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Cleft lip and palate 

Cleft lip and palate
Chapter:
Cleft lip and palate
Author(s):

Cyrus Kerawala

and Carrie Newlands

DOI:
10.1093/med/9780199688401.003.0007
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Incidence

  • Commonest facial anomaly in UK. ~1:1000 live births per year.

  • Cleft lip (CL), with or without cleft palate (CP), and isolated clefts of the palate are two distinct conditions.

Cleft lip

  • May or may not be associated with CP.

  • Wide range of potential problems ranging from incomplete unilateral cleft lip through complete unilateral cleft to bilateral cleft involving lip, alveolus, and palate.

  • Accounts for 60% of cleft lip and palate (CLP) cases:

    • 25% cleft lip alone (unilateral or bilateral);

    • 25% unilateral cleft lip and palate (UCLP);

    • 10% bilateral cleft lip and palate (BCLP).

  • Racial variation in incidence:

    • common in Chinese people (~1:500);

    • less common in Afro-Caribbean people (~1:2000).

  • Concordance rate in monozygotic twins ~60%.

  • 15–30% have cleft as part of a syndrome:

    • Van der Woude;

    • hemifacial microsomia;

    • 22q11 micro-deletion known as velocardiofacial syndrome (VCF) or DiGeorge syndrome;

    • EEC—ectrodactyly ectodermal dysplasia and clefting syndrome.

Cleft palate

  • Accounts for other 40% of CLP.

  • Range from complete clefts of hard and soft palate to partial incomplete clefts of the soft palate.

  • No racial variation, but ↑ association with syndromes:

    • Stickler syndrome;

    • 22q11 microdeletion (VCF);

    • trisomy 13;

    • trisomy 18.

Aetiology

Multifactorial

Genetic and environmental factors implicated.

In utero/maternal environmental factors

  • Anti-convulsants, such as phenobarbital and phenytoin (10-fold increase).

  • Alcohol use.

  • Cigarette smoking (2-fold increase).

  • Folic acid deficiency.

  • Corticosteroids (3-fold increase).

  • Hypoxia.

  • Retinoids (vitamin A).

Chromosome abnormalities

  • Methylenetetrahydrofolate reductase (MTHFR) gene on chromosome 1.

  • IRF6 on chromosome 1.

  • TGFA on chromosome 2.

  • MSX1 on chromosome 4.

  • BCL3 and TGF-B on chromosome 10.

  • TGFB3 on chromosome 14.

  • RARA on chromosome 17.

  • Point mutations in genes FOXE1, GL12, MSX2, SK1, SATB2, and SPRY2 may contribute to 6% of isolated CLP cases.

Genetic and environment interaction may increase risk

  • 6-fold ↑ risk if mother smokes cigarettes and has TGFA genotype on chromosome 2.

  • A MSX1 defect on chromosome 4 increases the risk for mothers who drink alcohol or smoke cigarettes.

  • Mothers are more susceptible to folate deficiency if they have MTHFR deficiency.

Diagnosis

Ultrasound

  • Possible to diagnose clefting as early as 12 weeks of gestation by transvaginal ultrasound.

  • Diagnosis is made by obtaining coronal and frontal planes of fetal face.

  • Full anatomical survey is indicated if an oral cleft is diagnosed to rule out other associated syndromes.

  • Detection rate dependent on a number of factors:

    • experience of operator;

    • gestational age at the time of the study;

    • indication for the study;

    • technology used.

  • Cleft Lip and Palate Association (CLAPA) survey (2006) reported that 45% of respondents had an antenatal diagnosis compared with only 15% in 1996.

  • 3D USS has been shown to increase diagnostic accuracy especially using the reverse face or ‘flip face’ view.

  • Ultrasound is more accurate in diagnosing isolated CL and CLP (67–93%), but is limited in making the diagnosis of isolated CP (7–22%).

  • Detection rates improve markedly after 20 weeks of gestation; therefore, patients with ↑ risk of clefting and a normal early ultrasound should have a repeat scan after 20 weeks.

  • Clefts may be classified on ultrasound into five types:

    • type 1—isolated cleft lip;

    • type 2—unilateral cleft of the lip and palate;

    • type 3—bilateral cleft of the lip and palate;

    • type 4—median cleft lip;

    • type 5—clefts associated with amniotic banding of limb-body-wall complex.

  • The type of the cleft has been correlated with chromosomal abnormalities, structural anomalies, and fetal death.

  • The more severe the cleft the greater the risk is of associated chromosomal abnormalities, structural anomalies, and fetal death.

  • Once diagnosis has been made antenatally parents should immediately be referred to the local cleft team for appropriate counselling and support.

  • False positive diagnoses do occur and patients should be informed accordingly.

At birth

  • In those cases where an antenatal diagnosis has not been made, the aim is to diagnose all clefts at birth and for parents to be contacted by a member of the local cleft team within 24h.

  • A recent CLAPA survey reported that 45.3% of clefts were diagnosed antenatally, 41% at birth, 4.4% within 24h, and a further 9.3% after 24h with one patient only diagnosed after 12 months.

  • Designated cleft nurse specialist should be available to give further counselling and support to the family, where necessary including advice by telephone and home visits.

  • Appropriate psychological support should be provided (see Cleft lip and palate Psychology, p. [link]).

  • Specialist feeding and nursing advice should be implemented at the time of birth.

  • The child and parent should be seen in a multidisciplinary outpatient clinic soon after birth by team members who will be involved in their long-term care.

  • Details of surgery should be discussed at this outpatient visit and dates for surgery provided where appropriate.

  • The parents should be taken to see the ward to familiarize them with the hospital facilities.

Genetic advice

  • All parents given the option of genetic counselling at birth (particularly important if syndromic diagnosis is suspected).

  • Traditionally risk has been given as a percentage:

    • risk of having a second child with cleft lip and/or palate—4%;

    • risk of having a third child—10%;

    • risk if a member of the family has CLP to a first-degree relative—2%;

    • risk if a member of the family has CLP to a second-degree relative—0.7%;

    • risk if a member of the family has CLP third-degree relative—0.3%.

  • Norwegian study has shown:

    • relative risk of cleft recurrence in first-degree relatives is 32 for any cleft lip and 56 for cleft palate alone;

    • risk of clefts in children of affected mothers is similar to the risk of clefts in children of affected fathers;

    • the parent–offspring risk is similar to the sibling–sibling risk;

    • association between cleft severity and patient’s sex;

    • does not affect the risk of having a cleft.

  • Genetic counselling recommended to young adults with cleft lip and/or palate.

Management of lip

Principles

  • Clefts of the lip cause cosmetic and functional problems.

  • Functional problems include impairment:

    • in the production of bilabial sounds (pa, pi);

    • of maxillary growth because of scar constriction.

  • Babies with isolated cleft lip can usually feed normally, although they may have difficulty creating an adequate lip seal.

Anatomical defects in UCLP include:

  • Discontinuity of skin, muscle, and oral mucosa of the upper lip on cleft side.

  • Vertical soft tissue deficiency on medial aspect of the cleft.

  • Abnormal muscle insertions into nasal spine and alar base.

  • Alveolar cleft in region of the canine tooth.

  • Defect in primary palate anterior to the incisive foramen.

  • Rotation of septum, columella, and nasal spine away from the cleft.

  • Separation of domes of the alar cartilages at the nasal tip and kinking of the lateral crus on the cleft side.

  • Dislocation of lower and upper lateral cartilages on the cleft side.

  • Displacement of alar base in all three planes of space.

  • Displacement and flattening of the nasal bone on the cleft side.

Anatomical defects in BCLP include:

  • Discontinuity of skin, muscle, and oral mucosa of the upper lip bilaterally.

  • Bilateral defect of alveolus and anterior palate.

  • Central segment consisting of prolabium and pre-maxilla with short columella.

  • Lack of orbicularis oris continuity.

  • Collapse of lateral palatal segments behind pre-maxilla.

  • Alar domes and middle crura are splayed, caudally rotated (bucket handle), and subluxed from their normal anatomic position overlying upper lateral cartilages.

Surgical options

  • Lip repair is usually carried out at 3–5 months in UK.

  • Neonatal lip repair, while still practised in some units abroad, has fallen into disrepute in the UK.

  • Many different protocols for lip and palate repair worldwide.

  • The following protocols and timings reflect practice in most UK cleft centres.

Unilateral cleft lip and palate

  • The lip, nose, and anterior palate are repaired at the first surgery at 3–5 months of age in healthy children.

  • Anterior palate is repaired with an unlined turnover flap from the vomer (popularized by surgeons in Oslo, Norway) (Fig. 7.1).

Fig. 7.1 Vomer flap for hard palate repair in a right unilateral cleft of the lip and palate.

Fig. 7.1 Vomer flap for hard palate repair in a right unilateral cleft of the lip and palate.

Important steps in lip repair include:

  • Skin incision design.

  • Subperiosteal mobilization.

  • Muscle repair.

  • Nasal floor restoration.

  • Alar base positioning and primary nasal dissection.

Design of skin incisions important to accomplish:

  • Symmetry.

  • Equal lip length.

  • Natural appearance of the cupid’s bow.

  • Inconspicuous scarring and restoration of the nostril floor.

Many different techniques have been described for lip repair:

  • Straight-line techniques (Rose–Thompson).

  • Rotation advancement techniques (Millard, Delaire).

  • Lower lip Z-plasties (Tennison–Randall).

  • Combinations of these.

Individual techniques have advantages and disadvantages:

  • Millard (Fig. 7.2):

    • scar mimics philtral column on cleft side;

    • reconstructs nasal sill;

    • allows some leniency in surgical technique (cut as you go);

    • may result in short lip on cleft side;

    • scar crosses philtral column at nasal base.

  • Tennison (Fig. 7.3):

    • useful for repairing a wide cleft;

    • may result in a long lip;

    • scar crosses philtral column inferiorly.

Many now use a combination of these two techniques to take advantage of the strengths of each technique and discard the weaknesses (Fig. 7.4).

  • Radical subperiosteal mobilization of tissues of the cleft side (which includes incision through inelastic periosteal layer) to orbital rim, zygomatic process, and pyriform rim. It is important to ensure that muscle and skin is closed without undue tension; this also aids in accurate repositioning of the alar base.

  • Dissection and repair of orbicularis oris muscle.

  • Skin of the lip is dissected off the muscle medially and laterally.

  • Muscle repaired with resorbable or non-resorbable sutures.

  • Primary bone grafting or gingivoperiosteoplasty (GPP; at the time of lip repair) is not widely practised in UK.

  • Primary bone grafting is thought to impair growth.

  • GPP requires pre-surgical orthopaedics to bring the alveolar segments into close apposition.

  • Pre-surgical orthopaedics for unilateral cleft lip and palate patients is not routinely carried out in UK.

  • Repair of the nose (not done by all in UK) is carried out by:

    • separating skin from the alar cartilage on the cleft side (as described by McComb and Andel);

    • approached both from lateral and medial aspects of the alar cartilage through the skin incisions already made for the lip repair;

    • repositioned structures of the nose are maintained using either buried slow-resorbing sutures (e.g. PDS) or external tie-over splints.

Fig. 7.4 Combination of Millard and Tennison repair.

Fig. 7.4 Combination of Millard and Tennison repair.

Bilateral cleft lip and palate

  • Very little conformity (2008 surgical interest group meeting) in UK about:

    • use of pre-surgical orthopaedics;

    • technique for repair;

    • timing of repair.

  • Formal surgical repair is often preceded by pre-surgical orthopaedics to reposition the premaxilla posteriorly and align the lateral alveolar segments.

Principles of repair

  • Symmetry should be maintained meaning that repair of both sides should occur simultaneously (asynchronous lip repair is practised by three units in UK and Ireland).

  • Establish primary muscle continuity (if undue muscle tension exists at time of primary repair then final muscle repair is deferred for about 9 months to a year).

  • Median tubercle may be reconstructed from either the prolabium (if patient has a well-developed white roll on the prolabium) or from the lateral lip elements (if there is a poorly developed white roll on the prolabium).

  • Reconstruction of the labial sulcus from the prolabial mucosa and lateral lip elements (some rely on muscle action to create a labial sulcus and do not formally reconstruct the sulcus).

  • Hard palate repair varies widely and includes:

    • complete closure with a vomer flap on both sides;

    • complete closure on one side with a full vomer flap and partial repair of the opposite side with a vomer flap raised up to the pre-vomerine suture;

    • complete closure on one side only leaving the contralateral side open.

  • Variation in hard palate repair exists because raising a full bilateral vomerine flap may compromise blood supply to the pre-maxilla, but raising the flap to the pre-vomerine suture and no further is thought to maintain the blood supply to the pre-maxilla. If only one side is repaired at the first surgery then the child is brought back after approximately 6 weeks to close the contralateral side with a vomer flap.

  • The nose is repaired as previously described for unilateral patients except both sides of the nose are dissected and primary positioning of the alar cartilages is carried out to construct the nasal tip and columella.

Incomplete lip

  • Principles are the same as for complete cases and a functional repair should always be carried out.

  • It is also important to address the nose in incomplete cases as there is usually a lip/nose deformity.

Management of palate

Principles

  • Functional palatal repair is important for speech and may be important for Eustachian tube function.

  • Timing of palatal repair is controversial:

    • early closure prior to 1 year has been shown to benefit speech;

    • delayed hard palate closure has only shown consistently improved growth when delayed into adolescence, but this results in unacceptably poor speech.

  • Common practice in UK is to close the soft palate in cleft lip and palate patients and the entire palate in isolated cleft palates between 6–9 months.

  • Underlying deformity includes (Fig. 7.5):

    • narrow and vertically displaced palatal shelves;

    • abnormal insertion of palatal muscles;

    • abnormal muscle in syndromic patients.

Fig. 7.5 Palatal muscles—normal vs cleft side.

Fig. 7.5 Palatal muscles—normal vs cleft side.

Surgical options

  • Hard palate in complete cleft lip and palate patients is repaired in the UK using a vomer flap (see Cleft lip and palate Management of lip, p. [link] and Fig. 7.1).

  • Many different techniques have been used to close the palate including:

    • Von Langenbeck;

    • Widmaier;

    • Veau–Wardill–Kilner;

    • Bardach two-flap technique with intravelar veloplasty;

    • Furlow double opposing Z-plasty;

    • Sommerlad radical micro-dissection.

  • Von Langenbeck releasing incisions are still used in wide clefts.

  • Veau–Wardill–Kilner push-back techniques still widely used in some countries, but abandoned in UK because of:

    • unfavourable mid-facial growth;

    • compromised dental arch form;

    • difficult to manage fistulae;

    • excessive palatal scarring;

    • poor speech outcome.

  • Sommerlad repair of the palate with microscopic magnification recreation of the levator veli palatini sling is widely practised in UK. Procedure involves:

    • incision along cleft margin at junction of oral and nasal mucosa;

    • mobilization of mucoperiosteum of hard palate in subperiosteal plane;

    • separation of oral mucosa with minor salivary glands from muscles and nasal mucosa;

    • division of oral insertion of tensor veli palatini tendon;

    • suturing nasal layer with soft palate muscles still attached;

    • separation of tensor veli palatini and palatopharyngeus from the back of the hard palate;

    • dissection of levator veli palatini from the nasal mucosa keeping the nasal mucosa intact;

    • alignment of the levators transversely and suture of the levators together in the midline to reconstitute the levator sling;

    • closure of oral layer using resorbable sutures;

    • lateral releasing incisions in the oral layer (Von Langenbeck) are used to reduce tension on the oral repair in <15% of cases (usually only) in extremely wide clefts;

    • 5% of patients repaired using the Sommerlad technique have required secondary speech surgery.

  • The Bardach double flap:

    • widely practised in USA;

    • hard palate is raised as two flaps subperiosteally giving access to soft palate musculature, which is repaired with an intravelar veloplasy;

    • secondary speech surgery needed in >20% of patients.

  • Furlow double-opposing Z-plasty:

    • widely practised in USA;

    • lengthens the palate and re-aligns levator veli palatini muscles;

    • difficult in wide clefts;

    • asymmetrical repair;

    • normal or near normal speech reported in >85% of patients.

Management of alveolus

Aims of alveolar bone graft

  • Stabilize alveolar arch.

  • Restore arch integrity.

  • Allow teeth to erupt into optimal position.

  • Permit orthodontic alignment of arch.

  • Simultaneous oronasal fistula repair.

  • Creation of a platform for prosthetic replacement, e.g. implants.

  • Optimize maxillary surgery.

  • Create support for the nose (alar base) and lip.

Assessment

  • OPT and occlusal radiograph.

  • Periapical radiograph if necessary.

  • CBCT scanning (becoming more popular).

  • Important to decide whether tooth extractions need to be done. At the time or at least 3 months before to allow attached gingiva to heal.

  • Pre-surgical orthodontic treatment to align alveolar segments and optimize position of teeth adjacent to the cleft.

Principles of surgery

  • Pioneered by Boyne and Sands.

  • Mucoperiosteal flaps are raised on both sides of the cleft on the buccal and palatal sides (Fig. 7.6).

  • Reconstruction of nasal layer and create a nasal seal.

  • Nasal dissection superiorly up to the piriform rim and nasal floor.

  • Some place collagen membrane below the nasal layer to improve the nasal seal.

  • Bone is packed into the space between the nasal layer and oral layer bridging the alveolar segments, and should extend posteriorly into the palatal cleft, superiorly to the nasal floor, and laterally to the paranasal region.

  • Defect should be over-filled as resorption of up to 30% may occur.

  • Oral closure is effected by advancing the lateral buccal mucoperiosteal flap medially to create an oral seal.

  • Lateral buccal flap advancement will leave exposed alveolar bone laterally, which will heal by secondary intention without complication.

  • Post-surgical orthodontics follows to provide stability, while bone graft is incorporated and heals.

Fig. 7.6 Alveolar bone graft—flaps raised and nasal layer closed.

Fig. 7.6 Alveolar bone graft—flaps raised and nasal layer closed.

Outcome assessment of alveolar bone

Outcome assessment of grafting is based on:

  • Success of fistula closure.

  • Eruption of canine and bony support for adjacent teeth.

  • Objective radiographic measurement using panoramic and occlusal radiographs to assess bone height, and more recently conventional or CBCT to assess bone height and width.

  • Common rating scales include the Bergland, Kindelan, and Chelsea (Witherow) scales.

  • Bergland scale compares the level of the inter-dental septum on the grafted side to the normal side and the aim is to achieve grade I or II:

    • grade I—normal height;

    • grade II—>75% normal height;

    • grade III—50–75% normal height;

    • grade IV—< 50%.

Timing

Primary bone grafting (at time of lip repair 3–6 months of age)

  • Began in the 1950s.

  • Carried out at same time as lip repair with wide dissection and bone (split rib) used to bridge gap.

  • Abandoned by all in original form as shown to severely inhibit maxillary growth.

  • Primary grafting still part of the treatment protocol in some units in the USA, but in a modified form.

Gingivoperiosteoplasty

  • Increasing support in recent times in some US and European centres.

  • Requires pre-surgical orthopaedics to bring alveolar segments into near contact.

  • Gingival flaps adjacent to the cleft are raised, taking care not to damage underlying tooth germs, and sutured together.

  • Evidence that GPP decreases the need for alveolar bone grafting later.

  • If alveolar bone grafting is needed later, there is limited evidence to suggest that those patients initially treated with GPP have a better outcome.

  • Others have shown that secondary bone grafting is superior to GPP and counsel against GPP.

  • GPP still not universally supported because of:

    • lack of long-term maxillary growth studies;

    • lack of consistent results between centres;

    • burden of care for parents.

Early secondary (2–5 years)

  • May improve periodontal support for central incisor on the cleft side and facilitate lateral incisor eruption if present.

  • Early post-grafting orthodontics to correct incisor crossbite at this age may improve horizontal maxillary hypoplasia.

  • Disadvantages include orthodontic compliance and prolonged orthodontic treatment.

  • Not widely practised as there is a lack of long-term maxillary growth outcome data.

Secondary (6–13 years): in the mixed dentition

  • Most commonly applied technique in UK.

  • Timing determined by development of the canine tooth on the cleft side.

  • Grafting is carried out when the canine root is half to two-thirds formed.

  • Allows eruption of the canine into the newly grafted zone.

  • Established technique with long-term growth and dental outcomes.

Late (>13 years): in the permanent dentition

  • This results in poor outcome with:

    • compromised periodontium;

    • ↑ tooth loss adjacent to cleft;

    • more need for prosthodontic rehabilitation;

    • higher fistula rate;

    • poor alar base support.

  • Not recommended in the UK for planned care.

At time of Le Fort I maxillary osteotomy

Not recommended as maxillary segments are not in continuity and it is easier to complete osteotomy if the maxilla is in one piece.

Donor site (for secondary alveolar bone grafting)

  • Grafting material for alveolar bone grafting may be autogenous, xenogenic, allogenic, or alloplastic.

  • Particulate bone grafts are superior to block cortical or corticocancellous grafts because they are more readily incorporated into the alveolus with the capacity for post-operative remodelling.

  • Several autogenous donor sites have been used for alveolar bone grafting including iliac crest, rib, calvarium, tibia, and mandibular symphysis.

Iliac crest

  • Regarded as the gold standard for alveolar bone grafting.

  • Advantages:

    • rich in osteogenic cells;

    • rapidly transformed into alveolar bone;

    • substantial amounts of cancellous bone can be easily obtained;

    • two-team operating shortens procedure time;

    • excellent long-term outcomes reported.

  • Disadvantages relate to donor site morbidity:

    • prolonged post-operative pain;

    • gait disturbances;

    • long hospital stay;

    • unsightly scars;

    • sensory disturbance (neuropraxia of lateral cutaneous nerve of thigh).

  • Immediate post-operative pain control using infusion techniques and minimally invasive harvesting techniques with trephines have reduced morbidity significantly.

Calvarial bone

  • Advantages:

    • minimal scarring;

    • minimal post-operative pain;

    • mesenchymal bone.

  • Disadvantages:

    • cortical bone with minimal cancellous bone;

    • small volumes;

    • dural tears and infections;

    • osteogenic potential of graft affected by technique used;

    • single-team operating.

  • Results not as good as iliac crest.

Tibia

  • Advantages:

    • ease of harvest;

    • minimal morbidity (pain, infections);

    • good long-term outcomes;

    • two-team operating.

  • Disadvantages:

    • scarring;

    • limited volume of bone;

    • tibial plateau fractures in up to 3% of cases.

Mandibular symphysis

  • Method of choice in some European centres.

  • Advantages:

    • mesenchymal bone;

    • no visible scar;

    • minimal pain and shortened hospital stay;

    • satisfactory long-term outcomes.

  • Disadvantages:

    • single-team operating;

    • technically difficult with significant learning curve;

    • damage to the mandibular permanent tooth germs with subsequent tooth loss;

    • limited amount of bone.

Rib

  • Now abandoned as:

    • significant resorption.

    • poor long-term outcome.

Bone substitutes

  • Advantages: no donor site morbidity.

  • Disadvantages:

    • slower graft revascularization;

    • long-term results equivocal;

    • unpredictable bone formation;

    • unpredictable resorption.

The use of bone substitutes is still not recommended in children.

Bone morphogenic proteins

  • Have shown promise in a number of animal models.

  • May replace autogenous grafting in the future, but a suitable carrier in humans still to be found.

Revision procedures

Lip

  • Residual imperfections and imbalances may occur following primary repair as a result of:

    • poor alignment at the time of surgery;

    • distortion during growth;

    • scar contracture;

    • closure under tension in wide clefts;

    • poor muscle reconstruction.

  • Lip distortions should be evaluated together with the nose, bony skeleton, and dentition.

  • Underlying skeletal imbalance will be reflected in the soft tissue drape and should be considered when planning lip revision (especially in the older child and adolescent).

  • Lip revision is indicated for:

    • lip length discrepancies;

    • vermillion deformities;

    • mucosal excess or deficiency;

    • inadequate muscle reconstruction;

    • poor cutaneous scars.

  • Each deformity should be considered on its own merits.

  • Mucosal and vermillion deformities can often be treated using:

    • local flaps;

    • Z-plasties;

    • V–Y advancements.

  • Lip length discrepancies, poor cutaneous scarring, and inadequate muscle reconstruction usually requires formal revision of the entire lip rather than an attempt to improve the deformities with limited intervention.

  • Timing of lip revision may vary and the decision to treat is taken together with parents and child (older children and adolescents will often make their own decisions).

  • Obvious deformity should, where possible, be treated before the child starts school to facilitate integration and avoid stigmatization.

  • In uncertainty or parental conflict a clinical psychologist should be involved.

Palate

Revisional surgery of the palate is indicated for two main reasons:

  • Velopharyngeal incompetence (VPI) secondary to physical inadequacy of the velopharyngeal mechanism.

  • Repair of oronasal fistulae.

Surgery for VPI

Philosophies vary from unit to unit regarding optimizing palatal function as identified by video fluoroscopy vs obturating the velopharyngeal gap identified on naso-endoscopy.

Assessment

  • Speech.

  • Clinical examination.

  • Lateral videofluoroscopy.

  • Nasendoscopy.

Surgical options based on fluoroscopy include:

  • Palatal re-repair to posteriorly reposition levator palatini in the velum and utilize full length of the palate.

  • Oral Z-plasy to gain length together with levator palatini repositioning surgery (Furlow’s reverse Z-plasty).

  • Hyne’s pharyngoplasty.

  • Coleman fat transfer.

  • Buccal interpositional flaps.

  • Z-plasty of the oral layer.

  • Speech prosthesis.

  • Superiorly-based pharyngeal flap.

In summary, the aim of this philosophy of treatment is to provide an optimally-functioning palate in the first instance and to address the anatomical abnormalities that exist thereafter, without creating permanent airway obstruction.

Nasendoscopic diagnosis is useful:

  • In those with a coronal closing pattern and a velopharyngeal gap laterally—the aim of surgery is close the lateral gaps (most commonly carried out with an Orticochea or Jackson sphincter pharyngoplasty).

  • In those with a lateral ‘sphincteric’ closing pattern with a gap in the midline—the aim of surgery is to close the midline gap with a midline pharyngeal flap pedicled either superiorly or inferiorly and tailored to the size of the gap.

These surgical options will tether the velum, narrow the velopharyngeal airway, and create a permanent obstruction with potential obstructive sequelae.

Speech in cleft patients has a tendency to deteriorate with age because of the relative increase in pharyngeal size due to growth, adenoid lympoid tissue involution, and pharyngeal shape change. This means long-term follow-up into adulthood.

Surgery for oro-nasal fistulae

  • Common sequelae of cleft palate repair.

  • May represent a failure in technique or a problem in wound healing which is often a result of excessive tension.

  • Reported incidence varies widely (0–34%).

  • Incidence is related to the extent of clefting and surgical technique, but not age at the time of palatoplasty or gender.

  • Symptoms associated with oro-nasal fistulae include:

    • audible nasal air escape during speech;

    • hypernasality;

    • food regurgitation into nose.

  • Not all fistulae are symptomatic and therefore not all require intervention.

  • The decision to treat fistulae is determined on an individual basis, and is guided by speech assessment and patient morbidity.

  • Successful fistula repair improves symptoms directly associated with the fistula and velopharyngeal function, therefore existing symptomatic fistula should be repaired before embarking on formal velopharyngeal management.

Fistula repair is often technically challenging because of:

  • Excessive scarring.

  • Lack of virgin local tissue.

  • Ease of access.

No single technique is consistently satisfactory and reported rate of successful closure varies from 33–90%. Many different techniques for closure have been described. Broadly divided into:

  • Local soft tissue flaps (turnover flaps, rotation flaps, island flaps).

  • Local soft tissue flaps with inter-positional grafts (conchal cartilage, alloplastic materials such as PDS or collagen membranes).

  • Regional intra-oral (tongue flaps, buccal flaps, facial artery myomucosal flaps).

  • Extra-oral (naso-labial).

  • Free tissue transfer (radial forearm, fibula flaps for composite reconstruction).

The technique chosen is based upon:

  • Position and size of the fistula.

  • Nature and number of previous attempted repairs.

The basic fundamental principle in repairing oro-nasal fistulae is to repair the defect in layers (especially ensuring adequate repair of the nasal layer). The addition of conchal cartilage or alloplastic interpositional grafting has improved success rates.

Management of nose

Primary

  • Primary repair carried out at the time as primary lip repair.

  • Historically it was believed that primary surgery to the nose would compromise nasal growth and was therefore discouraged.

  • Long-term outcomes of a consecutive series of unilateral and bilateral cases showed that primary rhinoplasty does not compromise nasal growth and paved the way for a more aggressive approach to the nose at the time of primary lip repair.

  • Various techniques to maintain cartilage repositioning following surgery include:

    • external and internal suspensory sutures;

    • temporary tie-over splints;

    • longer-term internal and external silicone splints.

Secondary

The nose, despite improved results following primary rhinoplasty at the time of lip repair, often requires adjustment on more than one occasion.

Early secondary rhinoplasty

  • Age 3 to adolescence, but often prior to school attendance.

  • Carried out to improve appearance of the nose to avoid peer ridicule and specifically to address:

    • obvious deformities of the tip, lower lateral cartilage, and alar base in UCLP patients;

    • shortened columella, tip deformities, and widened alar bases in BCLP patients.

  • Access to the nose may be via a rim incision in milder cases or more commonly through a trans-columella open tip approach.

Complete septorhinoplasty

  • Vomer osteotomies and grafting where necessary.

  • Delayed until skeletal maturation is complete.

  • Should also only be carried out after orthognathic surgery has been undertaken.

  • Cleft nose deformity complex—usual multiple interventions increases the difficulty of the surgery.

  • Unless only very minor adjustments are indicated, complete cleft septorhinoplasties should be carried out as open procedures.

  • Important to try to address the patient’s concerns and wishes as long as these are realistic.

  • Patients benefit from at least one session with the team psychologist to:

    • help them articulate their concerns;

    • gauge their expectations;

    • prepare them for surgery.

Orthognathic surgery

  • Need for orthognathic surgery varies according to cleft type.

  • Lack of maxillary growth may be inherent, but is probably a consequence of soft tissue scarring following primary surgery.

  • Improved primary surgical technique means that maxillary growth is compromised less.

  • Commonly the maxilla is hypoplastic in all three planes of space resulting in:

    • skeletal Class III jaw relationship;

    • maxillary narrowing (maxillary transverse discrepancy);

    • ↓ facial height.

  • Bi-maxillary surgery is commonly indicated, and may include all or a combination of the following:

    • maxillary advancement;

    • maxillary down-grafting;

    • segmental maxillary surgery;

    • mandibular setback;

    • genioplasty.

Traditional orthognathic surgery

Challenging because:

  • Large movements often required.

  • Extensive scarring from previous surgery makes mobilization and retention of final position difficult.

  • Presence of a pharyngeal flap.

  • Abnormal bony anatomy with potential to compromise the blood supply to the premaxilla in bilateral cleft cases.

  • ↑ tendency to relapse.

  • Despite all of these points relapse can be overcome by:

    • aggressive mobilization;

    • adjunctive bone grafting;

    • rigid fixation.

  • There is recent evidence to show that rate of relapse in traditional orthognathic surgery is comparable to distraction osteogenesis regardless of the distance moved.

  • Traditional orthognathic surgery has the following advantages:

    • more cost-effective than distraction;

    • patient only needs one procedure;

    • patient does not have the burden of prolonged treatment with unsightly distrators.

Distraction osteogenesis

  • Provides alternative to traditional orthognathic surgery especially in cases that require large movements.

  • Use external or internal distractors.

  • Advance 1mm/day after carrying out Le Fort 1 cuts.

  • Advantages:

    • in some series evidence that there may be less relapse especially after large advancements when distraction is used (not replicated in all studies);

    • may affect speech less;

    • able to adjust if speech becomes compromised.

  • Disadvantages:

    • need to wear unsightly frame for 6–8 weeks if using external distraction with negative psychological consequences;

    • expensive equipment;

    • extensive work-up if internal distraction is planned;

    • need for more than one surgical procedure.

Effect of orthognathic surgery/distraction on speech

  • It is important to be aware that maxillary advancement may compromise VPI function and speech.

  • Pre-operative speech assessment will give an indication of the risk of developing VPI, but there is no method that will reliably predict which patients will develop it. This is partly because:

    • it is not possible to adequately assess palatal functional reserve;

    • development of VPI after maxillary advancement is not always related to the distance moved.

  • Pre-orthognathic/distraction speech investigations include:

    • speech and language therapy assessment;

    • lateral videofluoroscopy;

    • nasendoscopy.

  • The chances of VPI developing can be minimized by:

    • careful planning and pre-operative assessment;

    • bi-maxillary surgery to minimize maxillary movement;

    • possibly by utilizing distraction techniques.

Speech

  • Development of speech is lengthy and complex, and usually proceeds through a number of stages.

  • Process is dependent on a number of factors, including the ability to hear and the presence of an intact velopharyngeal mechanism. Cleft patients may be compromised on both of these fronts.

  • The palate separates the oral and nasal cavity with the soft palate permitting two-way air flow at the velopharyngeal orifice.

  • In the normal patient, the soft palate is able to close off the nasal airway during speech. This enables the voice to be shaped by the mouth or oropharynx, and is therefore critical for the production of oral sounds.

  • English speakers need to produce oral pressures to make all sounds apart from ‘m’, ‘n’, and ‘ng’.

  • Elevation of the soft palate during swallowing prevents food/fluids passing into the naso-pharynx.

  • The presence of a cleft palate results in deviant and restricted sound development, and influences pre-speech development in cleft children. This continues to have an influence on speech development post-palate repair even in those children with competent velopharyngeal mechanisms.

  • Early babble patterns are indicative of how later speech may develop and this has influenced the timing of palatal repair.

  • Speech therapists encourage surgeons to repair the palate at least by the age of 1 year and in many cases earlier.

  • In the normal palate the levator veli palatini muscles run transversely across the central 40–50% of the palate and on contraction the palate is lifted to close the velopharynx.

  • In CP and submucous CP the levator veli palatini muscle insertions are abnormal:

    • in CP they insert into the side of the cleft;

    • in submucous CP they are discontinuous and variably anteriorly displaced.

  • The consequence of poor palatal function is poor speech, which influences social integration.

  • The aim of palatal surgery in general and of soft palate surgery in particular is to provide the patient with an adequately functioning velopharyngeal sphincter.

  • VPI in cleft patients may result from:

    • poor palatal function post-surgical repair;

    • undiagnosed submucous clefts of the palate;

    • palatal-pharyngeal disproportion;

    • neuromuscular dysfunction.

  • Characteristics of cleft speech maybe divided into:

    • resonance/airflow errors;

    • hypernasality;

    • hyponasality;

    • mixed resonance;

    • nasal emission;

    • nasal turbulence;

    • nasal facial grimace.

  • Cleft-type speech characteristics:

    • palatal realization;

    • lateral realization;

    • non-oral articulations;

    • nasal realizations.

  • These speech problems are related to the abnormal oral and/or nasal structure associated with cleft patients including:

    • VPI;

    • nasal obstruction;

    • oro-nasal fistulae;

    • dental malocclusions;

    • skeletal abnormalities.

  • Cleft speech is therefore complex, and requires assessment and treatment by speech and language therapists specifically trained in and dedicated to cleft patients.

Speech assessment

The role of the speech and language therapist is to:

  • Assess speech.

  • Determine the relationship between speech and structure.

  • Devise appropriate treatment strategies.

Speech assessment can be divided into:

  • Perceptual listening.

  • Clinical examination.

  • Use of adjunctive investigations.

Role of speech therapy

Speech therapy can be divided into:

  • Articulation therapy:

    • the bulk of speech therapy focuses on articulation therapy, which has been shown to result in overall speech improvement;

    • focus of treatment is to modify place of articulation to more anterior positions consistent with normal speech;

    • even in patients with hypernasality improvement in placement of consonant articulation will have a positive effect on intelligibility;

    • patient outcome is improved if therapy is offered on a frequent basis (more than weekly).

  • Resonance therapy: there is little support in the literature for therapy to modify hypernasality, although more recently there are some who believe that therapy might reduce it, but the evidence for treatment success is still awaited.

  • Behavioural therapy:

    • feedback therapy may be successful in certain selected cases and has the advantage that the patient, and the therapist are able to monitor the outcome of the intervention and in so doing change speech behaviour;

    • disadvantages of biofeedback include the need for expensive equipment, as well as patient dedication and compliance.

  • Voice therapy: the abnormalities of voice seen in cleft patients are often a response to velopharyngeal dysfunction and these needs to be addressed accordingly.

Hearing

  • Normal hearing is necessary for speech development.

  • Eustachian tube (EUT) dysfunction with permanent hearing loss is common in CP.

  • Impaired active dilatation and EUT dysfunction has been found in 100% of non-operated CP patients.

  • The pathophysiology of EUT dysfunction is related to:

    • muscle pull;

    • oro-nasal reflux, which causes mucosal irritation, oedema, and tubal occlusion (all of which contribute to hearing loss).

  • Functional palatal surgery improves velopharyngeal closure preventing reflux and allows tubal oedema to resolve.

  • Air insufflation, viewed as an index of EUT function, has shown improvement in passive opening of the EUT following CP repair, but no difference in active opening due to muscular contraction.

  • A high percentage of patients with CP will develop otitis media with effusion (OME) despite functional palatal surgery and all of these patients therefore require careful ENT and audiological surveillance throughout their childhood within the MDT setting.

  • Some teams opt for aggressive short-term ventilation based on the finding that OME is almost universal in the CP population.

  • Others will only place ventilation tubes if there is OME with significant hearing loss (>55dB HL on auditory-evoked brain stem response in the better ear).

  • The NICE review on the current evidence on OME in children with CP concluded that there is a lack of evidence on the optimal treatment.

  • Treatment should, therefore, be based on the needs of the individual child.

  • Insertion of ventilation tubes at the time of primary lip or palate closure should only be carried out after careful otological and audiological assessment, and not inserted routinely.

  • There is some case-review evidence for the effectiveness of hearing aids in these children and the alternative of early aiding should therefore be considered.

  • NICE recommend the insertion of ventilation tubes as an alternative to hearing aids in children with CP who have persistent bilateral OME, and a hearing level in the better ear of 25–30dB HL or worse confirmed over 3 months.

  • Some clinicians involved in the management of CP are concerned that the hearing level of 25–30dB HL recommended by NICE may precipitate an increase in the number of children undergoing ventilation tube insertion with their attendant complications.

Orthodontics

The role of the orthodontist is central to the care of patients with cleft lip and palate characterized by involvement throughout their development, beginning soon after birth and continuing through to the end of growth.

  • In the early stages the orthodontist may be involved in pre-surgical orthopaedics.

  • In the mixed dentition the orthodontist prepares the patient for secondary alveolar bone grafting.

  • In the adolescent the orthodontist will carry out definitive orthodontic treatment separately or as part of a combined surgical orthodontic (orthognathic) treatment plan.

Pre-lip surgery

Orthodontic involvement may include:

  • Primary impressions.

  • Fabrication of:

    • feeding plates (now felt to be ineffectual);

    • plates to protect the vomer from ulceration;

    • plates to prevent tongue displacement into the palatal cleft in patients with Pierre Robin sequence.

  • Pre-surgical orthopaedics (PSO) with or without naso-alveolar moulding (NAM).

Pre-alveolar bone graft

Primary dentition

Orthodontics in the primary dentition is rare and is limited to treatment of anterior crossbites.

Mixed dentition

  • Orthodontics in the mixed dentition is mainly carried out to prepare the maxillary arches for bone grafting.

  • The timing of therapy is based upon the development of the canine tooth in most units in the UK with the aim being to carry out grafting when the canine root is half to two-thirds formed, as stability of the bone graft is dependent on the presence of teeth within the area of the graft.

  • Pre-alveolar bone grafting orthodontics is carried out to:

    • approximate alveolar segments;

    • move adjacent teeth into ideal positions (to enable adequate soft tissue coverage of the graft).

  • Preparation for alveolar bone grafting in UCLP and BCLP cases may involve some or all of the following depending on individual need:

    • palatal expansion;

    • correction of anterior crossbite;

    • tipping of teeth;

    • removal of supernumary teeth that may compromise soft tissue closure.

  • BCLP cases are complicated by the position of the pre-maxilla, which may need one or a combination of the following:

    • retraction;

    • intrusion;

    • protrusion;

    • osteotomy at the time of grafting to aid positioning.

  • It is important to approximate the pre-maxilla and lateral alveolar segments to facilitate soft tissue closure.

  • Post-operatively orthodontic appliances are left in situ for stability until healing of the bone graft has occurred.

Adolescent

  • Therapy is usually accomplished with fixed appliances and treatment time is similar to non-cleft cases.

  • Cleft patients, however, pose unique problems, including:

    • missing and malformed teeth, especially lateral incisors;

    • unusual dental positions such as canine impactions;

    • arch collapse with crowding;

    • anterior and posterior crossbites;

    • associated skeletal abnormalities;

    • ↓ facial height;

    • maxillary hypoplasia in all three planes of space (AP, vertical, and transverse).

  • Where there is an underlying associated skeletal deformity the need for a combined orthodontic-orthognathic approach should be discussed early on in treatment.

  • Attempts should be made to retain the lateral incisor if present.

  • If the lateral incisor is missing then a decision needs to be made regarding its replacement either by:

    • closing the space and disguising the canine into the lateral incisor;

    • maintaining the space for prosthetic replacement.

  • Following completion of orthodontics these patients need to continue wearing retainers to maintain the final occlusion.

  • Final prosthodontic rehabilitation to replace the missing lateral incisor, where indicated will take place after completion of orthodontic treatment.

  • If osseointegrated implants are planned then placement should only take place after growth is complete, meaning that a period of temporization may be necessary if orthodontics is completed early in adolescence.

Psychology

  • Children pass through a number of developmental stages during which time certain tasks are mastered.

  • Healthy psychosocial adjustment depends on the child’s ability to successfully pass through each stage.

  • This cannot be accomplished alone and depends upon the interaction of the child with the systems within which they function including:

    • family;

    • educational;

    • social;

    • medical.

  • The child is at ↑ risk of psychosocial maladjustment during transitional points throughout development.

  • Individuals with CLP have the potential for healthy psychosocial adjustment comparable to their peers, but they are at risk of:

    • cognitive;

    • behavioural;

    • emotional;

    • social difficulties.

The clinical psychologist

  • Raises team members’ awareness of important transitional points.

  • Helps identify individuals at risk of psychosocial maladjustment who may benefit from psychological intervention to improve their psychosocial well-being.

  • The role of the psychologist changes as the child passes through the various developmental stages on the way to adulthood.

Research has shown that adults with repaired CLP at are ↑ risk of psychosocial adjustment problems relating to:

  • Psychological well-being (anxiety and depression).

  • Reduced social contacts.

  • Delays in marriage and having children.

  • Clinical psychology intervention therefore explores:

    • underlying causes of psychosocial maladjustment;

    • promoting self-acceptance;

    • discussing treatment options in collaboration with the team.