Transcript Surgical Aspects in the Management of Cleft Lip/Palate

Clinical Aspects of Cleft Lip/Palate
Reconstruction
Brian Clarke
MED II
Dalhousie University
Halifax, Nova Scotia
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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
Group II
Clefts of lip only
Clefts of palate only (2o)
Group III
Group IV
Clefts of lip,
alveolus, palate
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
1 in 11 (9%)
1%
One sibling and one parent
1 in 6 (16%)
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 10’s
Cleft Palate
IWK - 9-12 months of age
Hgb = 10g
Weight of 10lbs
Age 10wks
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 Cupid’s 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’ cupid’s 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: cont’d 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
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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