Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

complete cleft lip and palate

1st line – specialised feeding

Back
ONGOING
|
complete cleft lip and palate
1st line – 

specialised feeding

Feeding difficulties may necessitate extended postnatal hospitalisation, although NG tube feeding is seldom required.

Neonates with a cleft palate often cannot produce the negative pressures necessary for suction.

Evidence-based guidelines exist for the use of breastfeeding in patients with CLP, both pre-operatively and after cleft repair. These advocate both individualised support for nursing mothers and monitoring newborn weight gain and hydration status.[42][43]

The neonate will lose weight after birth (up to 10% of birth weight), but the birth weight is expected to be re-established within the first 2 postnatal weeks, and the infant should go on to gain at least 25 g (1 ounce) per day thereafter.

Nasal regurgitation is addressed with upright positioning of the infant during feeding. Bottle feeding is optimised using specialised fissured nipples and bottles controlling flow rate. Soft bottles are squeezed in synchronisation with infant sucking to reduce effort of feeding and maximise amount of feed entering the mouth. Infant should be burped during pauses, and feeding should not be continued for more than 30 minutes to avoid fatigue.

A Cochrane review of 5 randomised controlled trials examined various feeding interventions and their effects on weight of children at 6 weeks after surgery for repair of cleft lip and palate. While squeezable bottles appeared easier to use than rigid bottles, no differences in growth outcomes were noted. Maxillary plates also showed no evidence for improved growth at 6 weeks after surgery. Weak evidence exists to show that breastfeeding had a positive effect on weight gain after surgery when compared to spoon feeding.[44] [ Cochrane Clinical Answers logo ]

Consider – airway management

Back
ONGOING
|
complete cleft lip and palate
Consider – 

airway management

Additional treatment recommended for SOME patients in selected patient group

Symptoms of severe airway obstruction require immediate airway management.

Upper airway obstruction associated with Pierre Robin sequence (triad of cleft palate, microgenia, and glossoptosis) is treated with prone positioning, nasopharyngeal trumpet, and/or nasal CPAP.[45] Up to 23% of infants with micrognathia have a tongue-related obstruction requiring endotracheal intubation if the above measures are ineffective.[46]

Surgical management of airway obstruction in micrognathia includes tongue lip adhesion, mandibular distraction osteogenesis (to move the base of tongue forwards and open the airway), and tracheostomy, which should only be undertaken once all other options have been exhausted.

Consider – pre-surgical lip taping, oral appliances, or pre-surgical nasal alveolar moulding (PNAM)

Back
ONGOING
|
complete cleft lip and palate
|
0 to 3 months of age
Consider – 

pre-surgical lip taping, oral appliances, or pre-surgical nasal alveolar moulding (PNAM)

Additional treatment recommended for SOME patients in selected patient group

Infants with wide (>1 cm) clefts undergo the first stage of cleft lip repair with pre-surgical lip taping, oral appliances, or PNAM to narrow the cleft.

Daily lip taping with steristrips and benzoin to enhance adherence (as feeding causes wetting of the tape) is undertaken by the parents following instruction, and is used to protect the cheek skin.

The Latham oral device is used to actively re-position the lateral alveolar cleft segments while de-projecting the protruded pre-maxilla.[47]

In PNAM, an appliance is placed within the cleft and adjusted weekly to approximate the alveolar segments, reducing the width of the cleft alveolus and corresponding soft tissues of the cleft lip. The objectives of PNAM are to elongate the columella, expand the cleft nasal mucosa, and improve nasal tip symmetry. It should ideally be initiated within the first 6 postnatal weeks in order to utilise the early plasticity of the nasal cartilages.[52]

Plus – definitive cleft lip repair ± bilateral myringotomy and tympanostomy tube (T-tube) placement

Back
ONGOING
|
complete cleft lip and palate
|
3 to 7 months of age
Plus – 

definitive cleft lip repair ± bilateral myringotomy and tympanostomy tube (T-tube) placement

Treatment recommended for ALL patients in selected patient group

Infants with narrow (<1 cm) clefts receive definitive cleft lip repair at around 3 months of age without any pre-surgical procedure (e.g., pre-surgical nasal alveolar moulding [PNAM], lip taping, lip adhesion).

Infants with wide (>1 cm) clefts receive second-stage definitive cleft lip repair following previous PNAM and pre-surgical lip taping undertaken to narrow the cleft.

Repair of the unilateral or bilateral cleft lip involves approximation of the 2 sides of the cleft lip using precisely designed segments of tissue, creating exact proportions of the underlying oral mucosa, muscle, and lip contours.

Bilateral myringotomy and T-tube (Shepard type) placement is performed following tympanograms and head and neck surgery assessment if there is evidence of Eustachian tube dysfunction.

Audiometry is undertaken before and after T-tube placement.

Although infrequently performed, alterations to the cleft lip nasal deformity can be made with intermediate rhinoplasty at any time after definitive cleft lip repair and prior to definitive septorhinoplasty in select cases of lip clefting: for example, where there is gross aesthetic distortion or nostril stenosis with complete nasal obstruction.

Plus – palatoplasty and long-lasting tympanostomy tube (T-tube) placement ± V-Y columellar advancement

Back
ONGOING
|
complete cleft lip and palate
|
10 to 14 months of age
Plus – 

palatoplasty and long-lasting tympanostomy tube (T-tube) placement ± V-Y columellar advancement

Treatment recommended for ALL patients in selected patient group

The placement of longer-lasting T-tubes following bilateral myringotomy is performed concurrently with palatoplasty (cleft palate repair).

Audiometry is undertaken before and after T-tube placement.

V-Y columellar advancement (lengthening of short columellar skin, performed by advancing skin from the central lip on to the columella with a V-shaped end and closing the lip, resulting in a Y configuration) is performed concurrently in infants with complete bilateral cleft lip and palate if primary techniques (e.g., pre-surgical nasal alveolar moulding [PNAM]) are not done or prove inadequate.

Plus – speech and language therapy

Back
ONGOING
|
complete cleft lip and palate
|
2 to 5 years of age
Plus – 

speech and language therapy

Treatment recommended for ALL patients in selected patient group

Assessment with fluoroscopic speech examination and nasopharyngoscopy is undertaken to observe for velopharyngeal insufficiency (VPI) or dysfunction (VPD).

Speech and language therapy is instituted if VPD is present.

Consider – secondary speech surgery

Back
ONGOING
|
complete cleft lip and palate
|
2 to 5 years of age
Consider – 

secondary speech surgery

Additional treatment recommended for SOME patients in selected patient group

If hyper-nasal speech is not responsive to speech and language therapy, secondary speech surgery is warranted following a velopharyngeal dysfunction evaluation.

Surgical options for the treatment of velopharyngeal dysfunction include a superiorly based pharyngeal flap or dynamic pharyngoplasty. Occasionally, a palate-lengthening procedure (Furlow double-opposing Z-plasty) is performed.

May be complicated by obstructive sleep apnoea.[20]

Plus – alveolar cleft bone grafting with preparatory orthodontics

Back
ONGOING
|
complete cleft lip and palate
|
8 to 11 years of age
Plus – 

alveolar cleft bone grafting with preparatory orthodontics

Treatment recommended for ALL patients in selected patient group

Preparatory orthodontic maxillary expansion for alveolar cleft bone grafting with iliac crest bone on eruption of the key permanent dentition.

If maxillary segments and dentition on either side of the alveolar clefts are aligned, orthodontics can be postponed until bone grafting has been done and most of the permanent dental eruption is complete.

Plus – definitive septorhinoplasty ± prior orthodontics and orthognathic surgery

Back
ONGOING
|
complete cleft lip and palate
|
at skeletal maturity
Plus – 

definitive septorhinoplasty ± prior orthodontics and orthognathic surgery

Treatment recommended for ALL patients in selected patient group

Orthodontics and orthognathic surgery for dentofacial malocclusion, which may be necessary in order to obtain Angle Class 1 molar tooth relationships (in Angle Class I occlusion, there is a normal relationship between the maxillary and mandibular first molars), are performed prior to definitive septorhinoplasty.

LeFort advancements are sometimes performed to adjust the dentofacial relationship, particularly in the case of mid-face hypoplasia. Although providing increased mid-face protrusion, it is important to note that a moderate amount of relapse in the horizontal and vertical plane does occur.[63]

Definitive septorhinoplasty is completed using an open approach to correct the asymmetrical upper and lower lateral cartilages, and to re-align the caudal septum that is deviated to the non-cleft side. Symmetry of the alar base (the soft tissue and cartilaginous components of the nostril, which includes the lower lateral [alar] cartilages) is corrected with alar base excisions and, on the cleft side, is augmented with cartilage, bone, or allograft. Lateral crural strut grafting and nasal tip refining techniques (interdomal sutures and tip shield grafts) improve symmetry. Alar rim grafts and excision of nostril hooding are also effective in select cases. Osteotomies and dorsal refinement (excision or augmentation) are combined with spreader grafting between the upper lateral cartilages for enhanced support.

isolated cleft palate

1st line – specialised feeding

Back
ONGOING
|
isolated cleft palate
1st line – 

specialised feeding

Feeding difficulties may necessitate extended postnatal hospitalisation, although NG tube feeding is seldom required.

Neonates with a cleft palate often cannot produce the negative pressures necessary for suction.

Evidence-based guidelines exist for the use of breastfeeding in patients with CLP, both pre-operatively and after cleft repair. These advocate both individualised support for nursing mothers and monitoring newborn weight gain and hydration status.[42][43]

Nasal regurgitation is addressed with upright positioning of the infant during feeding. Bottle feeding is optimised using specialised fissured nipples and bottles controlling flow rate. Soft bottles are squeezed in synchronisation with infant sucking to reduce effort of feeding and maximise amount of feed entering the mouth. Infant should be burped during pauses, and feeding should not be continued for more than 30 minutes to avoid fatigue.

A Cochrane review of 5 randomised controlled trials examined various feeding interventions and their effects on weight of children at 6 weeks after surgery for repair of cleft lip and palate. While squeezable bottles appeared easier to use than rigid bottles, no differences in growth outcomes were noted. Maxillary plates also showed no evidence for improved growth at 6 weeks after surgery. Weak evidence exists to show that breastfeeding had a positive effect on weight gain after surgery when compared to spoon feeding.[44] [ Cochrane Clinical Answers logo ]

Consider – airway management

Back
ONGOING
|
isolated cleft palate
Consider – 

airway management

Additional treatment recommended for SOME patients in selected patient group

Symptoms of severe airway obstruction require immediate airway management.

Upper airway obstruction associated with Pierre Robin sequence (triad of cleft palate, microgenia, and glossoptosis) is treated with prone positioning, nasopharyngeal trumpet, and/or nasal CPAP.[45] Up to 23% of infants with micrognathia have a tongue-related obstruction requiring endotracheal intubation if the above measures are ineffective.[46]

Surgical management of airway obstruction in micrognathia includes tongue lip adhesion, mandibular distraction osteogenesis (to move the base of tongue forwards and open the airway), and tracheostomy, which should only be undertaken once all other options have been exhausted.

Consider – bilateral myringotomy and tympanostomy tube (T-tube) placement

Back
ONGOING
|
isolated cleft palate
|
3 to 6 months of age
Consider – 

bilateral myringotomy and tympanostomy tube (T-tube) placement

Additional treatment recommended for SOME patients in selected patient group

Bilateral myringotomy and T-tube (Shepard type) placement is performed following tympanograms and head and neck surgery assessment if there is evidence of Eustachian tube dysfunction.

Audiometry is undertaken before and after T-tube placement.

Plus – palatoplasty and long-lasting tympanostomy tube (T-tube) placement

Back
ONGOING
|
isolated cleft palate
|
10 to 14 months of age
Plus – 

palatoplasty and long-lasting tympanostomy tube (T-tube) placement

Treatment recommended for ALL patients in selected patient group

The placement of longer-lasting T-tubes following bilateral myringotomy is performed concurrently with palatoplasty (cleft palate repair).

Audiometry is undertaken before and after T-tube placement.

Plus – speech and language therapy

Back
ONGOING
|
isolated cleft palate
|
2 to 5 years of age
Plus – 

speech and language therapy

Treatment recommended for ALL patients in selected patient group

Assessment with fluoroscopic speech examination and nasopharyngoscopy is undertaken to observe for velopharyngeal insufficiency (VPI) or dysfunction (VPD).

Speech and language therapy is instituted if VPD is present.

Consider – secondary speech surgery

Back
ONGOING
|
isolated cleft palate
|
2 to 5 years of age
Consider – 

secondary speech surgery

Additional treatment recommended for SOME patients in selected patient group

If hyper-nasal speech is not responsive to speech and language therapy, secondary speech surgery is warranted following a velopharyngeal dysfunction evaluation.

Surgical options for the treatment of velopharyngeal dysfunction (VPD) include a superiorly based pharyngeal flap or dynamic pharyngoplasty. Occasionally, a palate-lengthening procedure (Furlow double-opposing Z-plasty) is performed.

A patient with a sub-mucous cleft palate may develop velopharyngeal dysfunction requiring surgical intervention. This may include a lengthening palatoplasty (Furlow double-opposing Z-plasty) or pharyngeal surgery (sphincter pharyngoplasty or pharyngeal flap procedure). Both are considered equally effective, although comparative studies have not yet been well-designed or powered enough to differentiate between the effectiveness.

May be complicated by obstructive sleep apnoea.[20]

Consider – preparatory orthodontics ± orthognathic surgery

Back
ONGOING
|
isolated cleft palate
|
at skeletal maturity
Consider – 

preparatory orthodontics ± orthognathic surgery

Additional treatment recommended for SOME patients in selected patient group

Orthodontics and orthognathic surgery for dentofacial malocclusion may be necessary in order to obtain Angle Class 1 molar tooth relationships (in Angle Class I occlusion, there is a normal relationship between the maxillary and mandibular first molars).

LeFort advancements are sometimes performed to adjust the dentofacial relationship, particularly in the case of mid-face hypoplasia. Although providing increased mid-face protrusion, it is important to note that a moderate amount of relapse in the horizontal and vertical plane does occur.[63]

isolated cleft lip

1st line – specialised feeding

Back
ONGOING
|
isolated cleft lip
1st line – 

specialised feeding

Feeding difficulties may necessitate extended postnatal hospitalisation, although NG tube feeding is seldom required.

Evidence-based guidelines exist for the use of breastfeeding in CLP patients, both pre-operatively and after cleft repair. These advocate both individualised support for nursing mothers and monitoring newborn weight gain and hydration status.[42][43]

Nasal regurgitation is addressed with upright positioning of the infant during feeding. Bottle feeding is optimised using specialised fissured nipples and bottles controlling flow rate. Soft bottles are squeezed in synchronisation with infant sucking to reduce effort of feeding and maximise amount of feed entering the mouth. Infant should be burped during pauses, and feeding should not be continued for more than 30 minutes to avoid fatigue.

A Cochrane review of 5 randomised controlled trials examined various feeding interventions and their effects on weight of children at 6 weeks after surgery for repair of cleft lip and palate. While squeezable bottles appeared easier to use than rigid bottles, no differences in growth outcomes were noted. Maxillary plates also showed no evidence for improved growth at 6 weeks after surgery. Weak evidence exists to show that breastfeeding had a positive effect on weight gain after surgery when compared to spoon feeding.[44] [ Cochrane Clinical Answers logo ]

Consider – pre-surgical lip taping, oral appliances, or pre-surgical nasal alveolar moulding (PNAM)

Back
ONGOING
|
isolated cleft lip
|
0 to 3 months of age
Consider – 

pre-surgical lip taping, oral appliances, or pre-surgical nasal alveolar moulding (PNAM)

Additional treatment recommended for SOME patients in selected patient group

Infants with wide (>1 cm) clefts undergo the first stage of cleft lip repair with pre-surgical lip taping, oral appliances, or PNAM to narrow the cleft.

Daily lip taping with steristrips and benzoin to enhance adherence (as feeding causes wetting of the tape) is undertaken by the parents following instruction, and is used to protect the cheek skin in both bilateral and unilateral cleft lip deformities.

The Latham oral device is used to actively re-position the lateral alveolar cleft segments while de-projecting the protruded pre-maxilla.[47]

Plus – definitive cleft lip repair

Back
ONGOING
|
isolated cleft lip
|
3 to 7 months of age
Plus – 

definitive cleft lip repair

Treatment recommended for ALL patients in selected patient group

Infants with narrow (<1 cm) clefts receive definitive cleft lip repair at around 3 months of age without any pre-surgical procedure (e.g., pre-surgical nasal alveolar moulding [PNAM], lip taping, lip adhesion).

Infants with wide (>1 cm) clefts receive second-stage definitive cleft lip repair following previous PNAM and pre-surgical lip taping undertaken to narrow the cleft.

Repair of the unilateral or bilateral cleft lip involves approximation of the 2 sides of the cleft lip, using precisely designed segments of tissue, creating exact proportions of the underlying oral mucosa, muscle, and lip contours.

Although infrequently performed, alterations to the cleft lip nasal deformity can be made with intermediate rhinoplasty at any time after definitive cleft lip repair and prior to definitive septorhinoplasty in select cases of lip clefting: for example, where there is gross aesthetic distortion or nostril stenosis with complete nasal obstruction.

Consider – V-Y columellar advancement

Back
ONGOING
|
isolated cleft lip
|
10 to 14 months of age
Consider – 

V-Y columellar advancement

Additional treatment recommended for SOME patients in selected patient group

Performed in infants with isolated bilateral cleft lip if primary techniques (e.g., pre-surgical nasal alveolar moulding [PNAM]) are not done or prove inadequate.

Consider – alveolar cleft bone grafting with preparatory orthodontics

Back
ONGOING
|
isolated cleft lip
|
8 to 11 years of age
Consider – 

alveolar cleft bone grafting with preparatory orthodontics

Additional treatment recommended for SOME patients in selected patient group

Only performed in bilateral isolated cleft lip.

Preparatory orthodontic maxillary expansion for alveolar cleft bone grafting with iliac crest bone on eruption of the key permanent dentition.

If maxillary segments and dentition on either side of the alveolar clefts are aligned, orthodontics can be postponed until bone grafting has been done and most of the permanent dental eruption is complete.

Plus – definitive septorhinoplasty ± prior orthodontics and orthognathic surgery

Back
ONGOING
|
isolated cleft lip
|
at skeletal maturity
Plus – 

definitive septorhinoplasty ± prior orthodontics and orthognathic surgery

Treatment recommended for ALL patients in selected patient group

Orthodontics and orthognathic surgery for dentofacial malocclusion, which may be necessary in order to obtain Angle Class 1 molar tooth relationships (in Angle Class I occlusion, there is a normal relationship between the maxillary and mandibular first molars), are only performed prior to definitive septorhinoplasty in bilateral isolated cleft lip.

LeFort advancements are sometimes performed to adjust the dentofacial relationship, particularly in the case of mid-face hypoplasia. Although providing increased mid-face protrusion, it is important to note that a moderate amount of relapse in the horizontal and vertical plane does occur.[63]

Definitive septorhinoplasty is completed using an open approach to correct the asymmetrical upper and lower lateral cartilages, and to realign the caudal septum that is deviated to the non-cleft side. Symmetry of the alar base (from the Latin 'ala' or wing; the soft tissue and cartilaginous components of the nostril, which includes the lower lateral [alar] cartilages) is corrected with alar base excisions and, on the cleft side, is augmented with cartilage, bone, or allograft. Lateral crural strut grafting and nasal tip refining techniques (interdomal sutures and tip shield grafts) improve symmetry. Alar rim grafts and excision of nostril hooding are also effective in select cases. Osteotomies and dorsal refinement (excision or augmentation) are combined with spreader grafting between the upper lateral cartilages for enhanced support.

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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