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Craniofacial Clefts

Tessier Classification of Craniofacial Clefting

  • Proposed by Tessier in 1976
  • All clefts result in regions of tissue deficiency
  • Clock face analogy number with “time zones” from 0 – 14
    • Point of reference is the orbit 
      • A horizontal axis connecting the medial and lateral canthus divides the face into upper and lower hemispheres
    • Clefts are categorized into hemisphere that they occupy
      • Facial clefts  (#0 – 7) occur in lower hemisphere (below the orbit) 
      • Cranial clefts (#8 – 14) occur in the upper hemisphere (above the orbit)
      • Midline mandibular cleft is denoted as #30

General Principles

  • Facial clefts may extend into the upper (cranial) hemisphere along their mirrored numeric axis
    • 0 and 14
    • 1 and 13
    • 2 and 12
    • 3 and 11
    • 4 and 10
    • 5 and 9
    • 6 and 8
    • 7 is the most lateral cleft
  • Each cleft may involve soft tissue and bone, but the number only assigns location
  • Clefts never pass through foramina or gutters of neurovascular bundles
    • Tunnels or foraminas may be converted into gutters by the marginal hypoplasia or adjacent clefts, bu the clefts do not pass through them
  • Clefting may be bilateral, but not necessarily equal in severity
  • Generally, clefts located medial to the infraorbital foramen have greater soft tissue than bony disruption, while clefts located lateral to the infraorbital foramen have greater bony than soft-tissue disruption 
  • Clefts 0 – 4 involve the maxilla and represent typical cleft lip and palate
  • Most common cleft = #7
  • Clefts #0 and 14 are generally not compatible with life (usually associated with holoproencephaly)

Embryologic Basis of Craniofacial Clefting

Centric vs. Acentric Clefts

  • Clefts 0, 1, 2, 3 and their corresponding cranial extensions, 14, 13, 12, and 11 are considered “centric” whereas facial clefts 4, 5, 6, 7, and 8 as well as cranial clefts 9 and 10 are “acentric”
    • Centric clefts cause structures lateral to the cleft (cranium, orbits, zygoma-maxilla) to be displaced laterally 
      • To correct these clefts, the displaced lateral structures must be re-positioned medially and will also require addition of tissue
    • Acentric clefts require only the addition of tissue (bone or soft tissue)

Tessier Cleft #0

  • True midline facial cleft
    • May be referred to as median craniofacial dysrhaphia
  • May cranial extension also in the midline (Tessier #14)
  • Incidence: 1/1,000,000 live births

Soft Tissue

  • Ranges from widened philtrum with bifid nasal tip and columella with central concavity to true midline cleft lip with laterally displaced nasal ala and widened alar base

Bone

  • Between central incisors
    • Results in characteristic sloping alveolar ridges towards the cleft bilaterally (described as keel shaped)
    • Creates anterior open bite deformity with vertical deficiency of the maxilla near the cleft site
  • Variable involvement of the nasal septum
    • Less involvement:  Mild thickening of septal cartilage and thickening and flattening of maxillary crest
    • More involvement: Lateral displacement of nasal processes of maxilla or true duplication of septum with lateral displacement of nasal bones
  • Superior extension (#0/14 combination)
    • Ethmoid sinuses: 
      • Volumetrically enlarged 
      • Prolapsed inferiorly and laterally
    • Widening of anterior cranial fossa floor and hypertelorism
    • Sphenoid sinus prolapse anteroinferiorly
      • Body of sphenoid bone remains normal
    • Lateral displacement of pterygoids

*Keel refers to the bottom-most structure of a boat or ship that runs the full length of the ship and around which the hull is built. Keel means “ship” in Old English and translate to “carina” in Latin.

Tessier Cleft #1

  • Similar to a typical cleft lip
  • Located paramedian within Cupid’s bow, extending superiorly to the dome of the alar cartilage and possibly to the medial aspect of the brow
    • Extension further superiorly indicates concomitant cranial extension (#13)

Soft Tissue

  • Cleft lip passing through Cupid’s bow
  • Short, broad columella
  • Alar notching near the soft triangle is distinctive
  • Nasal tip and septum deviate away from the cleft
  • The medial canthus may be malpositioned laterally resulting in telecanthus

Bone

  • Keel-shaped maxilla
    • Anterior incisors face toward cleft creating an anterior open bite
  • Rarely, an alveolar cleft is present between the central and lateral incisors
  • Cleft may extend posteriorly as a completely cleft of the hard and soft palate
  • The nasal floor is separated at the pyriform aperture just lateral to the nasal spine
    • May extend cephalad between the nasal bone and frontal process of maxilla
  • Nasal bones are displaced and flattened
  • Ethmoid sinus is expanded and can cause hypertelorism
  • Asymmetry of greater and lesser wings of the sphenoid bone, pterygoid plates and anterior cranial fossa

References

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