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23 Cards in this Set
- Front
- Back
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Cleft description |
LAHSAL - LipAlveolusHardSoftpalateAlveolusLipupper case denotes complete lahsal - lower case denotes incomplete |
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Cleft Palate in Isolation |
- 1 in 2000 births - Affects hard/soft/ both - Associated with syndromes |
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CL&P : Overview |
- Affects 1 in 600/700 babies - 1000 births per year in UK - 2 distinct presentations *cleft lip +/- cleft palate *isolated cleft palate |
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CL&P : Aetiology |
Genetic - family history in 40% of cases Environmental - possible causes include anticonvulsant drugs, nutritional deficiency,anaemia, alcohol, low socio-economic status. Probably a genetic predisposition triggered byenvironmental factors |
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Sub mucous cleftpalate |
- 1 in 12000 births - Not obvious and may not besuspected until child is older - Usually a previous history ofproblems withfeeding/failure to thrive Diagnostic features – notchon palatal shelfbifid uvula ‘blue translucent zone’or at surgery |
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CL&P :Diagnosis |
Ante natal scan, around 20/40 weeks - 20% Cleft lip (uni or bi lateral) - 50% CLP (uni or bilateral) - 30% Cleft Palate IN ADDITION 15 – 40% of cleft children haveassociated anomalies |
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Syndromes associated with cleft palate |
Van de Woude - Autosomal dominant - Lip Pits - CP - linked with cardiac anomalies - hypodontia Pierre Robin Sequence - Cleft palate - Glossoptosis - Mandibular retrognathia - Around 1/3 of cases are linked to - Sticklersyndrome- a connective tissue disorder Foetal Alcohol Syndrome |
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Organisation of Cleft care in UK |
- CSAG 1998 recommended re organisation of cleftcare – fewer centres caring for larger volume ofcleft patients. - 10 regional cleft units: - Trent regional cleft network set up 2000: - hub in Nottingham - spokes in Sheffield, Doncaster, Chesterfield,Lincoln, Boston, Derby, Leicester, |
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Neonatal period |
- Breathing - Feeding - Comorbidity insyndromic babies - Notification of cleftservice – Cleft NurseSpecialist review - Paediatrician review |
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Secondary interventions |
Cleft lip and palate repair - When the baby isthriving - Lip from 3 months - Posterior palate from 6months |
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Surgery |
Unilateral cleft lip and palate: lip and anterior palate repair - Soft tissue mobilisation - Vomerine flap and septal centralisation Isolated cleft palate repair - Incisions - Flap elevation (Von Langenbeck flaps raised off hard palate and muscles in soft palate *Greater palatine artery) Muscle dissection - Nasal mucosal closure - Muscle dissected off nasal mucosa undermicroscope - Muscle bundles rotated from oblique totransverse - Left and right muscles sutured together acrossthe cleft Nasal mucosa and muscle repair Muscle retropositioning Bioguide collagen membrane Oral layer closure Orthodontic intervention– Neo natal :Lip strapping Multidisciplinary Cleft Team Primary Cleft Surgeon Specialist Cleft NurseSpeech and language therapist Paediatric Dentist Orthodontist Secondary Cleft SurgeonPaediatrician, Clinical GeneticsPsychologist, ENT, Audiology,GMPGDP/CDS, Other paediatric specialists |
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Baby – toddler – school age |
- monitor speech development - ENT assessment/ hearing tests - NB : glue ear - dental health education - Pharyngoplasty or revision surgery to assist speech where indicated |
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Incidence of speech problems |
- 2/3rds of all children with cleftpalate repairs receive speechtherapy (Sell et al 2000). - Around half of all children withcleft palate will have persistentspeech difficulties - Speech can be nasal or producedtoo far back in the mouth ….. but speech difficulty notrelated to defect size. |
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Speech surgery |
Velopharyngeal function Speech surgery: - palatal lengthening - pharyngoplasty |
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6 to 10 years |
- 7 yr combined clinic appointment - dental development and regular dental care hypodontia / supernumeraries / microdontia in cleft site - orthodontic assessment - alveolar bone graft as necessary - speech and language therapy - ENT monitoring |
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11-20+ years |
- Support for transition to secondary school - Definitive orthodontic treatmentOr - Combined orthodontic/ orthognathic surgery - Rhinoplasty as appropriate - Referral to clinical geneticist if appropriate |
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Dental and Orthodontic problemsin cleft lip / palate |
Dental disease, poor oral hygiene - Cleft children have 2x bad teeth as peers despite 90% registered with dentist, 40% of 5yr olds active untx caries - poor OH Missing/malformed/extra teeth - Missing upper permanent lateralincisor occurs in 30-50% of cleftcases (5%in a non cleft population). - 54% of cleft cases have dentalanomalies (15% in non cleftpopulation). Unusual paths of eruption/ intra oral appearance Skeletal factors – Adverse effects of facial growthand previous surgery. - Scarring of soft tissues - Restriction of maxillary development - Mandibular development Dental occlusion and alignment. - Lack of bone support - Scarring and collapse of maxillary segment(s) |
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ORTHODONTIC TREATMENT |
- Preparation for alveolar bone graft age 9-11 yrs - Definitive orthodontics age 13 + yrs - Orthodontic/orthognathic surgery when growthcomplete. |
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Orthodontic preparation for ABG |
- Age 9-11 years, before permanentcanines erupt. Orthodontic preparation to - correct transverse maxillary archdiscrepancy where indicated. - open cleft site to facilitate placementof bone graft. (Alveolar Bone Graft surgery - ABG) - align rotated incisors if bone supportadequate. 13-15 yrs :Definitive orthodontic treatment *Treatment options totally dependent on quality of primary and secondary cleft surgery* |
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Adverse facial growth - Orthognathic option |
Orthognathic surgery in cleft patients: - Full speech assessment essential to estimate riskof speech deterioration. - Video fluoroscopy (2 d) - Nasendoscopy (3d) - Psychological assessment recommended by CSAG - Any other procedures eg.rhinoplasty, deferreduntil orthognathic phase complete. *Older cleft patients* - complain of nasal drip, food coming down nose, unhappy with appearance |
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Structure of cleft care in theUK |
- CSAG report 1998 looked at - provision of cleft care in UK - treatment outcomes for 2 age groupswith UCL&P. |
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Final result |
- Any restorative - Dental reconstruction (implants?) - Lip revision - Ala follows the orbicularis - Rhinoplasty lip revision - Life-long care |
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A case |
- Born with cleft lipand palate (1 in every 700 babies with cleft worldwide) - Problems eating anddrinking (special bottle) - At 12 weeksold I had my firstoperation at SheffieldChildren’s Hospital.The plastic surgeonsrepaired my lip- took 4 hours. - 2 kinds of stitches, including‘bolster’ stitches which are the white blobs onmy nose. had to have plastic splints on my arms tostop me from scratching and pulling thestitches out. - I stayed in hospital with my mum for a few days - After a couple of weeks I had the stitches out. - Having a cleft palate meant I had a huge holein the roof of my mouth - For years there were things I could not eatlike bread and ice cream because they gotstuck in the hole or because it hurt too much. - When I was 10 months old I had anotheroperation in Sheffield to mend my soft palate. - I had to learn how to talk with the speechtherapist every week. - When I was 3 I had my hard palate repaired. This is the roof of the mouth at the front - Having a cleft lip and palate also means Isometimes have problems hearing. I had to have another operation to putgrommets in my ears - When I was 5, I had togo to Nottingham foranother operation onthe back of my mouth - helpedme do better withspeech therapy. - I go to see the specialists regularly to check thateverything is ok. I had to have new grommets put in because thefirst pair came out! - When I was in Year 4, I hadanother big operation inNottingham. The maxilofacial surgeontook a piece of bone out ofmy hip and put it into thegap in my jaw. I had 2 teeth out at thesame time. - This year I have had lots of appointments with the orthodontist, They gave me a brace tohelp straighten my teeth. - Sometimes people don’t understand what it’sbeen like for me and they make fun of the wayI talk. - A lot of children don't have access to the healthcare I have had. |
