CHAPTER 05: CLEFT Flashcards

Question

What is the timing of lip repair?

Answer 1

Conventional 3 mths - Lip and ant palate 6 mths - Cleft in hard and soft palate Neonatal - within 48hrs Delaire 6-9mths - Lip and soft palate 12-18mths - Remaining palate Schweckendick < 1yr - Lip and soft palate 8yr - hard palate Gothenburg Lip and SP 3/12 HP 7/12 or 12/12 or 3/12 trial TOPS trial

Answer 2

Timing of Primary Surgery for Cleft Palate Multicentre RCT with parallel design - UK, Scandinavia and Brazil. 650 infants w isolated cleft palate medically fit for op @ 6 months & meet inclusion criteria, randomised to - Surgery at age 6 months, OR - Surgery at age 12 months. Main objective: determine whether surgery for cleft palate, using a specified technique, at age 6 months, when compared to surgery using the same technique age 12 months, improves speech development. Followed up at 12 months, 3 years and five years for assessment of the primary (age 5) and secondary outcomes.

Answer 3

Irritation to oral and nasal mucosa (daily cleaning, device checked weekly) Irritation to skin tape (duoderm to skin) Dislodged mould causing airway obstruction (hole made in palatal portion of mould to avoid this)

Answer 4

Devices that mould and manipulate alveolar palatal segments → narrows cleft and aligns alveolar processes. Passive devices - obturator plates - feeding plates Dynamic appliances - Latham appliance - Nasoalveolar moulding device

Answer 5

1. re-establishment of muscle continuity 2. re-creation of the normal landmarks of a lip 3. with minimal scars 1. Reconstruct the normal lip contour (Cupid's bow) 2. Restore vertical height of lip along philtral ridge (includes creation of a natural appearing philtral tubercle and avoiding whistle deformity) 3. Realign the fibers of orbicularis oris 4. Minimise the appearance of scars on lip 5. symmetrical alae (reposition displaced atrophic alar cartilages and alar base)

Answer 6

Rotation advancement technique Rotation of a flap (A flap) in the philtrum (non cleft side) downwards and advancement of a flap from cleft side (B flap) to meet the A flap Advantages - scars along & disguised by philtral columns - possible to adjust the degree of lip lengthening during surgery (cut as you go) - Secondary revision is possible by re-elevation and re-rotation Disadvantages - difficult technique to learn - places a scar across philtrum at nasal base - Lip too short with a good nostril sill, says Sommerlad

Answer 7

Most of Cupids bow and philtrum present - preserve these and throw nothing away Consider nasal deformity Remember the muscle alignment

Answer 8

Straight line, upper z-plasty, lower z-plasty, upper and lower z-plasties Straight line - Rose-Thompson - Mirault-Blair-Brown-McDowell Upper z-plasty - Millard (draw) - Wynn Lower z-plasty - Tennison-Randall (draw) - Le Mesurier - Noordhoff – Triangular Lateral Vermillion Flap Upper and lower - Skoog - Trauner

Answer 9

Triangular lower z-plasty Advantages - Relatively easy to learn Disadvantages - Not easy to adjust degree of lip lengthening intra-op - Scar crosses lower aspect of philtral column - Lip too long with a poor nostril sill, says Sommerlad

Answer 10

- deformity due to inadequate red margin / vermilion Surgical options - mucosal advancement from buccal sulcus, including mucosal VY advancement (Kapetansky PRS 1971) - Free graft from lower lip - Abbe flap

Answer 11

Primary / Secondary At time of lip repair / when nasal growth complete (open) McComb technique (PRS 1985) - Dissection over dorsum of nose b/t nasal cartilage and skin - Alar lift - Percutaneous mattress sutures through mobilised nasal cartilages and held in position with bolsters Tajima technique - Intranasal, reversed U-incision used to access nasal cartilages - A trapezoid suture is then placed between the cartilages to correct the nasal deformity Matsuma splints - uses splints to mould nasal cartilages - placed in the nostrils, pressure on lower laterals ↓ nasal deformity

Answer 12

Result of abnormal muscles (orbicularis oris, levator labii superioris alequae nasi and tongue) and asymmetric growth centre push - Widened alar base - Hypoplastic & flat alar cartilage, separation of domes at tip, loss of projection - No overlap of alar & upper lateral cartilages - Hypoplastic maxilla - Subluxed ala, rotated downwards - Caudal septum pulled towards non-cleft side - Flattened nasal bones

Answer 13

Aims - robust repair of palatal cleft - watertight closure of nasal lining - secure lip repair Usu age 6-11 Must correct orthodontics (mvmt of premaxilla and maxillary segments) before alveolar bone grafting Technique - raise gingival flaps - harvest cancellous autograft - pack into cleft, close flaps Complications - wound dehiscence, graft exposure, resorption

Answer 14

Presurgical orthopaedics Age 6: extraction of troublesome deciduous teeth, malocclusion correction of permanent teeth Age 11+: realign teeth, correct cross-bite, Le Fort I osteotomy, maxillary advancement or distraction

Answer 15

Insertion of bone graft into the alveolar gap at the site of the cleft. ``` Benefits o Helps with eruption of teeth o Stabilises alveolus o Support of the alar base and cartilages o Speech benefits o Restoration of 3D alveolar contour ```

Answer 16

Usually performed when the permanent canine is about to erupt (9 –11 yrs) ``` Cancellous bone graft harvest - tibial tuberosity - iliac crest Pack into alveolar defect Cover with local gingivoperiosteal flaps ```

Answer 17

Starts where the orthodontists left off Indication – problems with facial skeleton leading to malocclusion Distraction – over-corrects by 15-20%, 1mm/day, then consolidation phase Complications o Nerve damage (10% risk to mental nerve with mandibular distraction) o Bleeding o Nasal tip projection is increased – often a good thing in cleft patients

Answer 18

Gingivo-Periosteoplasty (=Gingivo-Alveoloplasty) At time of lip repair - create a watertight cavity that fills with blood then scars up. May be able to avoid ABG in 60%, but ?effect on canine eruption.

Answer 19

Bilateral defects in lip, alveolus and ant palate. Central segment of lip = remnant of medial nasal process of frontonasal prominence and consists of → 1. Soft tissue element → prolabium 2. Skeletal element → premaxilla (protrudes)

Answer 20

Presurgical orthopaedics / steristrips / lip adhesion no longer used. May require staged approach in wide clefts. Millard - 2 stage - Prolabium raised off underlying premaxilla. Superiorly raised forked flaps are created from lateral parts of prolabium. - These are then turned up laterally to run under the alar bases. - The muscle bundles from the lateral lip elements are then isolated and sutured to each other across the midline. - The central part of the prolabium is then replaced over the muscle to reconstruct the philtrum. - Banked flaps of alar base used later to lengthen columella Modified Manchester - 1 stage - used when prolabium is small - longitudinal straight line incision made down either side of the prolabium - prolabium is then sutured to lateral lip elements in layers

Answer 21

Palatal development = wk 7-10 - Palatal shelves (maxilla) are initially vertical - As the head grows, the neck straightens, the tongue falls away allowing the palatal shelves to rotate upwards into their normal horizontal positions - mediated by growth factors and hyaluronic acid - If tongue does not descend, upward rotation of palatal shelves is blocked →cleft palate

Answer 22

Hard palate consists of Ant: palatal process of maxilla Post: palatine bones CP = abnormality of 2ndary palate, posterior to incisive forarmen

Answer 23

Hard palate = - palatal processes of maxilla - horizontal plate of palatine bone - mucoperiosteum adheres tightly to bone by Sharpey's fibres Blood supply = greater palatine artery (from maxillary) through greater palatine foramen and runs towards incisive foramen Sensory supply (via pterygopalatine ganglion, maxillary nerve) - premaxilla = nasopalatine nerve - posteriorly = greater palatine nerve

Answer 24

1. Greater palatine arteries = main blood supply → pass anteriorly and medially from greater palatine foramen at the posteriolateral border of the palate 2. Sphenopalatine artery (ant) 3. Lesser palatine arteries (post)

Answer 25

Palate Muscles – 5 Pairs 1. Tensor veli palatini O: Eustachian tube, passes around hamular process of pterygoid plate I: Palatine aponeurosis - elevates soft palate – 1st Branchial arch CN V (rest are from 4th & 6th arches and supplied by superior laryngeal branch of X) ``` 2. Levator veli palatini O: Eustachian tube, descends downwards I: Palatine aponeurosis - Main functional muscle of palate - Elevates soft palate, closes opening b/t nasopharynx and oropharynx, opens auditory meatus ``` 3. Muscularis Uvulae small muscle, lies within uvula at centre of palate. May be small / bifid / absent in submucous CP 4. Palatoglossus O: palatine aponeurosis I: side of tongue - tenses soft palate and walls of pharynx in swallowing 5. Palatopharyngeus O: post nasal spine & palatine aponeurosis I: mucosa of uvula - shortens uvula & pulls it superiorly

Answer 26

Motor TVP = V (mandibular branch via medial pterygoid nerve) The other 4 pairs of muscles = X (pharyngeal branch of vagus through pharyngeal plexus) ``` Sensory (Maxillary branch of V) Greater palatine (hard palate) Nasopalatine (incisive region) Lesser palatine (soft palate) nerves ```

Answer 27

- lesser palatine arteries - ascending palatine branch of facial artery - palatine branches of ascending pharyngeal artery All muscles supplied by pharyngeal plexus (CN XI accessory and pharyngeal branches of vagus CN X) except TVP (mandibular nerve CN V)

Answer 28

Primary palate = lip, alveolus and hard palate anterior to incisive foramen - represents failure of mesenchymal penetration b/t medial and lat palatine processes & failure of fusion b/t maxillary and medial nasal processes Secondary palate = hard palate behind incisive foramen and soft palate - represents failure of lat palatine processes to fuse with each other and nasal septum / vomer Ant & post clefts may appear together / in isolation. Other rare clefts include Median clefts = failure of fusion of medial nasal swellings Oblique facial cleft = failure of fusion of maxillary and lateral nasal swellings

Answer 29

Complete → cleft involves full length of secondary palate Incomplete → involves a segment Unilateral → vomer is attached to one palatal shelf Bilateral → vomer is not attached Submucous cleft → palate looks normal but functions abnormally - Bifid uvula, notched hard palate, Zona Pellucida - 50% do not speak normally - May be picked up after tonsils & adenoids removed

Answer 30

``` CP (NOT CL or CL&P) may be assoc with: 1. Foetal alcohol syndrome 2. Anticonvulsant use 3. Maternal diabetes 4. Retinoic acid anomalies Some evidence that maternal folate supplements may ↓ incidence of CP ```

Answer 31

Incidence: 1 in 1000 Signs - bifid uvula - palpable notch in HP - blue line / furrow / translucency (zona pellucida) Calnan's classic triad = bifid uvula, shortened SP with muscle diastasis, groove in posterior palate Presentation - VPI (15%), feeding or hearing problems Treatment - Nothing / ENT / palatoplasty / pharyngoplasty

Answer 32

1. Velocardiofacial Syndrome (VCF, Di George) 2. Pierre Robin Sequence (small jaw → ↓tongue descent → CP) 3. Stickler’s Syndrome 4. Treacher-Collins Syndrome – more of a ‘Facial Cleft Syndrome’ 5. Apert’s syndrome – more of a Synostosis syndrome 5. Crouzon’s syndrome – more of a Synostosis syndrome (Down’s syndrome – not typically associated with cleft) Van der Woude syndrome is associated with CL or CLP NOT CP

Answer 33

Abnormal attachment of TVP + LVP, Eustachian tube is more horizontal and doesn't open normally during swallowing → negative pressure in middle ear collection → effusion → conductive hearing loss Treatment - Abx, hearing aid, myringotomy, grommets, adenoidectomy, palate repair doesn't necessary improve. Usu improves age 8 (drainage w gravity)

Answer 34

1. difficulty feeding - special teat, squeezy bottles 2. ear infections, hearing loss - audiology, grommets 3. dental problems 4. speech difficulties 5. cosmetic concerns - psychological support, follow-up / revision surgery

Answer 35

- Closure of oronasal fistula - Minimise maxillary growth retardation - Allow normal speech development - Facilitate velopharyngeal closure

Answer 36

``` Simple repair with no flaps Veau-Wardill-Kilner = Bardach (USA) - ‘V-Y Push back’ Von Langenbeck technique (1851) Medial von Langenbeck ```

Answer 37

Hard Palate - Repaired in 2 layers - Nasal mucosa sutured to vomer mucosa - Different techniques for oral surface Soft Palate Techniques - Straight line - direct closure - Unipedicled – V-Y or advancement - Bipedicled - Z-Plasties

Answer 38

Intra-velar Veloplasty - Edges of cleft are incised and muscles are released, realigned and repaired in 3 layers. 1. Nasal mucosa 2. Muscle 3. Oral mucosa - The lateral extent of muscle dissection is variable, some advocate as far as hamulus

Answer 39

``` GA, south-facing ETT, extend neck Dingman's gag throat pack mark incisions infiltrate with LA + adrenaline ```

Answer 40

Furlow Technique - Lengthening palate with 2 opposing Z plasties - First Z on oral mucosa and second on nasal mucosa. - Intervening muscle layer is included in the posterior based flap of each z plasty. - Muscle sling is reconstructed by suturing together muscle bundles contained in the posteriorly based flaps.

Answer 41

Sommerlad Technique - Complete repair by 6m when occlusive sounds are starting - Technique combines minimal HP dissection with radical retropositioning of velar musculature and tensor tenotomy - Uses operative microscope - 80% closed with incisions at margins of cleft without von Langenbeck incisions or mucoperiosteal flaps ``` Technique: o Expose TVP tendon o TVP tenotomy off hard palate and hamulus o Retroposition LVP o Suture oral to nasal mucosa ``` Results - secondary velopharyngeal rates 4.6% from 10% (but no one else can replicate these results!)

Answer 42

Airway - early (swelling) late (altered nasal morphology) Fistula - 0.34% - nasal escape, hypernasality, nasal regurg, deterioration of speech maxillary growth retardation VPI - pharyngoplasty (up to 20%, Sommerlad's figures of IVV = 4.6%)

Answer 43

Cohen PRS 1991 - multivariate statistical analysis of prevalance, aetiology and surgical management of palatal fistulas Risk factors - push-back palatoplasty (43%) - Veau 3&4 - operating surgeon Surgical management (recurrence rate 25%) - total revision of palate repair - local mucoperiosteal flap - vomerine flap - bone grafting + soft tissue closure - tongue flap

Answer 44

Acute - GA risks - bleeding (return to theatre, releasing stitches, packing) - breathing problems Late - fistula formation (25%) - fluid food regurg into nose - restrictive scarring, maxillary growth retardation - 20% - Speech - Hearing - Appearance - Feeding (Fistula) - Dental vs. Orthodontic vs. Max Facs - Psychiatric

Answer 45

MDT setting (plastic surgery / maxillofacial, ENT, orthodonist, dentist, speech therapist, audiologist, paediatrician, geneticist, psychologist, CNS. Speech Assessment - GOS.SP.ASS ’98 Resonance (hypernasal, hyponasal, mixed), Nasal Emission, Nasal Turbulence, Grimace; Consonant Production (labial, alveolar, post-alveolar, velar, glottal) Video fluoroscopy (age 4+) lateral soft tissue xray screening assesses upward movement of soft palate, shape & stretch normal = acute velar knee abnormal = concave posterior pharyngeal wall More useful says Per Hall, because you can see if genu is at 'ankle' (re-repair may not give you much more lengthening) or more anterior 'knee' (can consider re-repair) Nasoendoscopy (age 5+) flexible endoscope inserted through nose observe pattern and degree of VP orifice / sphincter closure Age of pt x 10 = likely % success of nasoendoscopy! Others: hearing assessment, MRI

Answer 46

- VPI is malfunction of the VP mechanism - Movement of soft palate and pharyngeal walls control air entry into nasopharynx during articulation - VPI results from inability to fully close valve → air escapes into nose → hypernasal speech VPI can be caused by disorders e.g. - anatomic, neuromuscular, mental disability, behavioural, combination Symptoms - hypernasality - audible nasal air escape - lack of voice projection and articulation

Answer 47

Non-surgical 1. Speech therapy Surgical 2. lengthening palate and improve function / palatal mvmt 3. reduce opening b/t nasal and oropharynx

Answer 48

1. eliminate hypernasality symptoms 2. eliminate audible nasal emissions 3. avoid complete obstruction of velopharyngeal port 4. allow for nasal breathing and nasal resonance

Answer 49

Non-surgical 1. Speech therapy 2. Prosthetics, palatal lifts, pharyngeal obturators (poorly tolerated) Surgical 1. lengthening palate and improve function / palatal mvmt - redirection of anteriorly displaced levator muscles (IVV palate re-repair) - palatal lengthening 2. reduce opening b/t nasal and oropharynx - posterior pharyngeal wall augmentation - sphincter pharyngoplasty - pharyngeal flap pharyngoplasty

Answer 50

1. intravelar veloplasty 2. double-opposing Z plasty 3. palate re-repair 4. V-Y pushback procedure (less used due to extensive mucoperiosteal stripping, affecting facial growth)

Answer 51

- better / more physiological muscle positioning → improve function - lengthen palate - started by Sommerlad Static - Posterior wall augmentation Dynamic - Pharyngoplasty

Answer 52

Static vs Dynamic 1. Pharyngeal Flap (static) - creates single subtotal central obstruction of pharyngeal port, leaving 2 lateral ports open ~0.5cm - suitable for pts with satisfactory lateral wall motion - modifications of this procedure - superior (better) vs inferiorly based flaps, different widths of flaps, mucosal lined vs raw surface - flap raised in plane of prevertebral fascia and inset into reflected flaps from soft palate or nasal mucosa - overcorrection → mouth breathing, hyponasality, OSA 2. Posterior Wall Augmentation (static) - rib cartilage, fat, rolled dermis, teflon, silicone 3. Sphincter Pharyngoplasty (dynamic) - diminishes cross-sectional area of the central port - suitable for pts with poor posterior pharyngeal wall movement, smaller VP ports, good velar elevation, poor lateral wall motion

Answer 53

``` O2 nasal cannula overnight monitoring pulse oximetry liquid / soft diet sleep head up oral hygiene ```

Answer 54

Hynes pharyngoplasty (1951) - static actually - superiorly based mucosal flaps from posterior tonsillar pillars (salpingopharyngeus) - preserve vagus nerve branches - flaps 3-4cm long, 1.5cm wide - inset into midline horizontal incision in post pharyngeal wall above Passavant's ridge, one above the other - donor closed directly Orticochea Modification (1968) - palatopharyngeus myomucosal flaps raised and inset into an inferiorly based posterior pharyngeal flap - donors heal by secondary intention - aim to keep central orifice opening ~1cm - check with suction in nasopharynx technique (Sommerlad) Jackson Modification (1985) - palatopharyngeus flaps sutured end-end - inset into transverse incision level with upper margin of tonsillar fossa under a superiorly based mucosal flap - only 1/3 retain significant muscle contraction, therefore most cases are not dynamic sphincteroplasty

Answer 55

Success rates - pharyngeal flap surgery 80-90% - sphincter pharyngoplasty 40-60% - Furlow >90%

Answer 56

1. Bleeding / haemorrhage - always palpate posterior pharyngeal wall for aberrant ICAs VCF syndrome 2. respiratory obstruction 3. obstructive sleep apnoea 4. wound dehiscence (increased incidence post T&A) 5. persistent hypernasality (undercorrection) 6. hyponasality (overcorrection) 7. speech may be worse 8. catarrh 9. difficulty breathing through nose

Answer 57

SYNDROME - A group of anomalies that contains multiple malformations of sequences. - A given anomaly may be incompletely expressed or absent. - Pathogenic relationship frequently not understood. SEQUENCE - A single developmental defect resulting in a chain of secondary defects. e. g. Pierre Robin → mandibular hypoplasia → post displaced tongue →airway obstruction + precludes closure of the palatal arches → cleft of secondary palate.

Answer 58

Mandibular hypoplasia* (usu retrognathia not micrognathia) → posteriorly displaced tongue → airway obstruction & preclusion of closure of palatal arches → cleft of secondary palate. - First described in 1822 - 1 in 8000 live births - Risk of further children affected = 1-5% - Can often be part of a larger syndrome (Stickler, Treacher-Collins, VCF, Craniofacial Microsomia) - Often grow by 3-18mths → normal profile by 6yrs. Mandibular advancement osteotomy rarely required - Palate repair at 12-18 months - Defects of ear and eye - Glossoptosis - Airway obstruction

Answer 59

MDT setting, cleft nurse Prenatal screening and counselling / see within 24hrs of birth Immediate Mx o Minor – lay on side, tongue falls away o Moderate – cannot feed needs NG tube o Severe – nasopharyngeal airway Airway obstruction (sleep and feeding) → FTT Nurse face down Apnoea monitor Emergency → tongue stitch/towel clip, NPA, nasotracheal intubation, Routledge stitch (suture to inner surface of lower lip), tracheostomy – rarely needed Palate repair later than normal

Answer 60

``` Velocardiofacial Syndrome (=DiGeorge = Shprintzen Syndrome) CATCH 22 C → Cardiac & Carotid anomalies A → Abnormal facies T → Thymic aplasia C → Cleft palate H → Hypocalcaemia 22 → Chromosome 22qll deformity – usually deletions on FISH test, rarely duplications (FISH test doesn’t spot these). ``` - Long face - flat in shape and expression - Epicanthic folds - Pinched nasal tip - 25% have abnormally positioned carotid arteries, deviated to the midline → hazard in post pharyngeal wall surgery - 10% psychosis

Answer 61

- AD - Associated with cleft lip and palate* - Multiple lip pits - Absent second premolars - Popliteal pterygia (webbing)

Answer 62

Hereditary Progressive Arthro-Ophthalmopathy - AD chr 12 - Dr. G.B. Stickler Mayo clinic 1965 - Severe progressive myopia, vitreal degeneration, retinal detachment - Progressive sensorineural hearing loss - Valvular prolapse - Scoliosis - Pierre Robin features → cleft palate, mandibular hypoplasia - Hyper and hypomobility of joints - Variable epiphyseal dysplasia →joint pain, dislocation or degeneration - Consider in any infant with congenitally enlarged wrists, knees or ankles, esp. if Pierre Robin, or anyone suspected of Marfan syndrome who has hearing loss, degenerative arthritis or retinal detachment.

Answer 63

12cm muscular tube, skull base to C6 level 4 layers = mucous membrane, submucous, muscular, buccopharyngeal fascia Muscles = overlapping superior, middle and inferior constrictors Blood supply = sup and inf laryngeal, ascending pharyngeal, arteries to palate Motor supply = pharyngeal plexus (pharyngeal br of X + XI) except stylopharyngeus (IX) and cricopharyngeus (recurrrent laryngeal br of X)

Answer 64

- Short neck, missing vertebrae | - CP (probably as a result of reduced mandibular movements)

Answer 65

pharyngeal tonsil = adenoids = post wall of nasopharynx palatine tonsils = b/t ant and post pillars in oropharynx Waldeyer's ring = lingual + tubal + palatine + pharyngeal tonsils

Answer 66

Examine baby o Airway – accessory muscles; intercostal recession; tracheal tug; paradoxical breathing; pale; tired; ↓feeding; ±stridor o Hypercapnia = breathing drive in baby o Cyanosed = hypercapnic o O2 sats useless o Therefore remove all clothes and examine baby in different positions

Answer 67

Whilst examining take history from parents (for syndromes), inc. FH Feeding – “how are you coping?” o Babies feed by milking the breast – easier to feed if multiparous with strong milk letdown reflex o Principles of feeding = gravity and compression

Answer 68

Toronto Sick Kids 1. Small central defect, high closure rating → FURLOW'S PALATOPLASTY. 2. Coronal closure patterns (poor lateral wall mvmt and lateral port deficiency) → SPHINCTER PHARYNGOPLASTY. 3. Coronal closure pattern and low closure rating → PHARYNGEAL FLAP PHARYNGOPLASTY. GOSH 1. Anteriorly inserted levators → PALATE RE-REPAIR. 2. Levators normally positioned / palate re-repair not enough to correct VPI → MODIFIED HYNES PHARYNGOPLASTY (posterior pharygeal wall augmentation pharyngoplasty)

Answer 69

Fraction of diameter of VP port that is closed off during attempted sphincter closure (Expressed in increments of 0.1 from 0-1.0)

Answer 70

It is ratio of nasal acoustic energy over nasal + oral acoustic energies (expressed as %age). It provides an objective measure (nasalance).

Answer 71

HP - greater palatine artery. SP - ascending palatine artery.