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Questions and Answers
Which of the following characteristics is NOT associated with Van der Woude syndrome?
Which of the following characteristics is NOT associated with Van der Woude syndrome?
What percentage of cleft cases are classified as non-syndromic?
What percentage of cleft cases are classified as non-syndromic?
Which oral health issue is specifically associated with submucous cleft palate?
Which oral health issue is specifically associated with submucous cleft palate?
At what age is the primary repair of the lip typically performed?
At what age is the primary repair of the lip typically performed?
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What is the main purpose of alveolar bone grafting in cleft patients?
What is the main purpose of alveolar bone grafting in cleft patients?
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Which of the following complications can arise following orthognathic surgery?
Which of the following complications can arise following orthognathic surgery?
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What age is the peak time for initiating interventional orthodontics in a cleft patient?
What age is the peak time for initiating interventional orthodontics in a cleft patient?
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Which of the following is a common psychosocial problem in patients with clefts?
Which of the following is a common psychosocial problem in patients with clefts?
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Which condition is a characteristic feature of Di George syndrome?
Which condition is a characteristic feature of Di George syndrome?
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What is the recommended weight for primary cleft repair according to the 'rule of 10'?
What is the recommended weight for primary cleft repair according to the 'rule of 10'?
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What is the primary anatomical feature involved in the formation of cleft lip?
What is the primary anatomical feature involved in the formation of cleft lip?
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At what stage of embryonic development does the formation of the palatal structures occur?
At what stage of embryonic development does the formation of the palatal structures occur?
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Which of the following describes a bilateral cleft palate?
Which of the following describes a bilateral cleft palate?
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What type of ossification process is responsible for the formation of the hard palate?
What type of ossification process is responsible for the formation of the hard palate?
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Which embryonic structure is NOT involved in the formation of the cleft palate?
Which embryonic structure is NOT involved in the formation of the cleft palate?
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What feature distinguishes an incomplete cleft palate from a complete cleft palate?
What feature distinguishes an incomplete cleft palate from a complete cleft palate?
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From which embryonic origin are the muscles of the soft palate derived?
From which embryonic origin are the muscles of the soft palate derived?
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What type of cleft is characterized as being limited to the soft or hard palate, or a bifid uvula?
What type of cleft is characterized as being limited to the soft or hard palate, or a bifid uvula?
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Which option correctly identifies how a unilateral cleft palate occurs?
Which option correctly identifies how a unilateral cleft palate occurs?
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What process is responsible for developing the primitive palate?
What process is responsible for developing the primitive palate?
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What is a significant psychosocial issue faced by individuals with clefts?
What is a significant psychosocial issue faced by individuals with clefts?
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Which syndrome is characterized by missing teeth and a cleft palate?
Which syndrome is characterized by missing teeth and a cleft palate?
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Which age range typically sees the initial stages of speech therapy for cleft patients?
Which age range typically sees the initial stages of speech therapy for cleft patients?
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What anatomical feature is most commonly absent in individuals with a submucous cleft palate?
What anatomical feature is most commonly absent in individuals with a submucous cleft palate?
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What is the significance of the 'rule of 10' in the context of cleft surgery?
What is the significance of the 'rule of 10' in the context of cleft surgery?
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What dental condition may result from poor periodontal support in cleft patients?
What dental condition may result from poor periodontal support in cleft patients?
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Which condition is almost impossible to diagnose prenatally?
Which condition is almost impossible to diagnose prenatally?
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What is the primary aim of surgery for cleft repairs?
What is the primary aim of surgery for cleft repairs?
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At what age is alveolar bone grafting typically performed in relation to canine eruption?
At what age is alveolar bone grafting typically performed in relation to canine eruption?
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What orthodontic issue is often seen in cleft patients related to tooth alignment?
What orthodontic issue is often seen in cleft patients related to tooth alignment?
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What structural components contribute to the formation of the cleft lip?
What structural components contribute to the formation of the cleft lip?
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Which type of cleft is characterized by non-fusion of one side of the palatine process with the premaxilla?
Which type of cleft is characterized by non-fusion of one side of the palatine process with the premaxilla?
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What is the primary cause of cleft palate formation?
What is the primary cause of cleft palate formation?
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During which weeks of embryonic development does the fusion of palatal shelves occur?
During which weeks of embryonic development does the fusion of palatal shelves occur?
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What anatomical structure fails to fuse in cases of bilateral cleft palate?
What anatomical structure fails to fuse in cases of bilateral cleft palate?
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Which embryonic structure is primarily responsible for the development of the hard palate through intramembranous ossification?
Which embryonic structure is primarily responsible for the development of the hard palate through intramembranous ossification?
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Which condition is associated with the clefting of the uvula?
Which condition is associated with the clefting of the uvula?
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What is the embryonic origin of the muscles of the soft palate?
What is the embryonic origin of the muscles of the soft palate?
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The presence of a Y-shaped cleft is indicative of which type of cleft?
The presence of a Y-shaped cleft is indicative of which type of cleft?
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What failure leads to the formation of a nose cleft?
What failure leads to the formation of a nose cleft?
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Study Notes
Cleft Lip and Palate Development
- Cleft lip and palate develop between weeks 6 and 12 of gestation.
- Formed from the fusion of 3 processes: 2 maxillary processes, 2 palatine processes, and the intermaxillary process (premaxilla).
- The palate mesoderm undergoes intramembranous ossification to form the hard palate.
- The posterior part of the palate remains unossified, forming the soft palate.
Types of Cleft Lip and Palate
-
Cleft palate: a failure of fusion of the palatine processes and premaxilla.
- Bilateral: failure of fusion on both sides, resulting in a Y-shaped cleft.
- Unilateral: non-fusion of one palatine process with the premaxilla.
- Incomplete: cleft limited to the soft palate or hard palate, or a bifid uvula.
- Cleft lip: a cleft of the primary palate, anterior to the incisive foramen, due to a lack of fusion of the medial nasal processes with the maxillary process.
- Nose cleft: failure of the intermaxillary segments and/or lateral nasal processes to fuse.
Syndromes Associated with Cleft Lip and Palate
- 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.
Epidemiology
- Prevalence: 1:700 in the UK.
- Syndromic vs. Non-syndromic: 70% are non-syndromic.
- Male: female ratio: 2:1.
- Left: Right ratio: 2/3: 1/3.
Subtypes
- Microform cleft: appears as a small dent, may affect underlying muscle tissue.
- Submucous cleft palate: no midline visible, associated with bifid/cleft uvula.
Common Features
- Post-nasal spine: almost always missing.
- Speech problems: common due to anatomical variations.
Dental Problems
- Feeding difficulties: due to structural issues.
- Hearing issues: potential for middle ear infections.
- Speech impediments: related to palate formation.
- Face growth disruption: affecting symmetry and appearance.
- Dental development disruption: leading to anomalies and tooth loss.
- Psychosocial considerations: impact on self-esteem and confidence.
Local Dental Problems
- Congenital missing teeth: hypodontia, oligodontia, hyperdontia.
- Natal/neonatal teeth: present at birth.
- Tooth morphology anomalies: microdontia, macrodontia.
- Enamel hypoplasia: weak enamel.
- Poor periodontal support: early tooth loss.
- Gemination: two teeth joined together.
- Dilacerations: distorted tooth roots.
Orthodontic Problems
- Class III malocclusion: underbite.
- Anterior/posterior crossbite: teeth misaligned.
- Spacing and crowding: uneven tooth alignment.
Prenatal Diagnosis
- Cleft lip: 81% diagnosed before birth.
- Cleft palate: difficult to identify before birth.
Management
- Birth: counselling, feeding plate, and pre-surgery assessment.
- 3 months: primary lip repair.
- 9-18 months: palate repair.
- 1-3 years: speech therapy.
- 3-6 years: speech therapy, masednoscopy and/or pharyngoplasty.
- 8-9 years: initial interventional orthodontics, preparation for alveolar bone grafting.
- 10 years: alveolar bone grafting.
- 12-14 years: definitive orthodontics.
- 17-20 years: orthognathic surgery, nasal revision surgery.
Impressions
- Taken to establish a cleft record.
- Used for future comparison.
- Part of the Cleft Surgical Audit Group (CSAG) audit.
Pre-surgical Orthodontics
- Maxillary strapping: helps shape the palate.
- Taping: used for repositioning.
- Nasoalveolar moulding appliances: reshapes the nose and premaxilla.
Aims of Surgery
- Restore normal anatomy.
- Minimize adverse effects.
- Reconstruct normal muscle function.
- Make the cleft unrecognizable.
"Rule of 10" for Surgery
- Primary repair: around 10 weeks of age.
- Weight: 10 lbs.
- Hemoglobin: 10g.
- White blood cell count: no higher than 10k.
Alveolar Bone Grafting
- Timing: precedes canine eruption on cleft side.
- Age: around 11 years old.
- Purpose: creates a bony bridge across the cleft to support the erupting canine and periodontium.
Orthognathic Surgery
- Age: 18 years and older.
- Common purpose: to correct Class III malocclusion.
- Potential risk: can compromise velopharyngeal function.
Cleft Lip + Palate Development
- Develops between weeks 6-12 during fetal development.
- Formed from the fusion of palatal shelves, which are derived from the first pharyngeal arch and the frontonasal process.
- The palate is formed by the fusion of three processes: two maxillary processes, two palatine processes of the maxillary processes, and the intermaxillary process, which forms the primitive palate.
- The hard palate is formed through intramembranous ossification of palatal mesoderm.
- The soft palate is formed in the posterior region where there is no ossification.
- The muscles of the soft palate originate from the fourth pharyngeal arch.
Cleft Palate Causes and Types
- Defective fusion of palatal processes results in cleft palate.
- Bilateral cleft palate occurs when both palatine processes and the premaxilla fail to fuse, resulting in a Y-shaped cleft.
- Unilateral cleft palate occurs when one side of the palatine process of the maxilla fails to fuse with the premaxilla.
- Incomplete cleft palate involves a cleft limited to either the soft or hard palate, or a bifid uvula.
Cleft Lip Causes and Types
- Cleft lip results from a lack of fusion between the medial nasal processes of the frontonasal prominence and the maxillary process, anterior to the incisive foramen.
- Nose clefts occur when the intermaxillary segments or lateral nasal processes fail to properly fuse.
Cleft Lip + Palate Syndromes
- Van der Woudes syndrome: Characterized by a lip pit, missing teeth, cleft palate, and delayed language development.
- Pierre Robin anomaly: Features micrognathia, natal teeth, glossoptosis, a high arched palate, and often a cleft palate.
- Di George Syndrome (velocardiofacial syndrome): Includes wide-set eyes, distinctive ears, a congenital heart defect, and susceptibility to infections.
Cleft Lip + Palate Epidemiology
- Prevalence: 1 in 700 in the UK.
- Non-Syndromic: Approximately 70% of cases are not associated with a syndrome.
- Gender: Male to female ratio is 2:1.
- Side: Left to right ratio is 2/3: 1/3.
Microform Cleft
- Presents as a small dent in the lip.
- Underlying muscle tissue can be affected and may require surgery.
Submucous Cleft Palate
- Characterized by a lack of a midline and muscle tissue in the palate, often associated with a bifid or cleft uvula.
- The post-nasal spine is usually absent in cases of submucous cleft palate.
Cleft Lip + Palate Complications
- Feeding and Speech: Difficulties can arise due to altered anatomy.
- Hearing: Potential for hearing loss due to middle ear infections.
- Dental Development: May experience disruptions in tooth development, tooth anomalies, and poor periodontal support.
- Facial Growth: Abnormalities in facial development and growth can occur.
- Psychosocial Impacts: The condition can impact self-esteem and social interactions.
Cleft Lip + Palate Dental Complications
- Missing Teeth: Congenital absence of teeth (hypodontia or oligodontia).
- Supernumerary Teeth: Presence of extra teeth (hyperdontia).
- Natal/Neonatal Teeth: Presence of teeth at birth or shortly after.
- Tooth Morphology: Variations in tooth shape, including microdontia (small teeth) and macrodontia (large teeth).
- Enamel Hypoplasia: Defective enamel formation.
- Periodontal Issues: Reduced periodontal support can lead to early tooth loss.
- Geminations: Two teeth fused together.
- Dilacerations: Abnormal bending or twisting of teeth.
Pre-Natal Diagnosis
- Cleft lip can be diagnosed in approximately 81% of cases before birth.
- Cleft palate is rarely identified before birth.
Cleft Lip and Palate Management
- Birth: Counselling, feeding plate, and pre-surgical assessments.
- 3 Months: Primary lip repair.
- 9-18 Months: Palate repair.
- 1-3 Years: Speech therapy.
- 3-6 Years: Speech therapy, nasendoscopy, and/or pharyngoplasty if needed.
- 8-9 Years: Initial interventional orthodontics and preparation for alveolar bone grafting.
- 10 Years: Alveolar bone grafting.
- 12-14 Years: Definitive orthodontic treatment.
- 17-20 Years: Orthognathic surgery and nasal revision surgery if required.
Impressions and Records
- Impressions: Taken to document the cleft and compare it to future changes, used in the cleft surveillance and audit group (CSAG) audit.
Pre-Surgical Orthodontics
- Techniques include maxillary strapping, taping, and nasoalveolar moulding appliances.
Alveolar Bone Grafting
- Typically performed around 11 years of age, before canine eruption on the cleft side.
- Creates a bony bridge across the cleft to support the erupting canine and periodontium.
Orthognathic Surgery
- Performed after 18 years of age.
- Primarily used to correct Class III malocclusion.
- May potentially compromise velopharyngeal function.
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Description
This quiz explores the development and types of cleft lip and palate, focusing on their formation during gestation and the processes involved. It covers classifications such as unilateral, bilateral, and incomplete clefts, as well as the structures affected. Test your knowledge on this important aspect of congenital anomalies.
