clinical aspects of cleft lip & palate reconstruction

Clinical Aspects of Cleft Lip/Palate Clinical Aspects of Cleft Lip/Palate ReconstructionReconstruction

Brian ClarkeBrian Clarke

MED IIMED IIDalhousie UniversityDalhousie UniversityHalifax, Nova ScotiaHalifax, Nova Scotia

OverviewOverview• Relevant AnatomyRelevant Anatomy• Embryology of Facial CleftingEmbryology of Facial Clefting• Classification/EpidemiologyClassification/Epidemiology• Principles of ManagementPrinciples of Management

• AssessmentAssessment– Indications/ContraindicationsIndications/Contraindications

• Surgical TechniquesSurgical Techniques– Millard Millard – Wardill-KilnerWardill-Kilner

• Post-op managementPost-op management– Complications– Follow up

Clinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction

Anatomic PrinciplesAnatomic PrinciplesNormal LipNormal Lip

1) Central Philtrum

Lateral margins - philtral columns

Inferior border - Cupids bow and tubercle

2) Vermillion-cutaneous border


Anatomic PrinciplesAnatomic Principles3) Muscles

Orbicularis oris (superficial and deep)

Levator labii superioris

Levator superioris alaeque

Transverse nasalis

End result of cleft lip: End result of cleft lip:

Disruption of the normal termination of the muscle fibers that cross the embryologic Disruption of the normal termination of the muscle fibers that cross the embryologic fault line of the maxillary and nasal processes, resulting in abnormal muscular forces fault line of the maxillary and nasal processes, resulting in abnormal muscular forces between the normal equilibrium that exists with the nasolabial and oral groups of between the normal equilibrium that exists with the nasolabial and oral groups of musclesmuscles


Anatomic PrinciplesAnatomic PrinciplesNormal PalateNormal Palate

Primary palatePrimary palate

Secondary palateSecondary palate

Soft palateSoft palate

Hard palateHard palate


Embryology of CleftingEmbryology of Clefting

Facial Development - 4Facial Development - 4thth - 10 - 10th th week of developmentweek of development

Formed by the fusion of five prominencesFormed by the fusion of five prominences

Unpaired frontonasal processUnpaired frontonasal process

- - lateral/medial nasal processeslateral/medial nasal processes

Paired maxillary swellings

Paired mandibular swelling

Nose/Philtrum of upper lipNose/Philtrum of upper lip

Cheeks/Upper lip (-philtrum)Cheeks/Upper lip (-philtrum)

Lower face (lower lip/chin)


Embryology of CleftingEmbryology of CleftingFacial DevelopmentFacial Development

Medial nasal processesMedial nasal processes (green) migrate toward

each other and fuse

Inferior tips of medial nasal processes expand laterally to form the intermaxillary process

Tips of maxillary swellings (yellow) grow to meet the intermaxillary process and fuse

66thth week week

7th week

Failure of maxillary swellings to fuse with intermaxillary process = cleft lipFailure of maxillary swellings to fuse with intermaxillary process = cleft lip


Formation of the PalateFormation of the Palate

66th th weekweek

1) As nasal pits of lateral nasal process invaginate and 1) As nasal pits of lateral nasal process invaginate and fuse, intermaxillary process extends to form primary fuse, intermaxillary process extends to form primary palatepalate


88th th - 9- 9th th weekweek

2) Medial walls of maxillary processes produce palatine shelves2) Medial walls of maxillary processes produce palatine shelves

3) Shelves grow downwards, parallel to lateral suface of 3) Shelves grow downwards, parallel to lateral suface of tonguetongue

4) End of week 9, rotate upward into a horizontal position 4) End of week 9, rotate upward into a horizontal position and fuse with each other and primary palate to form and fuse with each other and primary palate to form secondary palatesecondary palate


Cleft VariantsCleft Variants

Great anatomic variation in types of clefts!Great anatomic variation in types of clefts!

Anatomic Classification based on:Anatomic Classification based on:

1) Location 1) Location

2) Completeness (Incomplete/Complete)2) Completeness (Incomplete/Complete)

3) Extent3) Extent

Since lip, alveolus, and hard palate differ in embryologic Since lip, alveolus, and hard palate differ in embryologic origin, any combination can occurorigin, any combination can occur


Iowa ClassificationIowa Classification

Group IGroup I

Clefts of lip onlyClefts of lip only

Group IIGroup II

Clefts of palate only (Clefts of palate only (22oo))

Group IIIGroup III

Clefts of lip, Clefts of lip, alveolus, palatealveolus, palate

Group IVGroup IV

Clefts of lip and Clefts of lip and alveolus alveolus (primary (primary cleft palate and cleft palate and lip)lip)

Group VGroup V

MiscellaneousMiscellaneous


Striped YStriped Y

1 & 5 - Floor of nose on right & left sides1 & 5 - Floor of nose on right & left sides

2 & 6 - Lip2 & 6 - Lip

3 & 7 - Alveolar ridges3 & 7 - Alveolar ridges

4 & 8 - Premaxilla to incisive foramen4 & 8 - Premaxilla to incisive foramen

9 & 10 - Each half of the hard palate9 & 10 - Each half of the hard palate

11 - Soft palate11 - Soft palate

12 - Congenital velopharyngeal incompetence without obvious clefts12 - Congenital velopharyngeal incompetence without obvious clefts

13 - Protrusion of premaxilla13 - Protrusion of premaxilla


Cleft VariantsCleft Variants

1) Isolated Incomplete1) Isolated Incomplete

Intact skin/muscle between the lip and noseIntact skin/muscle between the lip and nose

Less distortion brought on by abnormal muscle pullLess distortion brought on by abnormal muscle pull

Bilateral/UnilateralBilateral/Unilateral

Cleft LipCleft LipExpressed in structures anterior to incisive foramenExpressed in structures anterior to incisive foramen

- prepalatal alveolus, maxilla, lip, nasal structures- prepalatal alveolus, maxilla, lip, nasal structures

Gaping cleft of alveolus/lip structures to mere Gaping cleft of alveolus/lip structures to mere ‘scar’ (‘scar’ (forme frusteforme fruste))

Deficiency in skin, muscles, mucous membranes, Deficiency in skin, muscles, mucous membranes, maxillary/nasal bones, nasal cartilagesmaxillary/nasal bones, nasal cartilages


2) Isolated Complete *2) Isolated Complete *

Bilateral/UnilateralBilateral/Unilateral

Cleft runs entire length of lip to floor of noseCleft runs entire length of lip to floor of nose

Abnormal muscle pull distorts nose extensively and creates wide Abnormal muscle pull distorts nose extensively and creates wide clefts between the lip segmentsclefts between the lip segments


Cleft VariantsCleft VariantsIsolated Cleft PalateIsolated Cleft Palate

Complete/IncompleteComplete/Incomplete

Soft PalateSoft Palate

-cleft can extend into the hard palate to -cleft can extend into the hard palate to any extentany extent

Hard PalateHard Palate

Primary Palate (CL)Primary Palate (CL)

Secondary PalateSecondary Palate


Cleft VariantsCleft VariantsCombined CleftsCombined Clefts

Complete lip/palateComplete lip/palate

Incomplete lip/palateIncomplete lip/palateClinical Aspects of Cleft Lip/Palate ReconstructionClinical Aspects of Cleft Lip/Palate Reconstruction

EpidemiologyEpidemiologyCleft lip/palate are second most common congenital abnormalitiesCleft lip/palate are second most common congenital abnormalities

Overall incidence of CP w CL and isolated CLOverall incidence of CP w CL and isolated CL= 1 in 1000 live births= 1 in 1000 live births

Isolated CP = 1 in 2000 live birthsIsolated CP = 1 in 2000 live birthsIncidence of CL/P varies with race and genderIncidence of CL/P varies with race and gender

Among total number of clefts:Among total number of clefts:20% CL (18% unilateral, 2% bilateral)20% CL (18% unilateral, 2% bilateral)50% CL and CP (38% unilateral, 12% bilateral)50% CL and CP (38% unilateral, 12% bilateral)30 % CP alone30 % CP alone

Asian>Caucasian>African AmericanAsian>Caucasian>African American

Male>Female (exception isolated cleft palate)Male>Female (exception isolated cleft palate)


EpidemiologyEpidemiology

Clustering noted in particular familiesClustering noted in particular families

Associated with over 150 syndromes!Associated with over 150 syndromes!

Genetic BasisGenetic Basis

Overall incidence of associated anomalies (eg cardiac) = 30% Overall incidence of associated anomalies (eg cardiac) = 30%

Family Makeup Risk of cleft lip/palate Risk of cleft palate

One affected sibling or parent 1 in 25 (4%) 2.5%

Two affected siblings 1 in 11 (9%) 1%

One sibling and one parent 1 in 6 (16%) 15%


Risk increases with parental age (>30yrs; particular paternal age)Risk increases with parental age (>30yrs; particular paternal age)

Environmental FactorsEnvironmental Factors

Viral infections (rubella)Viral infections (rubella)

Teratogens (steroids, anticonvulsants, alcohol, retinoic acid Teratogens (steroids, anticonvulsants, alcohol, retinoic acid derivatives)derivatives)


Principles of ManagementPrinciples of ManagementAssessmentAssessment

IndicationsIndications: restoring normal morphologic form and function: restoring normal morphologic form and function

Important for normal dentition, mastication, speech, hearing, and breathingImportant for normal dentition, mastication, speech, hearing, and breathing

ContraindicationsContraindications: malnutrition, anemia or other conditions that render infant : malnutrition, anemia or other conditions that render infant unable to tolerate general anesthesiaunable to tolerate general anesthesia- airway obstruction, otitis media with CP- airway obstruction, otitis media with CP

Work-upWork-up(1) Thorough PE to uncover any associated anomalies(1) Thorough PE to uncover any associated anomalies

Additional work-up determined by physical findings that suggest involvement Additional work-up determined by physical findings that suggest involvement of other organ systemsof other organ systems

(2) Weight, oral intake, growth/development are of primary concern (2) Weight, oral intake, growth/development are of primary concern

and must be followed closelyand must be followed closely (3) Routine lab studies generally not required; Hgb level before surgery(3) Routine lab studies generally not required; Hgb level before surgery


Surgical ManagementSurgical Management

Multidisciplinary approachMultidisciplinary approach

Beyond lip repair are other issues:Beyond lip repair are other issues:

Hearing (otolaryngologists)Hearing (otolaryngologists)

Speech (speech pathologists)Speech (speech pathologists)

Dental (oromaxillofacial surgeons)Dental (oromaxillofacial surgeons)

PsychosocialPsychosocial

Integration with team-based approachIntegration with team-based approach

Each case is assessed independently by those involved and a global treatment plan Each case is assessed independently by those involved and a global treatment plan is instituted based on present need in his/her developmentis instituted based on present need in his/her development

Cleft Lip and PalateCleft Lip and Palate

NutritionNutrition


Surgical ManagementSurgical ManagementStaging and Timing of SurgeryStaging and Timing of Surgery

Different institutions = different practiceDifferent institutions = different practice

Rule of 10’sRule of 10’sHgb = 10gHgb = 10g

Weight of 10lbsWeight of 10lbs

Age 10wksAge 10wks

IWK - 6-8 weeksIWK - 6-8 weeks

Cleft LipCleft Lip Cleft PalateCleft PalateIWK - 9-12 months of ageIWK - 9-12 months of age


Surgical ManagementSurgical ManagementUnilateral Complete Cleft LipUnilateral Complete Cleft Lip

Goal: Symmetric shaped nostrils, nasal sill, and alar bases; well defined Goal: Symmetric shaped nostrils, nasal sill, and alar bases; well defined philtral dimple and columns; natural appearing Cupid’s bow; functional philtral dimple and columns; natural appearing Cupid’s bow; functional muscle repairmuscle repair

Surgical Principle: Lengthen medial side of cleft so that it Surgical Principle: Lengthen medial side of cleft so that it equals the vertical dimensions of non-cleft sideequals the vertical dimensions of non-cleft side

Flap designs:Flap designs:

1) Triangular (Tennison-Randall)1) Triangular (Tennison-Randall)

2) Quadrangular 2) Quadrangular

3) Rotation-advancement (Millard*, Mohler)3) Rotation-advancement (Millard*, Mohler)


Millard TechniqueMillard Technique

Scar placed in more anatomically correct position along philtral column

““Cut as you go” techniqueCut as you go” technique

1) Medial flap rotates downward to 1) Medial flap rotates downward to achieve necessary lengtheningachieve necessary lengthening

2) Lateral flap advances into the defect produced 2) Lateral flap advances into the defect produced by downward displacement of medial flapby downward displacement of medial flap

3) Small pennant-shaped medial flap can be 3) Small pennant-shaped medial flap can be used to restore nostril sill or lengthen the used to restore nostril sill or lengthen the columellacolumella

Preserves’ cupid’s bow and philtral dimplePreserves’ cupid’s bow and philtral dimple

Tension of closure under the alar base; reduces flair and promotes better molding of Tension of closure under the alar base; reduces flair and promotes better molding of the underlying alveolar processesthe underlying alveolar processes

In simple medical student terms:In simple medical student terms:


In Complex Resident/Staff Terms:In Complex Resident/Staff Terms:


Post-op ManagementPost-op Management

1) Feedings administered with catheter tip syringe fitted 1) Feedings administered with catheter tip syringe fitted with small red rubber catheter for the first 10 days post-with small red rubber catheter for the first 10 days post-opop

2) Nipples are avoided to minimize strain on the 2) Nipples are avoided to minimize strain on the muscle/skin suturesmuscle/skin sutures

3) Velcro arm restraints to protect repair from 3) Velcro arm restraints to protect repair from flailing hands/fingersflailing hands/fingers

4) Suture line care: PRN cleansing with half strength 4) Suture line care: PRN cleansing with half strength peroxide followed with polymixin B-bacitracin ointmentperoxide followed with polymixin B-bacitracin ointment

Cleft LipCleft Lip



Scar contractureScar contracture

ErythemaErythema

FirmnessFirmness

Inform the parents of:Inform the parents of:

Avoid placing in direct sunlight until the scar fully maturesAvoid placing in direct sunlight until the scar fully matures


ComplicationsComplicationsPost-op ManagementPost-op Management

• AestheticAesthetic– vermilion-cutaneous vermilion-cutaneous

mismatchmismatch

– vermilion notchingvermilion notching

– tight appearing lateral tight appearing lateral lip segementlip segement

– lateral muscle buldgelateral muscle buldge

– laterally displaced alalaterally displaced ala

– constricted appearing constricted appearing nostrilnostril

• OtherOther– dehiscencedehiscence

– excessive scar excessive scar formationformation


Surgical ManagementSurgical ManagementCleft PalateCleft Palate

Goal: Production of a competent velopharyngeal sphincterGoal: Production of a competent velopharyngeal sphincter

Two most common repairs:Two most common repairs:

1) V-Y (Veau-Wardill-Kilner)*1) V-Y (Veau-Wardill-Kilner)*

2) von Langenbeck2) von Langenbeck

Main difference: V-Y repair involves Main difference: V-Y repair involves elongation of the palateelongation of the palate, while , while von Langenbeck does notvon Langenbeck does not


Wardill-KilnerWardill-Kilner1) Incisions made along free margins of cleft and extend 1) Incisions made along free margins of cleft and extend anteriorly to apexanteriorly to apex

2) Dissection continued posteriorly along oral side of 2) Dissection continued posteriorly along oral side of alveolar ridge to retromolar trigonealveolar ridge to retromolar trigone


Wardill-KilnerWardill-Kilner3) Mucoperiosteal flaps are elevated from 3) Mucoperiosteal flaps are elevated from nasal/oral surfaces of bony palatenasal/oral surfaces of bony palate

4) Dissection of the greater palatine vessels from 4) Dissection of the greater palatine vessels from the foramen lengthens the pediclethe foramen lengthens the pedicle

5) Tensor veli palatini muscle is elevated off the 5) Tensor veli palatini muscle is elevated off the hamulus to aid in relaxing the midline closurehamulus to aid in relaxing the midline closure


Wardill-KilnerWardill-Kilner

6) Nasal mucosa freed from bony palate 6) Nasal mucosa freed from bony palate and closed to either side, or if necessary and closed to either side, or if necessary closed by using vomer flapsclosed by using vomer flaps

7) Muscle and oral mucosa closed in a 7) Muscle and oral mucosa closed in a second single layer in a horizontal fashionsecond single layer in a horizontal fashion


Wardill-KilnerWardill-Kilner8) Anteriorly, the oral mucoperiosteal flaps are 8) Anteriorly, the oral mucoperiosteal flaps are attached to the third flap (mucosa overlying the attached to the third flap (mucosa overlying the primary palateprimary palate

9) Posteriorly, the palate is closed in 3 layers9) Posteriorly, the palate is closed in 3 layersNasal mucosaNasal mucosaLevator muscleLevator muscleOral mucosaOral mucosa


Post-op ManagementPost-op ManagementCleft PalateCleft Palate

Immediate concerns: Immediate concerns:

1) Airway management1) Airway management

2) Analgesia2) Analgesia

Risk of oversedation and subsequent airway comprimiseRisk of oversedation and subsequent airway comprimise

Acetominophen, Codeine sufficient: cont’d for 7-10 daysAcetominophen, Codeine sufficient: cont’d for 7-10 days

Arm restraints to prevent placing fingers in mouthArm restraints to prevent placing fingers in mouth

Diet restricted to liquids, soft foods (x3wks): bottles avoidedDiet restricted to liquids, soft foods (x3wks): bottles avoided

Change in nasal/oral airway dynamicsChange in nasal/oral airway dynamics



• Airway obstructionAirway obstruction

• Intraoperative bleedingIntraoperative bleeding

• Palatal fistulaPalatal fistula

• Midface abnormalities (early interventions)Midface abnormalities (early interventions)

ComplicationsComplications


Cleft Palate ClinicsCleft Palate Clinics

Through a protocol of sequential, regular evaluations by a Through a protocol of sequential, regular evaluations by a team composed of plastic surgeon, speech pathologist, team composed of plastic surgeon, speech pathologist, orthodontist, and audiologist, great strides have been made in orthodontist, and audiologist, great strides have been made in improving all aspects of care of the child with cleft palateimproving all aspects of care of the child with cleft palate


clinical aspects of cleft lip & palate reconstruction

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