CLEFT LIP AND PALATE Grand Rounds Presentation by Greg Young, M.D. Ronald Deskin, M.D.

Introduction l Facial clefting is the second most common congenital deformity (after clubfoot). l Affects 1in 750 births l Problems are cosmetic, dental, speech, swallowing, hearing, facial growth, emotional l Otolaryngologist holds key role on CP team

Anatomy l Hard Palate –Bones: Maxilla( Palatine Processes) + Palatine Bones(Horizontal Lamina) –Blood Supply: Greater Palatine Artery –Nerve Supply: Anterior Palatine Nerve

Anatomy l Soft Palate –Fibromuscular shelf attached like a shelf to posterior portion of hard palate –Tenses, elevates, contacts Passavants Ridge –Muscles: Tensor Veli Palatini(CNV), Levator Veli Palatini(Primary Elevator), Musculus Uvulae, Palatoglossus, Palatopharyngeus(CN IX and X)

Embryology l Primary Palate- Triangular area of hard palate anterior to incisive foramen to point just lateral to lateral incisor teeth –Includes that portion of alveolar ridge and four incisor teeth. l Secondary Palate- Remaining hard palate and all of soft palate

Embryology l Primary Palate –Forms during 4th to 7th week of Gestation –Two maxillary swellings merge –Two medial nasal swelling fuse –Intermaxillary Segment Forms: Labial Component(Philtrum) Maxilla Component(Alveolus + 4 Incisors) Palatal Component(Triangular Primary Palate)

Embryology l Secondary Palate –Forms in 6th to 9th weeks of gestation –Palatal shelves change from vertical to horizontal position and fuse –Tongue must migrate antero-inferiorly

Cleft Formation l Cleft result in a deficiency of tissue l Cleft lip occurs when an epithelial bridge fails l Clefts of primary palate occur anterior to incisive foramen l Clefts of secondary palate occur posterior to incisive foramen

Cleft Formation l Secondary Palate closes 1 week later in females l Cleft of lip increases liklihood of cleft of palate because tongue gets trapped.

Unilateral Cleft Lip l Nasal floor communicates with oral cavity l Maxilla on cleft side is hypoplastic l Columella is displaced to normal side l Nasal ala on cleft side is laterally, posteriorly, and inferiorly displaced l Lower lat on cleft side -lower, more obtuse l Lip muscles insert into ala and columella

Palatal Clefts l Soft palate muscles insert on posterior margin of remaining hard palate rather than midline raphe. l Associated Dental Abnormalities –Supernumery Teeth- 20% –Dystrophic Teeth- 30% –Missing Teeth- 50% –Malocclusion- 100%

Genetics l Non-syndromic inheritance is multifactorial –Cleft Lip, With or Without Cleft Palate: One Parent-2% One Sibling- 4% Two Siblings- 9% One Parent + One Sibling- 15% –Cleft Palate: One Parent- 7% One Sibling- 2% Two Siblings- 1% One Parent + One Sibling- 17%

Genetics l Increased clefts with chromosome aberations l Clefts a part of a Syndrome 15-60% of time l More than 200 syndromes include clefts l Cleft Palate- Aperts, Sticklers, Treacher l Cleft Lip +/- Palate- Van der Woudes, Waardenbergs

Epidemiology l Cleft Lip +/- Palate- 2 Male: 1 Female l Cleft Palate - 2 Female: 1 Male l Cleft Lip +/- Palate- Native Americans > Oriental and Caucasians > Blacks l Cleft Palate- Same among ethnic groups l Environmental: Ethanol, Rubella virus, thalidomide, aminopterin

Epidemiology l Increased Clefts with maternal diabetes mellitus and amniotic band syndrome l Increased Clefts with increased paternal age l Cleft Lip + Palate- 50% l Cleft Palate- 30% l Cleft Lip- 20% l Cleft Lip + Alveolus- 5%

Management l Team Approach l Otolaryngologist has a pivotal role l Initial Head and Neck Examination l Speech Disorders l Ear Disease l Airway Problems l Surgical Repair

Head and Neck Exam l Head- facial symmetry l Otologic- auricle and canal development and location, pneumatic otoscopy, forks l Rhinoscopy- identifies clefting, septal anomalies, masses, choanal atresia l Oral Exam- cleft, dental, tongue l Upper airway- phonation, cough, swallow

Speech Disorders l Errors in Articulation: Fricatives, Affricates l Velopharyngeal Competence- Most important determinant of speech quality in cleft palate patients-75% achieve competence after initial palate surgery l Incompetence- nasal emission or snort l Evaluation- Direct exam, Fiberoptic Exam

Ear Disease l Cleft Lip- Incidence similar to normal pop. l Cleft Palate- Almost all with ETD, CHL l ETD- Due to abnormal insertion of levator veli palatini and tensor veli palatini into posterior hard palate l ETD- Returns to normal by mid-adolescent l Cleft Palate- Increased Cholesteatoma(7%)

Ear Disease l Otologic Goals For Cleft Palate Patients –Adequate hearing –Ossicular chain continuity –Adequate middle ear space –Prevent TM deterioration l Indications for Myringotomy Tubes –CHL, Persistent/Recurrent effusion, Retraction –Cleft palate: Multiple BMTs from 3mo yrs

Airway Problems l More common in Cleft Palate patients with concomitant structural or functional anomalies. l e.g. Pierre-Robin Sequence –Micrognathia, Cleft Palate, Glossoptosis –May develop airway distress from tongue becoming lodged in palatal defect

Surgical Repair- Cleft Lip l Lip Adhesions- –2 weeks of age –Converts complete cleft into incomplete cleft –Serves as temporizing measure for those with feeding problems –May interfere with definitive lip repair –Less often needed in recent years due to wider variety of specialty feeding nipples

Surgical Repair- Cleft Lip l Cleft lip repaired at 10 weeks l Rotation-advancement method- Most common in the U.S. l Nine Landmarks l Rotation Flap cuts made first l Advancement cuts made next l Cleft side nasal ala cuts made last

Surgical Repair- Cleft Palate l Several Techniques- Trend is towards less scarring and less tension on palate l Scarring of palate may cause impaired mid-facial growth(alveolar arch collapse, midface retrusion, malocclusion) l Facial growth may be less affected if surgery is delayed until months, but feeding, speech, socialization may suffer.

Surgical Repair- Cleft Palate l Bardach Method- Two Flap technique –Medial incisions made, which separate oral and nasal mucosa –Lateral incisions made at junction of palate and alveolar ridge –Elevate flaps, preserve greater palatine artery. –Detach velar muscles from posterior palate –Close in 3 layers

Non-Surgical Treatment l Dental Obturator –For high-risk patients or those that refuse surgery. –Advantage- High rate of closure –Disadvantage- Need to wear a prosthesis, and need to modify prosthesis as child grows.

Conclusions l Cleft Lip and Palate are common congenital deformities that often affect speech, hearing, and cosmesis; and may at times lead to airway compromise. l The otolaryngologist is a key member of the cleft palate team, and is in a unique position to identify and manage many of these problems.