Etiology:

Many contributing risk factors: Genes & environment

• Genetic predisposition
• Syndromes associated with cleft li and palate
• Nutrition: iron, zinc, folic acid, B12 deficiency
• Drugs: phenytoin, valproic aid
• Alcohol
• Maternal smoking
• Stress and disturbance during development

Cleft lips: Failure of medial & lateral nasal process to be penetrated by epithelial cells during 6th week in utero.

Cleft palate: Lack of lateral palatal segment fusion – ingrowth of mesoderm – and epithelial breakdown during 8th week in utero

Results in – Mastication, speech, deglutition defect

Veau classification:

Cleft lips:

Class I	Unilateral notch of vermillion border, not extending to lip
Class II	Unilateral cleft of lip, not extending to floor of nose
Class III	Unilateral cleft of lip, extending to floor of nose
Class Iv	Bilateral cleft of lip

Cleft palate:

Class I	Unilateral cleft limited to soft palate
Class II	Unilateral cleft of hard and soft palate – not extending past incisive foramen (2ry palate)
Class III	Unilateral cleft extending from uvula to alveolar process
Class Iv	Bilateral cleft of soft and hard palate

Diagnosis:

• Ultrasonography for baby in utero

Management:

• Lip surgery
• Palate repair – before speech development (1 and half years age)
• Speech therapy
• Alveolar bone grafting
• Orthodontic treatment
• Orthognathic surgery (jaw exercise)

NB:

• Age 1-3 months – Lip taping and naso-alveolar molding
• Age 3 months – Repair of cleft lip
• Age 9-12 month – Repair of cleft palate
• Age 1-7 years – Orthodontic treatment
• Age 7-8 years – Alveolar bone graft
• 18 years old or skeletal maturity – Midface advancement, continued orthodontic treatment

Rule of 10’s:

• Weight ≥ 10 pounds
• Hemoglobin ≥ 10 mg/dl
• Age ≥ 10 weeks

Associated syndromes:

• Apert syndrome
• Pierre Robin syndrome
• Treacher Collin syndrome

---

Van der Woude syndrome

• Lower lip pits in patient with cleft lip and palate
• AD
• Mutations in interferon regulatory factor 6 gene (IRF6)
• Associated features:
  • Hypodontia
  • Molar incisor hypomineralization
  • Ankyloglossia (tongue tie)
  • Syndactyly (webbed fingers)

Picture