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Clinical Aspects of Cleft Lip/Palate Reconstruction

Brian Clarke
MED II Dalhousie University Halifax, Nova Scotia

Overview
Relevant Anatomy Embryology of Facial Clefting Classification/Epidemiology Principles of Management
Assessment
Indications/Contraindications

Surgical Techniques
Millard Wardill-Kilner

Post-op management
Complications Follow up
Clinical Aspects of Cleft Lip/Palate Reconstruction

Anatomic Principles
Normal Lip
1) Central Philtrum Lateral margins - philtral columns Inferior border - Cupids bow and tubercle 2) Vermillion-cutaneous border

Clinical Aspects of Cleft Lip/Palate Reconstruction

Anatomic Principles
3) Muscles Orbicularis oris (superficial and deep) Levator labii superioris Levator superioris alaeque Transverse nasalis

End result of cleft lip: 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 between the normal equilibrium that exists with the nasolabial and oral groups of muscles
Clinical Aspects of Cleft Lip/Palate Reconstruction

Anatomic Principles
Normal Palate Primary palate Secondary palate Soft palate Hard palate

Clinical Aspects of Cleft Lip/Palate Reconstruction

Embryology of Clefting
Facial Development - 4th - 10th week of development
Formed by the fusion of five prominences Unpaired frontonasal process

- lateral/medial nasal processes


Nose/Philtrum of upper lip

Paired maxillary swellings


Cheeks/Upper lip (-philtrum)

Paired mandibular swelling


Lower face (lower lip/chin)
Clinical Aspects of Cleft Lip/Palate Reconstruction

Embryology of Clefting
Facial Development 6th week
Medial nasal processes (green) migrate toward each other and fuse

7th week
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
Failure of maxillary swellings to fuse with intermaxillary process = cleft lip
Clinical Aspects of Cleft Lip/Palate Reconstruction

Formation of the Palate 6th week


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

Clinical Aspects of Cleft Lip/Palate Reconstruction

8th - 9th week


2) Medial walls of maxillary processes produce palatine shelves 3) Shelves grow downwards, parallel to lateral suface of tongue

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

Clinical Aspects of Cleft Lip/Palate Reconstruction

Cleft Variants
Great anatomic variation in types of clefts!
Anatomic Classification based on: 1) Location 2) Completeness (Incomplete/Complete) 3) Extent Since lip, alveolus, and hard palate differ in embryologic origin, any combination can occur
Clinical Aspects of Cleft Lip/Palate Reconstruction

Iowa Classification
Group I Clefts of lip only Group II Clefts of palate only (2o)

Group III Clefts of lip, alveolus, palate

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

Group V Miscellaneous
Clinical Aspects of Cleft Lip/Palate Reconstruction

Striped Y

1 & 5 - Floor of nose on right & left sides 2 & 6 - Lip 3 & 7 - Alveolar ridges 4 & 8 - Premaxilla to incisive foramen 9 & 10 - Each half of the hard palate 11 - Soft palate 12 - Congenital velopharyngeal incompetence without obvious clefts

13 - Protrusion of premaxilla

Clinical Aspects of Cleft Lip/Palate Reconstruction

Cleft Variants
Cleft Lip
Expressed in structures anterior to incisive foramen
- prepalatal alveolus, maxilla, lip, nasal structures

Deficiency in skin, muscles, mucous membranes, maxillary/nasal bones, nasal cartilages 1) Isolated Incomplete
Bilateral/Unilateral

Intact skin/muscle between the lip and nose Less distortion brought on by abnormal muscle pull Gaping cleft of alveolus/lip structures to mere scar (forme fruste)
Clinical Aspects of Cleft Lip/Palate Reconstruction

2) Isolated Complete *
Bilateral/Unilateral Cleft runs entire length of lip to floor of nose Abnormal muscle pull distorts nose extensively and creates wide clefts between the lip segments

Clinical Aspects of Cleft Lip/Palate Reconstruction

Cleft Variants
Isolated Cleft Palate

Primary Palate (CL) Secondary Palate


Soft Palate Hard Palate Complete/Incomplete -cleft can extend into the hard palate to any extent

Clinical Aspects of Cleft Lip/Palate Reconstruction

Cleft Variants
Combined Clefts
Complete lip/palate

Incomplete lip/palate
Clinical Aspects of Cleft Lip/Palate Reconstruction

Epidemiology
Cleft lip/palate are second most common congenital abnormalities Overall incidence of CP w CL and isolated CL = 1 in 1000 live births Isolated CP = 1 in 2000 live births
Incidence of CL/P varies with race and gender Asian>Caucasian>African American Male>Female (exception isolated cleft palate)

Among total number of clefts: 20% CL (18% unilateral, 2% bilateral) 50% CL and CP (38% unilateral, 12% bilateral) 30 % CP alone Clinical Aspects of Cleft Lip/Palate Reconstruction

Epidemiology
Genetic Basis
Clustering noted in particular families Associated with over 150 syndromes!

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 One sibling and one parent

1 in 11 (9%) 1 in 6 (16%)

1% 15%

Clinical Aspects of Cleft Lip/Palate Reconstruction

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

Environmental Factors

Viral infections (rubella) Teratogens (steroids, anticonvulsants, alcohol, retinoic acid derivatives)

Clinical Aspects of Cleft Lip/Palate Reconstruction

Principles of Management
Assessment
Indications: restoring normal morphologic form and function Important for normal dentition, mastication, speech, hearing, and breathing
Contraindications: malnutrition, anemia or other conditions that render infant unable to tolerate general anesthesia - airway obstruction, otitis media with CP

Work-up
(1) Thorough PE to uncover any associated anomalies Additional work-up determined by physical findings that suggest involvement of other organ systems (2) Weight, oral intake, growth/development are of primary concern and must be followed closely (3) Routine lab studies generally not required; Hgb level before surgery
Clinical Aspects of Cleft Lip/Palate Reconstruction

Surgical Management
Cleft Lip and Palate
Multidisciplinary approach
Beyond lip repair are other issues: Hearing (otolaryngologists) Speech (speech pathologists) Dental (oromaxillofacial surgeons) Nutrition Psychosocial

Integration with team-based approach


Each case is assessed independently by those involved and a global treatment plan is instituted based on present need in his/her development
Clinical Aspects of Cleft Lip/Palate Reconstruction

Surgical Management
Staging and Timing of Surgery
Different institutions = different practice

Cleft Lip
Rule of 10s
Hgb = 10g Weight of 10lbs Age 10wks

Cleft Palate
IWK - 9-12 months of age

IWK - 6-8 weeks

Clinical Aspects of Cleft Lip/Palate Reconstruction

Surgical Management
Unilateral Complete Cleft Lip
Goal: Symmetric shaped nostrils, nasal sill, and alar bases; well defined philtral dimple and columns; natural appearing Cupids bow; functional muscle repair

Surgical Principle: Lengthen medial side of cleft so that it equals the vertical dimensions of non-cleft side
Flap designs: 1) Triangular (Tennison-Randall) 2) Quadrangular 3) Rotation-advancement (Millard*, Mohler)
Clinical Aspects of Cleft Lip/Palate Reconstruction

Millard Technique
Cut as you go technique Preserves cupids bow and philtral dimple Scar placed in more anatomically correct position along philtral column Tension of closure under the alar base; reduces flair and promotes better molding of the underlying alveolar processes

In simple medical student terms:


1) Medial flap rotates downward to achieve necessary lengthening

2) Lateral flap advances into the defect produced by downward displacement of medial flap
3) Small pennant-shaped medial flap can be used to restore nostril sill or lengthen the columella
Clinical Aspects of Cleft Lip/Palate Reconstruction

In Complex Resident/Staff Terms:

Clinical Aspects of Cleft Lip/Palate Reconstruction

Clinical Aspects of Cleft Lip/Palate Reconstruction

Post-op Management
Cleft Lip
1) Feedings administered with catheter tip syringe fitted with small red rubber catheter for the first 10 days postop 2) Nipples are avoided to minimize strain on the muscle/skin sutures
3) Velcro arm restraints to protect repair from flailing hands/fingers 4) Suture line care: PRN cleansing with half strength peroxide followed with polymixin B-bacitracin ointment

Clinical Aspects of Cleft Lip/Palate Reconstruction

Post-op Management
Inform the parents of: Scar contracture Erythema Firmness Avoid placing in direct sunlight until the scar fully matures

Clinical Aspects of Cleft Lip/Palate Reconstruction

Post-op Management
Complications

Aesthetic
vermilion-cutaneous mismatch vermilion notching tight appearing lateral lip segement lateral muscle buldge laterally displaced ala constricted appearing nostril

Other
dehiscence excessive scar formation

Clinical Aspects of Cleft Lip/Palate Reconstruction

Surgical Management
Cleft Palate
Goal: Production of a competent velopharyngeal sphincter

Two most common repairs:

1) V-Y (Veau-Wardill-Kilner)*
2) von Langenbeck

Main difference: V-Y repair involves elongation of the palate, while von Langenbeck does not
Clinical Aspects of Cleft Lip/Palate Reconstruction

Wardill-Kilner
1) Incisions made along free margins of cleft and extend anteriorly to apex 2) Dissection continued posteriorly along oral side of alveolar ridge to retromolar trigone

Clinical Aspects of Cleft Lip/Palate Reconstruction

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

4) Dissection of the greater palatine vessels from the foramen lengthens the pedicle 5) Tensor veli palatini muscle is elevated off the hamulus to aid in relaxing the midline closure
Clinical Aspects of Cleft Lip/Palate Reconstruction

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

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

Clinical Aspects of Cleft Lip/Palate Reconstruction

Wardill-Kilner
8) Anteriorly, the oral mucoperiosteal flaps are attached to the third flap (mucosa overlying the primary palate 9) Posteriorly, the palate is closed in 3 layers Nasal mucosa Levator muscle Oral mucosa

Clinical Aspects of Cleft Lip/Palate Reconstruction

Post-op Management
Cleft Palate
Immediate concerns:
1) Airway management
Change in nasal/oral airway dynamics

2) Analgesia
Risk of oversedation and subsequent airway comprimise Acetominophen, Codeine sufficient: contd for 7-10 days

Arm restraints to prevent placing fingers in mouth Diet restricted to liquids, soft foods (x3wks): bottles avoided
Clinical Aspects of Cleft Lip/Palate Reconstruction

Post-op Management
Complications
Airway obstruction Intraoperative bleeding Palatal fistula Midface abnormalities (early interventions)

Clinical Aspects of Cleft Lip/Palate Reconstruction

Cleft Palate Clinics


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

Clinical Aspects of Cleft Lip/Palate Reconstruction

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