Cleft Lip and Cleft Palate 

Etiology

• Family history and 1^st degree relatives
• Environmental factors – maternal epilepsy, drugs (steroids, diazepam), teratogens (thalidomide)
• Can be part of Pierre Robin syndrome
  • isolated cleft palate, micrognathia, posteriorly displaced tongue, respiratory and feeding issues

Classification

•  LAHSHAL – anatomic classification according site, size, extent and type of cleft
  • Upper case signifies complete cleft
  • Lower case signifies incomplete cleft

Cleft lip – due to disruption of muscles of the upper lip and nasiolabial region

• Nasolabial muscles
  • 1. Transverse labialis
  • 2. Levator labii superioris alaeques nasi
  • 3. Levator labii superioris
• Bilabial muscles
  • 4. Orbicularis oris – oblique head (upper lip)
  • 5. Orbicularis oris – horizontal head (upper lip)
  • 6. Orbicularis oris – lower lip
• Labiomental muscles
  • 7. Depressor anguli oris
  • 8. Depressor labii inferioris
  • 9. Mentalis

Types of Cleft Lip

• Unilateral – rings 1 and 2 disrupted on one side
  • Asymmetry of external nasal cartilage, nasal septum, maxilla
  • Displacement of nasal skin onto lip and retraction of labial skin
• Bilateral – rings 1and 2 disrupted on both sides
• Upper and lower
• Complete and incomplete – whether it extends to nostrils or not
• Simple or alveolar – if it involves bony alveolus or not
• Complicated or uncomplicated – if it is associated with cleft palate or not

Cleft palate

• Embryologically
  • 1^o palate – structure anterior to incisive foramen (alveolus + upper lip)
  • 2^o palate – structures posterior to incisive foramen (hard + soft palate)
• Anatomy of soft palate
  • Muscle fibres of soft palate are oriented transversely with no significant attachment to the hard palate
• Anatomy of hard palate –  3 layers
  • Palatal fibromucosa, maxillary fibromucosa and gingival fibromucosa
• Failure of fusion of 2 palatine processes (PP) – results in different types
  • Complete (type I) – failure of fusion of premaxilla and 2 PPs
  • Incomplete (type II) – incomplete fusion of premaxilla and 2 PPs
    • IIa – results in bifid uvula
    • IIb – results in bifid soft palate
    • IIc – results in bifid soft palate and posterior hard palate

Complications

• Impaired feeding – difficult suckling
• Wide pharynx – regurgitation of food
• Difficult speech – especially consonants D, P, T, B, K, J
• Nose and teeth malformation
• Pierre Robin Syn

Treatment

Cleft lip

• Attention to feeding – special spoon
• Millards operation – performed if criteria is fulfilled
  • Bare the edge of the cleft to create a raw surface in zig-zag pattern
  • Dissect the lip from the maxilla by an incision done in the gingivo-labial fold
  • Suture the cleft in 3 layers – skin to skin, muscle to muscle, mucous membrane to mucous membrane
    • Aim is to get the vermillion of the lip in alignment

Cleft lip revision – delayed for at least 2 years after 1^st operation

• To improve 1^o reconstruction
• Indications – lip deformities, misaligned vermillion, deviated nasal septum, nasal deformity

Cleft palate

• Surgery done at 3-6 months (not before as tissues are still delicate)
• Langerbeck Repair
  • Bare edges of cleft
  • Release incisions are done on either side of palate on the oral mucoperiosteum
  • Closure of the cleft in layers – first the nasal mucosa, then mucoperiosteum of the hard palate
• Delaire technique and sequence
  • Cleft lip alone
    • Unilateral – 1 operation at 5-6 months
    • Bilateral – 1 operation at 4-5 months
  • Cleft palate alone
    • Soft palate – 1 operation at 6 months
    • Soft and hard palates – 1 soft palate operation at 6 months and 1 hard palate operation at 15 months
  • Cleft lip and cleft palate
    • 2 operations – cleft lip and soft palate at 6 months, then hard palate operation at 18 months

Secondary management

• Hearing – patients have higher incidence of sensorineural and conductive hearing loss
• Speech problems
• Dental – delayed teeth eruption