Summary

This document provides an overview of cleft lip and palate, including its development, types, causes, and associated syndromes. It also covers diagnosis and potential management strategies.

Full Transcript

# CLEFT LIP + PALATE - Develops between week 6-12 - Fusion of palatal shelves - Development from 1<sup>st</sup> pharyngeal arch + frontonasal process - Formed from 3 processes: - 2 maxillary processes - 2 palatine processes of max processes - Primitive palate formed from intermaxillary p...

# CLEFT LIP + PALATE - Develops between week 6-12 - Fusion of palatal shelves - Development from 1<sup>st</sup> pharyngeal arch + frontonasal process - Formed from 3 processes: - 2 maxillary processes - 2 palatine processes of max processes - Primitive palate formed from intermaxillary process (premaxilla) - Palate mesoderm -> intramembranous ossification -> hard palate formation - No ossification in posterior part = soft palate - Muscles of soft palate derived from 4<sup>th</sup> pharyngeal arch - Defective fusion of processes = cleft palate - Bilateral = failure of fusion of both palatine processes + premaxilla = Y shaped cleft - Unilateral = non-fusion of one side of palatine process of maxilla w premaxilla - Incomplete = cleft limited to soft/hard palate or bifid uvula - Cleft lip = cleft of primary palate anterior to incisive foramen -> lack of fusion of medial nasal processes of frontal nasal prominence w maxillary process - Nose cleft = intermaxillary segments fail and/or lateral nasal process fail to fuse ## Diagram The document includes 5 diagrams, illustrating various types of cleft lip and palate: **Diagram 1:** - Shows a bilateral cleft palate with cleft lip, where the palatine processes (A) and the premaxilla (B) are not fused on both sides. **Diagram 2:** - Shows an incomplete cleft palate (involving both hard and soft palate), where the palatine processes (A) are fused on one side but not on the other. **Diagram 3:** - Shows a unilateral cleft palate with cleft lip, where the palatine processes (A) and the premaxilla (B) are not fused on one side. **Diagram 4:** - Shows an incomplete cleft palate (involving soft palate), where the palatine processes (A) are fused on one side and the premaxilla (B) is not fused to the palatine processes on the other. **Diagram 5:** - Shows a bifid uvula, where the uvula is split into two parts. ## Cleft as part of syndrome - **Van der Woudes**: lip pit, missing teeth, cleft palate, delayed language development - **Pierre Robin anomaly**: micrognathia, natal teeth, glossoptosis, high arched/cleft palate - **Di George Syndrome (velocardiofacial)**: wide eyes, distinctive ears, congenital heart defect, infection prone ## Cleft epidemiology - **1: 700 UK** - **70% non-syndromic** - **Male: female = 2:1** - **L: R = 2/3: 1/3** ## Microform cleft - Looks like little dent - Muscle tissue underneath can be affected -> surgery ## Submucous cleft palate - No midline/no muscle - Associated w bifid/cleft uvula ## Post nasal spine almost always missing ## Common speech problems ## Prob in dent: - Feeding - Hearing - Speech - Disruption of face growth - Disruption of dent development + dent anomalies - Psychosocial ## Local dent problems: - Congenital missing teeth - Hypodontia - Oligodontia - Hyperdontia - Presence of natal/neonatal teeth - Anomalies of tooth morph -> microdontia, macrodontia - Enamel hypoplasia - Poor periodontal support -> early teeth loss - Gemination - Dilacerations ## Ortho problems: - Class III - Ant/post crossbite - Spacing + crowding ## Pre-natal diagnosis: - 81% of cleft lip diagnosed before birth - Cleft palate almost impossible to identify before birth ## Management: ### Birth: - Counselling - Feeding plate - Pre-surgery assessment ### 3m: - Primary lip repair ### 9-18m: - Palate repair ### 1-3y: - Speech therapy ### 3-6y: - Speech therapy - Masednoscopy and/or pharyngoplasty ### 8-9y: - Initial interventional ortho - Prep for alveolar bone grafting ### 10y: - Alveolar bone grafting ### 12-14y: - Definite ortho ### 17-20y: - Orthognathic surgery - Nasal revision surgery ## Impressions: - Taken to form part of the cleft record - Compares cleft in future - Part of CSAG audit ## Pre-surgical orthopaedics: - Maxillary strapping - Taping - Nasoalveolar moulding appliances ## Aims of surgery: - Restore normal anatomy - Minimise adverse effects - Reconstruct normal muscle - Make cleft unrecognisable ## Surgery: "rule of 10" - Primary repair around 10w - Weigh 10lb - Haemoglobin 10g - White count no higher than 10k - At least 10 weeks of age ## Alveolar bone grafting: - Timed to precede canine eruption on cleft side - Around 11y - Makes bony bridge across cleft so canine can erupt into new bone and to support periodontium ## Orthognathic surgery: - 18y+ - Almost always to correct class III - Can compromise velopharyngeal function

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