Escolar Documentos
Profissional Documentos
Cultura Documentos
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
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
Embryology of Clefting
Facial Development - 4th - 10th week of development
Formed by the fusion of five prominences Unpaired frontonasal process
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
4) End of week 9, rotate upward into a horizontal position and fuse with each other and primary palate to form secondary palate
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 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
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
Cleft Variants
Isolated Cleft Palate
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!
1 in 25 (4%)
2.5%
1 in 11 (9%) 1 in 6 (16%)
1% 15%
Environmental Factors
Viral infections (rubella) Teratogens (steroids, anticonvulsants, alcohol, retinoic acid derivatives)
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
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
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
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
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
Post-op Management
Inform the parents of: Scar contracture Erythema Firmness Avoid placing in direct sunlight until the scar fully matures
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
Surgical Management
Cleft Palate
Goal: Production of a competent velopharyngeal sphincter
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
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
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
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)