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Plastics - Cleft lip and Palate - Coggle Diagram
Plastics - Cleft lip and Palate
Embryology
Lip and anterior palate form at 4-5 weeks (B)
Seconday palate (posterior to incisive foramen) forms at 6-12 weeks (C)
Midface fuses outward from the incisive foramen - determines patterns of clefts
Orofacial cLefts
Epidemiology
16 per 10,000 births in Ireland
50/50 cleft lip + palate or just cleft palate
2/3 are an isolated anomaly in normal child
1/3 associated with syndrome, chromosome anamoly or other anaomlies (isolated cleft palate more likely)
CL+P and CP are genetically seperate condition
Mostly multifactorial threshold model of inheritance
Range of Clefts
Normal
Forme fruste
Incomplete cleft lip
Gum affected
Complete unilateral cleft lip and palate
Nasal septum affected - deviated - attached to main segment
Complete Bilateral Cleft lip and palate
Isolated cleft palate - bifid uvula
Complete cleft of soft palate
Complete cleft of hard and soft palate
Effects of Clefts
Airway (Pierre Robin Sequence)
Feeding
Speech and language
Hearing
Cosmestic
Dentition
Facial growth
Pierre Robin Sequence
Features
U shaped cleft palate
Glossoptosis
Retrognathia
Presentation
Stridor
Respiratory distress
Airway distress when supine - tongue obstructs airway
Airway distress during feeding - stress and disruption of suck, swallow, breath co-ordination
Mangement
No force feeding
NG tube
Prone / lateral positioning temporarily
Severe cases may require intubation and transfer to PICU
Transfer to cleft centre for MDT assessment
Maintaining airway : nasopharyngeal tube
MDT
Admission under joint care of neonatology and cleft team
ENT - assess need for nasopharyngeal airway (NPA) / tracheostomy
May need to exlude other airway pathology
Identify co-existing conditions (cardiac)
Resp med - Sleep study before and after NPA if needed
Sleep study needs to be satisfactory before discharge home
SLT and cleft nurse specialist :Assess for oral feeding
Don't discharge child dependent on prone position
Parents are trained in care of NPA for discharge
Feeding
NG tube initially - rest airway and avoid stress - oral aversion syndrome
SLT and cleft specialist nurse will assess feeding and institute oral feeding safely (special bottles and teats)
Gastro-osopahgeal reflux very common in Robin sequence
Avoid prolonged NG intubation
Reflux
Aspiration
Oral aversion syndrome
Consider PEG if tube feeding will be prolonged
Surgery
Repair palate at 6-9 months
Mandible catches us
In general don't do tongue-lip adhesion procedures or noenatal mandible distraction
Stickler's Syndrome
Screening
All isolated cleft palates need an opth assessment
Genetics
AD inheritance usually
Clinical Features
Fhx of eye problems
Myopia
Retinal detachment
Fhx of cleft palate and Pierre robins
Floppy joints
Early arthritis
Treacher Collins Syndrome
Genetics
Autosomal domiant
TCOF1 gene (treacle) ribosome deficiency in developing face
Clinical features
DEficienct zygomatic bones
Mandibular hypoplasia
Deficient eyelids (lower)
External ear abnormalities
Sevre middle ear anomalies / deafness
Complete Cleft Lip and Palate
MDT
Plastic surgeon
Specialist cleft nurse
Speech therapist
Orthodontist, specialist dentists
Maxillofacial surgeon
Cleft co-ordinator
Problems
Feeding
Less airway problems
Appearance / cosmetics
Management
Palate Function in Speech
Controls nasality of vowels - oral verus nasal resonance
Aloows oral pressure consonants
Failure of the Veolpharyngeal Valve
Hypernasal resonance
Nasal Emmision (audible or inaudible)
Failure to make oral pressure consonants
Maladaptive compensatory articulation patterns (this my be difficult to correct even if palate function is restored)
History + Exam
Early infant feeding
Breast or bottle
How was it? Compare with siblings
Quiet nasal regurgitation of milk during
settled
feeding?
PMHx
Reurrent ear infections
Failed hearing test
22q11 deletion - di George, Velocardiofacial etc
Exam
Look
Bifid uvula
Obvious cleft
Zona pellucida
Feel
Palpable notch in the midline at junction of hard and soft palate
Di George Syndrome - 22q11.2 Microdeletion
Features
Dysfunction of structures derived from branchial arch neural crest
Underdeveloped facial muscles
Deficienct parathyoids
Thymic deficiency - immune deficiecy
Palatal defects
Cardiac outflow tract anaomalies (Tetralogy of fallot)
Complex Neurodevelopment defects
Psychiatric illness
Lower Iq
Social functioning issues
