-
as the rest of the population. In isolated cleft palate, the thia), a retrodisplaced tongue, and, classically, airway prob-
genetic influence appears to be less marked. A number of lems in the neonatal period. The Pierre Robin sequence
genes associated with clefts have been identified, but their includes patients with:
significance is still uncertain in most cases. • Sticklers syndrome: Where there is associated myopia and
• Teratogens: There is good evidence that maternal smoking joint problems
increases the incidence of clefting, as does high alcohol • Craniofacial microsomia
intake and certain antiepileptic drugs (such as, phenytoin • Treacher Collins syndrome
and sodium valproate). • Velocardiofacial syndrome (22q11.2 deletion syndrome):
• Syndromes: See later. This microdeletion, identified by the fluorescent in situ
hybridization (FISH) test, may be associated with overt cleft
Anatomy palate, classical SMCP and varying degrees of less classical
The musculature of the cleft velum is disordered. The levator SMCP, and, in some cases, simply VPI with no apparent
palati muscle arises from its normal origin in the skull base and palatal abnormality.
passes downward and medially to be inserted into the cleft • Van der Woude syndrome: This autosomal dominant condi-
margin in the anterior half of the velum (but not directly into tion (with varying expressivity) is usually diagnosed because
the back of the hard palate as commonly stated). This is in dis- of associated lower lip pits, and it is unusual in that it may
tinction to the anatomy in the normal cleft velum, where the be expressed as either cleft lip, with or without cleft palate,
levator muscle reaches the midline in the middle 40% of the or isolated cleft palate.
velum.4 The palatopharyngeus and the palatoglossus muscles • Apert and Crouzon syndromes: In these syndromes, the cleft
(on the oral or caudal side of the velum) are also attached some- is unusual in that there is usually a very high arched palate.
what anteriorly with fibers fanning out—the more lateral fibers There are, however, many other syndromes associated with
being orientated in a lateroposterior direction and passing clefting.
toward the maxillary tuberosity and the more posterior fibers
being more oblique. These fibers occupy the anterior two-thirds SURGERY
to three-quarters of the velum. In contrast, in the normal cleft
velum, these fibers rarely reach the back of the hard palate. The Cleft Palate (CULP, Bilateral Cleft Lip and Palate,
presence of the musculus uvulae muscles in the cleft palate is and Isolated Cleft Palate)
controversial and rarely seen (except, possibly, in some very The main debates over indications in cleft palate repair are
occult submucous clefts). The role of the musculus uvulae in the about:
non-cleft is debated. Its absence has been thought by some to • Timing
produce the V-shaped groove or lack of convexity of the nasal • Whether closure of the hard palate should be delayed
surface of the palate and therefore to be a major cause of velo- It is generally agreed that at least the soft palate should be
pharyngeal incompetence (VPI).4 It may have a role in elevation repaired ideally before 12 months old. The main contraindica-
of the posterior velum to increase contact with the posterior tions to palate repair at the optimal age are:
pharyngeal wall after elevation of the velum by the levator. • Associated conditions: An example is congenital cardiac con-
Others argue that the musculus uvulae may not be very impor- ditions, which result in an unacceptable anesthetic risk.
tant in normal velopharyngeal function.4 The tensor palati • Suboptimal weight: Although repair of the palate may
muscle is not actually a palatal muscle but passes down from improve feeding and actually increase weight gain.
skull base to the pterygoid hamulus where, in the cleft, it par- • Unrepaired clefts in an adult: Arguably, compensatory pat-
tially attaches and appears to diverge into two components: (1) terns of articulation may be so well embedded in some adults
a triangular tendinous insertion into the back of the hard palate, with unrepaired clefts that palate repair is unlikely to make
lying closely applied to the nasal mucosa, and (2) a more flimsy very much difference to speech. Certainly, such patients
component, which passes orally toward the oral surface. In the usually require considerable speech therapy input.
normal palate, in comparison, the tensor tendon surrounds the Repair of the SMCP is not always mandatory.
974 CHAPTER 68  Technique for Cleft Palate Repair

PREOPERATIVE HISTORY AND CONSIDERATIONS premaxilla and vomer to become continuous with the lateral lip
incision (see Chapter 67, Figure 67-21). The flap is elevated and
Management in the Neonatal Period inserted underneath the oral mucoperiosteum, which has been
Feeding elevated following incision at the margins of the cleft using a
Because of reduced ability to suck and particularly to draw the Beaver 69 blade. Double-breasting of the flap beneath the oral
nipple into the mouth, babies with clefts of the palate usually mucoperiosteum is important to achieve successful closure (see
have some difficulty feeding, and breastfeeding can only be Chapter 67, Figure 67-22). In a bilateral cleft, complete eleva-
achieved with considerable efforts and modification of tech- tion of the vomerine flap on both sides may jeopardize the
niques. In the Western world most babies with cleft palates are blood supply of the premaxilla and prolabium. The author now
mostly bottle fed, although many mothers use expressed milk. carries out only a very conservative vomerine flap on the second
The principle of bottle feeding is to allow a steady stream of side (usually the narrowest) with the flap not being elevated
milk, usually by a modified teat and a soft bottle. only behind the pre-vomerine suture.
In unilateral clefts of the lip and palate, this results in a
Airway degree of closure in most cases, which leaves virtually a cleft of
Babies with Pierre Robin sequence may have significant airway the soft palate for palate repair. Closure will be less adequate on
problems, largely because of the retrodisplaced tongue, which the less radically treated side in a bilateral cleft.
obstructs both the nasal and the oral airway.
Many would argue about the indications for each of these Palate Repair
techniques and the order of this algorithm. There are enthusi- At palate repair, the author aims to repair the cleft from the
asts for tongue-lip adhesions and others who rarely perform margins without lateral releasing incisions. This is now possible
them. The role of mandibular distraction is debated. All would in approximately 80% of cases, and in only about 20% of cases
agree that tracheostomy is best avoided if possible, but it can be are lateral releasing incisions (the von Langenbeck technique8,9)
life-saving and can result in a previously underweight baby necessary (Figure 68-1).
thriving by reducing the work of respiration. However, trache- The operative steps are as follows:
ostomy has a morbidity and mortality rate. Nasopharyngeal 1. Insertion of a modified mouth gag (Figure 68-2).10 This is
airways are a very safe and nonsurgical solution for many designed to allow good exposure, particularly with an oper-
patients. However, the introduction and positioning is critical. ating microscope, to avoid compression of the endotracheal
They can be managed by parents at home.5 tube against the mandible.
2. Insertion of a gauze throat pack.
Pre-Surgical Orthopedics 3. Infiltration of local anesthetic with adrenaline.
There has been much controversy about the role of pre-surgical
orthopedics. These involve a plate, which may be active or
passive. In clefts of the lip, this may be associated with tech-
niques of strapping or nasoalveolar molding. A recent prospec-
tive randomized controlled trial failed to show benefits for
feeding or significant benefits for speech.6

Hearing
Neonatal assessment of hearing, as in all babies, is important.
If there is evidence of persistent conductive hearing loss or glue
ear in a cleft baby, it may be necessary to insert ventilation tubes
(grommets) at the time of palate repair. There is some evidence Bilateral von
that repairing the palate musculature improves eustachian Langenbeck
function and therefore hearing, and the author prefers to defer relieving
grommet insertion and reassess the hearing 3 to 6 months after incisions
surgery. If there is persistent significant hearing loss at that
stage, either grommet insertion or short-term hearing aids are
recommended.

OPERATIVE APPROACH
The Author’s Technique
Single Layer Vomerine Flap Closure of the Hard Palate
at Lip Repair
The author aims to repair the entire palate at 6 months. The
operating microscope has been used in all palate repairs since
1991.7 Since 1993, the author has used a single layer vomerine
flap closure of the hard palate in clefts of the lip and palate to
repair the hard palate and nasal floor.7 An incision is made at
the junction of palatal and vomerine mucosa, extending poste-
riorly down the crest of the vomer and anteriorly around the FIG 68-1  Cleft margin incision with von Langenbeck relieving
side of the premaxilla on the oral side if the groove between the incisions bilaterally. (Courtesy Steve Atherton and David Drake.)
CHAPTER 68  Technique for Cleft Palate Repair 975

Modified
mouth
gag

FIG 68-2  Modified mouth gag. (Courtesy Steve Atherton and FIG 68-3  Incision along cleft margin at junction of oral and nasal
David Drake.) mucosa. (Courtesy Steve Atherton and David Drake.)

4. Incision along the margin of the cleft at the junction of the


oral and nasal mucosa (Figure 68-3). This junction is
usually clearly seen, because the nasal mucosa is more pink
and telangiectatic or more pigmented in patients with
darker skin and is usually on the oral side of the edge of the
cleft. This incision extends from the midline in the front of
the cleft far enough to enable the oral flaps to be elevated
in a subperiosteal plane, to expose the bony palatal shelves
(Figure 68-4).
5. The oral mucoperiosteal flaps are elevated with the dissec-
tion extending back to the posterior edge of the hard palate.
There are frequently dimples seen on the oral side of the
palate at a point where oral mucous glands are attached to
the posterior border of the hard palate and anterior tensor
palati tendon. Care needs to be taken to elevate these
mucous glands intact from the posterior border of the hard
palate and the white tendinous nasal component of the
tensor palati tendon.
6. If necessary, the greater palatine neurovascular bundle is
exposed, and an instrument is passed around the greater
palatine neurovascular bundle to free it—sometimes after
gentle incision of the periosteal sheath surrounding it. This
step of the procedure is only undertaken in wider clefts,
where radical mobilization of the oral layer is necessary.
7. The oral component of the tensor tendon can be seen and
felt behind the greater palatine vessels, and this is incised if
necessary to achieve mobilization of the oral layer.
8. The dissection is continued back into the soft palate, sepa-
rating the oral mucosa and underlying mucous glands from FIG 68-4  Oral flap elevated, exposing palatal shelf. (Courtesy
the underlying musculature—the palatopharyngeus and Steve Atherton and David Drake.)
976 CHAPTER 68  Technique for Cleft Palate Repair

FIG 68-5  Mobilization of nasal mucosa from nasal surface of FIG 68-6  Closure of nasal layer with eversion of nasal mucosa
palatal shelf. (Courtesy Steve Atherton and David Drake.) toward nasal surface. (Courtesy Steve Atherton and David
Drake.)

palatoglossus muscles. This is done by a combination of laterally are divided from the back of the hard palate by
knife and blunt dissection and extends to the posterior knife dissection to the nasal mucosa and by a combination
border of the vomer laterally to the pterygoid hamulus. of gentle knife incision and brushing back, the beginning
9. The nasal mucosa is then mobilized if necessary from the of the retrodisplacement of the musculature is performed.
nasal surface of the palatal shelves (Figure 68-5). This is best 14. The knife then turns almost at right angles and in an
done after an incision at the edge of the palatal shelves, and anteroposterior direction, and most of the tensor palati
then a blunt dissection is made in a subperiosteal plane. tendon is divided just medial to the hamulus.
10. The nasal mucosa, with its attached muscle, is then sutured 15. With tension maintained on the muscle, the combined
with non-absorbable sutures (the author uses 5/0 Monocryl) levator palati and palatopharyngeus muscle is further retro-
inserted in the nasal mucosa close to the edge but then displaced with a combination of sharp and blunt dissection.
picking up more of the mucous glands (and a little muscle When the most posterior fibers of the tensor palati tendon
anteriorly). The aim is a suture that will evert the nasal layer are divided medial to the posterior end of the hamulus, a
toward the nasal surface (Figure 68-6). noticeable freeing is usually achieved, and the palatopha-
11. Where a vomerine flap has been used for closure of the hard ryngeus fibers can be split with a dental scaler to demon-
palate in UCLP at the time of lip repair, a posterior-based strate the levator palati muscle with its thin sheaths passing
flap of the neo-mucosa overlying the vomer may be raised cranially toward the skull base. Frequently there is a vessel
to facilitate closure of the anterior nasal layer. lying anterior to the levator palati muscle, and a small
12. An incision is then made on each side of the midline, begin- branch passing anteriorly can be divided after coagulation,
ning 3 to 5 mm from the midline posteriorly, and passing leaving the major vessel intact as it is retrodisplaced.
closer to the midline anteriorly, just lateral to the previously 16. Dissection continues until the muscle bundle is free from
inserted nasal layer sutures. This knife dissection extends the nasal layer and mobile (Figure 68-7). Care is taken to
deeply to the nasal mucosa, leaving mucous glands laterally. avoid unnecessary damage to the neurovascular structures.
The nasal mucosa is recognized because of its almost blue 17. A stab incision is made into the nasal mucosa (unless an
color and a fine plexus of vessels running on it. Using knife accidental hole has been created). This helps to avoid a
dissection and a sucker for retraction, the plane is then hematoma.
developed between the nasal mucosa and the palatal 18. The muscle is united, usually in the posterior half of the
muscles. This allows the nasal surface of the levator palati velum, with non-absorbable 4/0 or 5/0 nylon sutures
muscle to be identified as it passes toward the cleft margin (Figure 68-8). The posterior suture is inserted to maintain
in the anterior half of the velum. correct orientation and rotation of the muscles. Two sutures
13. With a skin hook in the musculature, the palatopharyngeus are then inserted in the levator palati muscle. The knots are
medially and the nasal component of the tensor tendon on the nasal side. The anterior of these muscle sutures
CHAPTER 68  Technique for Cleft Palate Repair 977

FIG 68-7  Levator palati muscle bundle mobilized and free from FIG 68-9  Closure of oral layer with bilateral von Langenbeck
nasal layer. (Courtesy Steve Atherton and David Drake.) relieving incisions. (Courtesy Steve Atherton and David Drake.)

includes the divided and retrodisplaced tendons of the


tensor palati tendon.
19. Closure of the oral layer commences. The first suture in the
oral layer is inserted just in front of the retrodisplaced
muscle sling and picks up the nasal mucous glands. A
twisted loop mattress suture is used at this point and usually
one or two further sutures anteriorly.11 (A twist in the loop
with the needle inserted through the twisted loop from the
side to which it is twisted reduces the likelihood of stran-
gulating the small segments of oral mucosa.) The aim of
these sutures, uniting oral and nasal layers, is to keep the
muscle in a posterior position and to occlude dead space
and further reduce the chances of hematoma. The author
uses a 4/0 Monocryl suture on a modified 5/8 curved needle
(Ethicon B-17).
20. Closure of the remainder of the oral layer overlying the
muscle and including the uvula and also anteriorly is com-
pleted. In very wide clefts (usually more than 10 mm in
Levator
width at the back of the hard palate and 6 mm in the hard
palati
united palate), lateral releasing incisions are sometimes necessary
(in about 20% of cases), and these lateral releasing incisions
are sometimes made prospectively at the beginning of the
procedure if it is thought that they will be necessary, but
otherwise they are sometimes performed at the end of the
procedure if closure, particularly at the junction of hard
and soft palate, is thought to be too tight (Figure 68-9).
However, the tension in the oral layer is reduced as soon as
the gag is released, and it may be worth partly releasing the
gag and retesting the tension before embarking on late
FIG 68-8  Levator palati muscle bundles united in posterior half lateral releasing incisions.
of velum. (Courtesy Steve Atherton and David Drake.) 21. After aspiration of the pharynx, the throat pack is removed.
978 CHAPTER 68  Technique for Cleft Palate Repair

22. The author then performs a suction test, which involves old and delayed repair of the hard palate until 12 years old.19
putting a sucker in one nostril, occluding the nose around Others have repaired the hard palate at 7 to 8 years old or
it, and testing the passive length and mobility of the velum. younger.20 The soft palate closure may be performed by a similar
If the velum closes against the posterior pharyngeal wall technique to that described by the author. Closure of the ante-
spontaneously and stays up when the suction catheter is rior nasal layer may be facilitated by a posteriorly based flap
removed, this is graded as follows: from the vomer.
0—No closure
1—Remains elevated after gentle elevation with finger POSTOPERATIVE CARE
2—Elevates spontaneously
3—Remains elevated after removal of the sucker Airway
This has been shown to be predictive of outcome in both The airway may be compromised in the early postoperative
primary repairs and palate re-repairs. period. A tongue stitch, inserted at operation, may be a useful
emergency measure. If problems are anticipated, a nasopharyn-
Submucous Cleft Palate Repair geal airway can be very helpful but needs to be of appropriate
The SMCP is repaired using a similar technique with the fol- length (with the end situated below the palate but above the
lowing differences: epiglottis) and requires regular effective suction. A blocked
• The incision does not have to reach as far forward, but the nasopharyngeal airway is worse than none.
oral flaps do need to be elevated subperiosteally to expose
the back of the hard palate. Feeding
• Mobilization of the greater palatine vessel and division of the oral The author allows babies to feed immediately after repair by
component of the tensor palati tendon should not be necessary. whichever method they fed before. This needs to be linked with
• The aim is to keep the nasal layer intact. This can be chal- effective analgesia, which does not sedate the baby too much.
lenging in patients with a very thin translucent zone. In these
cases, careful instillation of local anesthetic between the oral Antibiotics
and nasal layers may facilitate this dissection, which should The need for antibiotics is debated. The author uses penicil-
be made with a knife. lin V elixir for 5 days in infants as prophylaxis against infec-
• The dissection lateral to the midline needs to be carried out tion with β-hemolytic streptococcus, which can be potentially
with care. There are often very few nasal mucous glands very disruptive to the repair. The evidence for this is not
present and the nasal layer may be thin. strong.

ALTERNATIVE TECHNIQUES Arm Restrainers (Splints)


Many surgeons recommend restrainers to prevent infants
Pushback Procedures potentially disrupting the repair with fingers or thumb. Follow-
A three or four flap Veau-Wardill-Kilner pushback procedure, ing a small randomized controlled trial on the author’s patients,
which aimed to lengthen the palate, has not been shown to be this has been abandoned.21
effective in improving speech and almost certainly increases the
incidence of crossbite and maxillary retrusion.12-15 It may result OUTCOMES
in fistulae at the junction of the flaps, which can be extremely
difficult to close. The author believes that this technique should Closure of the palate is the most important component of cleft
be consigned to history. surgery, and the aim is to optimize the following:
• Speech and language
The Two-Flap Technique • Maxillary growth and arch development
The long two-flap technique, which was popularized by Bardach, • Hearing
does not aim to achieve pushback but is believed by its propo- • Appearance, which is influenced to a major degree by maxil-
nents to improve exposure.16 Many specialists simply replace lary retrusion
the flaps to avoid leaving raw areas. • Psychological and social well-being, which is also influenced
by appearance as well as by speech and hearing
The Double Opposing Z-plasty All of these factors are very dependent on appropriate, well-
The double opposing Z-plasty technique, as described by performed palate surgery.
Furlow, is popular in many centers.17 It may be performed The final outcome of palate surgery cannot be assessed until
without lateral releasing incisions or with lateral releasing inci- maturity, because maxillary arch relationship and maxillary
sions.18 The aim is to achieve lengthening of the velum by growth tend to become less satisfactory with age and as VPI also
Z-plasty and retrodisplacement of the muscle with the mucosal may become unsatisfactory later in life.7
Z flaps, with the muscle left attached to the oral flap on one side Consideration also needs to be given to the effect of speech
and the nasal flap on the other. problems or maxillary retrusion in early childhood, even if they
Many surgeons feel unhappy with this technique in wide can be corrected by secondary surgery. The aim of primary
clefts and, therefore, use it in easy and narrow clefts. It is diffi- surgery should be to achieve the best possible outcome with
cult to compare outcomes with other techniques. minimal need for secondary surgery. The burden of care for
patients and for parents of children is important.
Delayed Hard Palate Closure Because of the poor quality of much of the literature, few
The technique of delayed hard palate closure was pioneered by well conducted inter-center or prospective studies with large
Schweckendiek, who repaired soft palate at about 18 months numbers of patients, and even fewer randomized trials, the
CHAPTER 68  Technique for Cleft Palate Repair 979

evidence for individual techniques is poor. However, some that there were differences between the surgeons in the study.
general conclusions can be made. A later study comparing outcomes in patients from the original
randomized controlled trial with later patients who had more
Speech and Language radical muscle dissection demonstrated better speech and
It is generally agreed that at least the soft palate is best repaired hearing in patients having the more radical dissection.25
before 12 months old to provide the basis for the acquisition A study of the author’s speech outcomes has recently been
of speech and language and to minimize the need for speech carried out.26 This involved an independent analysis of speech
and language therapy.22 Velopharyngeal competence is usually recordings taken at 5 years old by two specialist speech and
recorded as pharyngoplasty rates, and these are dependent on: language pathologists blinded to the study. The patients were
• Inclusions and exclusions born between 1993 and 2005, and all non-syndromic clefts were
• The length and completeness of follow-up included in the preliminary study. Unfortunately, of 877 patients
• The threshold of the cleft team to perform operations, such operated on in that period, 165 had significant syndromes
as pharyngoplasty affecting learning, 251 did not attend for 5-year review, and 70
• The patient’s and/or parent’s wishes had unsatisfactory audio/visual samples; 391 patients were
Follow-up in most series has been relatively short. A major blindly assessed. The inter- and intra-reliability of the observers
survey of patients with UCLP in the United Kingdom showed was confirmed.
the need for secondary velopharyngeal surgery in about 30% of Of the 391 patients, 156 (40%) had UCLPs, 51 (13%) had
cases in 5- and 6-year-olds and 12- and 13-year-olds. Two of BCLPs, and 184 (4.7%) had isolated clefts of the palate. Of these
the reported series have been those of Cutting, who reported a 184 patients with isolated cleft palates, 72 were classified as
higher pharyngoplasty rate at 6 years old, and Sommerlad, who Pierre Robin sequence.
reported a secondary surgery rate of 4.9% at 6 years old and At 5 years old, 10 patients (2.6%) had had secondary surgery
5.9% at 10 years old.23 However, a few patients will require for speech, two had insufficient velopharyngeal function, and
surgery between 10 and 20 years old. All such figures need to eight had borderline velopharyngeal function. The remaining
be taken with some skepticism because of the difficulties 381 children were regarded as having sufficient velopharyngeal
described earlier. A randomized controlled trial of muscle repair function. The overall VPI rate (including those who had had
(intravelar veloplasty) versus none showed no significant differ- VPI surgery) was 5.2%; 15% (58) had had speech therapy for
ence.24 However, the author has confirmed that the intravelar cleft-related errors. The outcomes in these 391 patients are
veloplasty did not involve very radical muscle correction and shown in Figure 68-10.

FIG 68-10  Independently assessed speech outcomes from recordings in 391 unselected 5-year-
old patients after cleft palate repair. Each row of four boxes represents one patient. Columns
represent speech score. Column 1 is nasality, column 2 is audible nasal emission, column 3 is
nasal turbulence, and column 4 is passive cleft speech characteristics (indicative of velopharyn-
geal dysfunction). Dark green, Normal; light green, inconsistent; yellow, mildly abnormal; orange,
moderately abnormal; red, severely abnormal; purple, patients where assessment of recordings
is not possible. (Adapted from Bardach J, Salyer KE: Cleft palate repair. In Bardach J, Salyer KE,
editors: Surgical techniques in cleft lip and palate, St Louis, 1991, Mosby Year Book.)
980 CHAPTER 68  Technique for Cleft Palate Repair

The 10 patients (2.6%) who had had secondary surgery tubes (grommets) may be indicated. If necessary, hearing aids
had been managed by palate re-repair (5), modified Hynes should be used.
pharyngoplasty (3), secondary Furlow (1), and buccinator flap
lengthening (1). There were correlations between the overall Appearance
velopharyngeal function (VPC sum score), the extent of the Although it is generally assumed that in patients with cleft lip
cleft, and the diagnosis of van der Woude syndrome. Overall and palate it is the lip repair that is most important for appear-
fistula rate was 12.8% and was greater in those who had had ance, in fact the palate repair is the most important factor in
lateral releasing incisions, those performed earlier in the period determining maxillary growth (and as maxillary retrusion is
(and therefore earlier in the surgeon’s learning curve), and in often the most obvious cleft feature visually); palate repair is
wider clefts. important in determining appearance, particularly during ado-
lescent years. Although patients can be submitted to orthogna-
Maxillary Growth and Arch Development thic surgery and even early distraction, the fact that a child is
Maxillary growth is commonly measured by: obviously different from his or her peers in the school years can
• Arch relationship, recorded by models of the upper and be a major problem.
lower arches in occlusion: A simple but reproducible scoring
system has been devised by Mars—the Goslon scoring Psychological and Social Well-Being
system; where 1 and 2 are good, 3 is fair (and probably By being crucial for speech and communication, perhaps rele-
manageable orthodontically), and 4 and 5 are poor and vant for hearing and also because of the effect on maxillary
indications for orthognathic surgery.27 This system does growth and therefore appearance, an effective and minimally-
measure the relationship of the maxilla to the mandible destructive palate repair is crucial for the development of a
(which may also be retruded) but not the position in relation rounded individual. However, many studies have shown poor
to skull base. correlation between the objective outcomes in these factors and
• Cephalometry, which measures maxillary position in rela- satisfaction with appearance.
tion to the skull base: SNA measures the relationship of the
maxilla to the skull base, and SNB measures the relationship COMPLICATIONS AND SIDE-EFFECTS
of the mandible. ANB records the difference between maxilla
and mandible and should be positive. The major complications and side-effects of cleft palate repair
Outcome in relation to maxillary growth has been the focus can be divided into early, intermediate, and late.
of many important inter-center studies (Eurocleft, Americleft,
and CSAG).28-30 This has shown wide variation between centers Early Complications
and stimulated the changes in cleft care that have occurred in The two most worrying immediate complications are bleeding
Europe and are now occurring in the United States. and airway obstruction:
It is still not clear which regime is best as far as maxillary • Bleeding: Bleeding probably occurs most commonly from the
growth is concerned. For example, delayed hard palate repair lateral releasing incisions or from the margins of elevated
has been shown in some series to give a good result in maxillary flaps. Hemostasis needs to be meticulous. Some surgeons
growth.31 There are others where this has not been confirmed. pack any spaces left after elevation of palatal flaps. The team
In summary, the consensus view can be summarized as managing the infant postoperatively needs to be aware of
follows: silent bleeding, which is where the infant swallows blood.
• Impairment of maxillary growth in clefts is largely but not • Airway: Closure of the palate may compromise the airway,
entirely due to surgery, with both lip repair and palate repair partly because of postoperative swelling and also because of
being implicated. There is some debate as to which is the changes produced in the anatomy of the pharynx by closure.
more important. Most adult patients with an unrepaired This is particularly relevant in babies with Pierre Robin
cleft do not show evidence of impaired maxillary growth. sequence. Some surgeons use a temporary tunnel stitch,
Nevertheless, there are some patients who are intrinsically which does allow immediate control of the tongue position
hypoplastic. and potential improvement in the airway in the first postop-
• The most important surgical factor is the amount of surgery erative hours. The use of an optimally positioned nasopha-
carried out on the hard palate as part of palate closure. Tech- ryngeal airway can be very helpful in the postoperative
niques that minimize surgery on the hard palate appear to period.
produce better growth.
• Delayed repair of the hard palate may be beneficial for Intermediate Complications
growth but is almost certainly detrimental to speech. • Fistulae: Fistula rates are variably reported from 0% to 50%
• Recording maxillary growth at 10 years old (as in many probably based to some extent on the care taken to look
studies) is inadequate, because the relationship of maxilla for them.
and mandible usually deteriorates during adolescence. • Dehiscence: Dehiscence of the repair is probably usually the
result of either infection or hematoma, or closure under
Hearing excess tension. Partial dehiscence results in fistula formation.
It is uncertain whether hearing outcome is related to the age In the Wardill-Kilner pushback technique, these often occur
and technique of palate repair, because good inter-center studies at the junction of the flaps in the hard palate and are very
are not available. difficult to close. In other repairs, they most commonly
However, for an infant in the important months of acquisi- occur at the junction of the hard and soft palate. They can
tion of speech and language, optimal hearing is indicated and, be minimized by the avoidance of hematoma and by avoid-
if necessary, despite the potential disadvantages, ventilation ing undue tension.
CHAPTER 68  Technique for Cleft Palate Repair 981

Late Complications outcomes.40 Significant elongation in palate length is obtained


• Maxillary retrusion: Cleft palate surgery in the developing using with improved velopharyngeal competence.41
child is known to be associated with maxillary hypoplasia.
The decreased prominence of maxillary complex could be Growth
caused mainly by the shortened maxillary length; mean- The isolated cleft palate repair using the Sommerlad intravelar
while, posterior position of the maxillary body may have veloplasty has been shown to have the advantages of less injury
some influence on the maxillary protrusion.32 and less scar tissue, indicating less inhibitory effect on the
growth of the maxilla.42 The Sommerlad intravelar veloplasty
POSTOPERATIVE CARE with levator veli palatini retropositioning reduces the extent of
denuded palatal bone, making the Sommerlad technique advan-
Early tageous for maxillary growth.43
Antibiotics
Primary closure of cleft lip and palate is classified as a clean-
contaminated operation, and wound infection is a recognized PITFALLS
risk. The risks are associated with the duration of operation
• Delayed repair of the hard palate may be beneficial for growth but is
with primary cleft operations often requiring 1 to 2 hours of almost certainly detrimental to speech. The aim of primary surgery
operating time.33 The consequences of surgical wound infection should be to achieve the best possible outcome with minimal need
after repair of cleft lip or palate can be devastating in both the for secondary surgery.
short term and the long term. A major wound infection after • A recent prospective randomized controlled trial failed to show ben-
primary repair of a cleft is likely to require a further admission efits for pre-surgical orthopedics in terms of feeding or speech.
for a secondary intervention; however, final outcomes such as • The Veau-Wardill-Kilner pushback procedure may result in fistulae,
which can be extremely difficult to close. This technique should be
speech and growth may also be compromised. Antibiotics are
consigned to history.
likely to reduce the incidence of wound infection and complica- • There are some patients who have an intrinsically hypoplastic maxilla.
tions, but this has never been clearly shown in randomized Recording maxillary growth at 10 years old (as in many studies) is
clinical trials in repair of clefts.34 inadequate, because the relationship of maxilla and mandible usually
deteriorates during adolescence.
Airway
Anesthesia for cleft surgery in infants and children carries a
higher risk with general anesthesia and airway complications ACKNOWLEDGMENTS
due to associated respiratory problems. The literature shows a
higher incidence of perioperative respiratory complications Thank you to Steve Atherton of Medical Illustration and
when associated with the common cold symptoms in children David Drake, Consultant and Cleft and Oral and Maxillofacial
for cleft repairs.35 Morbidity during general anesthesia is associ- Surgeon, both of Morriston Hospital, Swansea, Abertawe Bro
ated with the difficult airway, endotracheal tube compression/ Morgannwg, for providing the photos for this chapter.
disconnection, disconnection or accidental extubation, and
postoperative airway obstruction.36,37 Assessment of the degree
of difficulty during intubation is not always possible preopera- REFERENCES
tively. Any child with hypoplastic mandible or wide cleft palate 1. Kernahan DA: The striped Y—a symbolic classification for cleft lip and
increases the risk of tongue prolapse into the nasopharynx and palate. Plast Reconstr Surg 47(5):469–470, 1971.
poses a problem during induction of anesthesia.38 2. Kaplan EN: The occult submucous cleft palate. Cleft Palate J 12:356–368,
1975.
Feeding 3. Sommerlad BC, Fenn C, Harland K, et al: Submucous cleft palate: a
Provision of efficacious feeding instructions to the parents after grading system and review of 40 consecutive submucous cleft palate
cleft palate repair can be a challenge for health care providers. repairs. Cleft Palate Craniofac J 41(2):114–123, 2004.
Feeding should commence as soon as the baby is awake after 4. Boorman JG, Sommerlad BC: Musculus uvulae and levator palati: their
anatomical and functional relationship in velopharyngeal closure. Br J
receiving anesthetic. A nasogastric tube may be used if difficul-
Plast Surg 38(3):333–338, 1985.
ties are encountered with feeding. Children should only be dis- 5. Mackay DR: Controversies in the diagnosis and management of the
charged when they are back to a normal feeding pattern of milk Robin sequence. J Craniofac Surg 22(2):415–420, 2011.
and pureed food. 6. Masarei AG, Wade A, Mars M, et al: A randomized control trial
investigating the effect of presurgical orthopedics on feeding in infants
Arm Splints with cleft lip and/or palate. Cleft Palate Craniofac J 44(2):182–193, 2007.
Arm restraints are used in children after cleft surgery to prevent 7. Sommerlad BC: A technique for cleft palate repair. Plast Reconstr Surg
traumatic disruption of the repair. However, a prospective ran- 112(6):1542–1548, 2003.
domized trial of 46 children having primary cleft palate repair 8. von Langenbeck B: Weitere erfahrungen im gebiete der uranoplastic
showed that arm splints did not decrease the incidence of oro- mittels ablusung des mucosperriostealen gaumenüberzuges. Arch Klin
Chir 5:170, 1861.
nasal fistulae.39 The use of arm splints after cleft palate repair
9. von Langenbeck B: Die uranoplastik mittles ablosung des
has been abandoned by the authors. microsperiostalen gaumenüberzuges. Arch Klin Chir 2:205, 1862.
10. Sommerlad BC, Mehendale FV: A modified gag for cleft palate repair.
Late Br J Plast Surg 53(1):63–64, 2000.
Speech 11. Rees LS, Sommerlad B: Twisted loop mattress suture. Ann R Coll Surg
The Sommerlad intravelar veloplasty has the least negative Engl 94(4):274, 2012.
impact on maxillary growth and is associated with good speech 12. Veau V: Division palatine, Paris, 1931, Masson & Cie.
982 CHAPTER 68  Technique for Cleft Palate Repair

13. Veau V, Ruppie C: Anatomie chirurgicale de la division palatine. J Chir cleft lip and palate part 2. Dental arch relationships. Cleft Palate
(Paris) 20:1, 1992. Craniofac J 48(3):244–251, 2011.
14. Wardill WEM: Technique of operation for cleft lip and palate. Br J Surg 30. Bearn D, Mildinhall S, Murphy T, et al: Cleft lip and palate care in the
25:97, 1937. United Kingdom—the Clinical Standards Advisory Group (CSAG) Study.
15. Kilner TP: Cleft lip and palate repair technique. In Maingot R, editor: Part 4: outcome comparisons, training, and conclusions. Cleft Palate
Postgraduate surgery, vol 3, London, 1937, Medical Publishers. Craniofac J 38(1):38–43, 2001.
16. Bardach J, Salyer KE: Cleft palate repair. In Bardach J, Salyer KE, editors: 31. Lilja J, Mars M, Elander A, et al: Analysis of dental arch relationships in
Surgical techniques in cleft lip and palate, St Louis, 1991, Mosby Year Swedish unilateral cleft lip and palate subjects: 20-year longitudinal
Book, pp 224–273. consecutive series treated with delayed hard palate closure. Cleft Palate
17. Furlow LT, Jr: Cleft palate repair by double opposing Z-plasty. Plast Craniofac J 43(5):606–611, 2006.
Reconstr Surg 78(6):724–738, 1986. 32. Jiang C, Yin N, Zheng Y, et al: Characteristics of maxillary morphology
18. Randall P: The management of cleft-lip and cleft-palate patients. Am J in unilateral cleft lip and palate patients compared to normal subjects
Med Sci 233(2):204–219, 1957. and skeletal class III patients. J Craniofac Surg 26(6):e517–e523, 2015.
19. Schweckendiek W: [Primary closure of cleft lip and cleft palate]. 33. Culver DH, Horan TC, Gaynes RP, et al: Surgical wound infection rates
Zahnarztl Prax 34(8):317–320, 1983 [Article in German]. by wound class, operative procedure and patient risk index. National
20. Friede H, Enemark H: Long-term evidence for favourable midfacial Nosocomial Infections Surveillance System. Am J Med 91(3B):152–157,
growth after delayed hard palate repair in UCLP patients. Cleft Palate 1991.
Craniofac J 38(4):323–329, 2001. 34. Smyth AG, Knepil GJ: Prophylactic antibiotics and surgery for primary
21. Jigjinni V, Kantesu T, Sommerlad BC: Do babies require arm splints after clefts. Br J Oral Maxillofac Surg 46(2):107–109, 2008.
cleft palate repair? Br J Plast Surg 46:681–685, 1993. 35. Sandberg DJ, Magee WP, Jr, Denk MJ: Neonatal cleft lip and cleft palate
22. Ysunza A, Pamplona MC, Mendoza M, et al: Speech outcome and repair. AORN J 75(3):490–498, 2002.
maxillary growth in patients with unilateral complete cleft lip/palate 36. Law RC, de Klerk C: Anesthesia for cleft lip and palate surgery. Update
operated on at 6 versus 12 months of age. Plast Reconstr Surg Anesth 14:27–30, 2002.
102(3):675–679, 1998. 37. Takemura H, Yasumoto K, Toi T, et al: Correlation of cleft type with
23. Cutting CB, Rosenbaum J, Rovati L: The technique of muscle incidence of perioperative respiratory complications in infants with cleft
repair in the cleft soft palate. Operat Tech Plast Reconstr Surg 2:215, lip and palate. Paediatr Anaesth 12:585–588, 2002.
1995. 38. Davis PJ, Hall S, Deshpande JK, et al: Clinical management of special
24. Marsh JL, Grames LM, Holtman B: Intravelar veloplasty; a prospective surgical problems. In Davis PJ, Cladis FP, editors: Smith’s anesthesia for
study. Cleft Palate J 26:46–50, 1989. infants and children, ed 6, St Louis, 1996, Mosby Publishers, pp 599–601.
25. Grames L, Marsh J, Skolnik G, et al: Velopharyngeal outcomes at age 39. Jigjinni V, Kangesu T, Sommerlad BC: Do babies require arm splints after
six for three types of palatoplasty abstract, Indianapolis, 2014, ACPA cleft palate repair? Br J Plast Surg 46(8):681–685, 1993.
meeting. 40. Dissaux C, Grollemund B, Bodin F, et al: Evaluation of 5-year-old
26. Rost Wood L, Sell D, Sommerlad B: Speech and velopharyngeal function children with complete cleft lip and palate: multicenter study. Part 2:
outcome following primary palatoplasty by a single surgeon, Orlando, FL, Functional results. J Craniomaxillofac Surg 44(2):94–103, 2016.
2013, Proceedings Cleft 2013. 41. Xiao WL, Yuan C, Shi B: [Preliminary study of palatal lengthening by
27. Mars M, Plint DA, Houston WJ, et al: The Goslon Yardstick: a new levator veli palatini retropositioning according to Sommerlad
system of assessing dental arch relationships in children with unilateral palatoplasty]. Shanghai Kou Qiang Yi Xue 23(6):718–721, 2014
clefts of the lip and palate. Cleft Palate J 24:314–322, 1987. [Article in Chinese].
28. Shaw WC, Brattström V, Mølsted K, et al: The Eurocleft study: 42. Lu Y, Shi B, Wang Z, et al: [Study on wound healing after Sommerlad
intercenter study of treatment outcome in patients with complete cleft technique repair of isolated cleft palate]. Zhongguo Xiu Fu Chong Jian
lip and palate. Part 5: discussion and conclusions. Cleft Palate Craniofac J Wai Ke Za Zhi 28(7):869–872, 2014 [Article in Chinese].
42(1):93–98, 2005. 43. Lu Y, Shi B, Zheng Q: [A study on lateral incision after palatoplasty with
29. Hathaway R, Daskalogiannakis J, Mercado A, et al: The Americleft study: the levator veli palatini retropositioning according to Sommerlad]. Hua
an inter-center study of treatment outcomes for patients with unilateral Xi Kou Qiang Yi Xue Za Zhi 27(4):425–429, 2009 [Article in Chinese].

You might also like