[23.1] Cleft

• Development happens because of ectoderm, which is the outermost part of the cell
• ectoderm migrate to designated area to form neural and connective tissue of skull brachial
• • Frontonasal process to forebrain
  • Left and right maxillary to lateral (longer process)
• Etiology: Endogenous (internal)

  • genetics/chromosomes
  • internal penetrance: jumps from one generation of family to another
  • cell mitigation
  • increase paternal age
• Etiology: Exogenous (external)

  • environmental teratogens
  • smoke, phenytoin (anticonvulsant drug)
  • vitamins: folic acid, B6
  • increase obesity
• Cleft lip (with and without palate) is twice as much prevalent and severe in men than female
• Cleft palate is twice as much prevalent/severe in female than male
• horizontal positioning and closure of palate occurs earlier in male
• secondary palate develop longer in female, making them susceptible to teratogens and cleft palate
• Mechanical Interference

  • Uterus Crowding (Pierre Robin Syndrome)
  • Dorsal Positioning of the Head
  • Retraction of Mandible
• Prevalence

  • 4th common
  • 10 in 10000
  • PH: 1 in 500
  • 0.2 to 0.5 per 1000 birth
  • 1 in 1000
• Kernahan and Stark (1958)

  • embryological development for clefts that is commonly used
• incisive foramen: line that separates hard and soft palate
• Primary Palate

  • hard palate
  • anterior to incisive foramen
  • fuse at 7 weeks
  • alveolus and lips
• Secondary Palate

  • soft palate
  • posterior to incisive foramen
  • fuse at 9 weeks
  • hard palate (except alveolus), velum
• Kernahan Strip Y

  • Upper Y: Primary Palate
  • Base of Y: Secondary Palate
• Prolabium

  • Ball-shaped tissue found in bilateral lip/cleft palate
  • under columella and before lips
• Complete Cleft

  • entire nostril sill, lip, alveolus
  • complete primary palate
  • anterior position at birth
• Incomplete Cleft

  • not extend to incisive foramen
  • still has lips ad nose intact
• • Bilateral: complete notch that would affect philtrum
  • Unilateral: one side, usually left
• Cleft Lip Form Fuste

  • Overlying skin is intact, but underlying muscles nasal cartilage and oral sphincter function
  • partial or arrested form of cleft lip
• Cleft Palate

  • Secondary and soft palate
  • posterior to incisive foramen
  • uvula, velum, hard palate
• Facial Cleft can be oblique or midline due to failure of neural cell mitigation
• Pierre Robin Sequence

  • Micrognathia
  • Bird's Profile: underdeveloped chin, protruding upper lips
  • Glossoptosis
• Moebius Syndrome

  • Genetic Disorder
  • Uncontrollable Lips, Open Mouth Posture, lack of Jaw movement
  • High Palatal Vault
  • excessive drooling
  • eyes towards center
• Hemifacial Microsomia

  • Mandibular: small mandible, lips situated on the weaker side
  • Hypoplasia, Facial Weakness
• Beckwith - Wiedemann Syndrome

  • Macroglossia: over enlarged tongue
  • Omphalocele
  • Hypoglycemia
  • abnormalities in Kidneys, Pancreas, Adrenal Cortexa
• CHARGE: coloboma, heart defects, atresia choanae (also known as choanal atresia), growth retardation, genital abnormalities, and ear abnormalities.
• Charge Syndrome

  • Choanal Atresia: blockage at the back of nasal passage
• Colobomas: eye, lower lids, iris, retina
• Pre-surgical Treatment

  • Align segments, reduce tension, improve outcome
  • Change Feeding technique: modify drink or syringe
  • Confirm VPI: airway obstruction
• Align segments, reduce tension, improve outcome

  • adhesive tape
  • dental elastics
  • Nasalveolar Mold (NAM)
  • Latham Appliance
• Cheiloplasty: Cleft lip repair
• Palatoplasty: palate repair
• Oronasal Fistula Repair (6-7)

  • Intentional
  • Unintentional: 5 - 30 %
• VPI Surgery (3 years old) : soft palate does not close velopharyngeal port
• Cheiloplasty: Rule of 10s

  • 10 weeks of age: enough tissue
  • 10 pounds: enough nutrition
  • 10 grams of hemoglobin: enough blood
• Palatoplasty

  • early: 6 - 15 months
  • late: 15 - 24 months
• Prosthetic Management: Denture, Facial, Feeding obturators
• Speech Appliances

  • Palatal Lift: velopharyngeal incompetence
  • Palatal Obturator: close defects of soft and Hard palate
  • Speech Bulb Obturator: velopharyngeal insuffeciency