Craniofacial Clefts

Mar 14, 2021 3 min read
Craniofacial Clefts

Craniofacial Clefts are various soft tissue and bone deformities. This articles looks at classification, managmenet and associated syndromes.

5 Key Points – Craniofacial Clefts

  1. Craniofacial clefts are often described as “atypical” clefts to avoid confusion with the “typical” cleft lip/palate.
  2. Craniofacial cleft is a sub-classification to the American Society of Cleft Lip and Palate Classification.
  3. Tessier’s classification is an anatomical description of craniofacial clefts centred on the clefts orientation from the palpebral fissure
  4. van der Meulens classification is the embryological classification of craniofacial clefts based on the time and degree of arrest/dysplasia.
  5. Associated conditions include Treacher Collins Syndrome and Pierre Robin Sequence.
A patient with median cleft syndrome – marked lateralization of orbits and nose that has undergone primary lip repair​
A patient with median cleft syndrome – marked lateralization of orbits and nose that has undergone primary lip repair​1​

Classification of Craniofacial Clefts

Craniofacial clefts is an umbrella term that encompasses a wide of of soft-tissue and bone deformities. It is a type of craniofacial deformity as defined by the American Society of Cleft Lip and Palate

There are two common classifications of craniofacial clefts – Anatomical or Embryological. A universally accepted classification scheme that is all-encompassing does not exist.

Anatomical – Tessier Classification

Tessier’s Topographic Classification is the most frequently used anatomical classification​2​. It is commonly used by surgeons because it is purely description and makes no pretence at causation and developmental relationships. There is also an ease of correlation between the anatomical defect and the required reconstructive surgery​3​.

Tips to Understanding the Classification:
  • Tessier Classification differentiates 15 locations (0-14) for craniofacial clefts with the palpebral fissure (orbit) as central landmark.
  • Cleft axes 0-7 are below the fissure/orbit, and therefore are facial clefts.
  • Cleft axes 8-14 are above the fissure/orbit, and therefore a cranial cleft.
  • Each sequential cleft is more lateral than the last.
Tessier Soft Tissue Classification of Craniofacial Clefts
Tessier Soft Tissue Classification of Craniofacial Clefts​2​
Tessier Bone Classification of Craniofacial Clefts
Tessier Bone Classification of Craniofacial Clefts​2​
Clinical Craniofacial Cleft Pearls:
  • Lateral clefts tend to have more severe bony abnormalities
  • Medial clefts tend to have more severe soft-tissue abnormalities.
  • Clefts do not pass through bony foraminae which are the site of neurovascular structures.
  • Bilateral cases may present different clefts on each side.
Memory Hook for Tessier Classification: “Groups of 3”
  • 0, 1, 2: Lip to nose
  • 3, 4 ,5: Lip to lower eyelid
  • 6, 7, 8: Treacher Collins Syndrome
  • 9, 10, 11: Orbit to upper eyelid
  • 12, 13, 14: Medial to orbit.

Embryological – van der Meulen Classification

van der Meulen embryological craniofacial cleft classification is the most comprehensive​4​.

In this system, the term dysplasia is used since not all malformations are true “clefts”. These dysplasia are classified according to time and location of development arrest.

Original Drawing of van der Muelen's embryological classification of Craniofacial Cleft
Original Drawing of van der Muelen’s embryological classification of Craniofacial Cleft​4​

Associated Syndromes

There are many syndromes and genetic patterns associated with Craniofacial Clefts. These include Treacher Collins Syndrome, Pierre Robin Sequence. Other are summarised in the table below by Irene et al​1​:

Types of Craniofacial Clefts, causal genetic defect, type of inheritance pattern and incidence
Types, causal genetic defect, type of inheritance pattern and incidence

Principles of Surgery

Functional

  • Oral Competence
  • Speech
  • Eyelid Reconstruction

Separation of Cavities

  • Oral
  • Nasal
  • Orbital


References

  1. 1. Mathijssen IMJ, Versnel SL. Craniofacial clefts. In: Plastic and Reconstructive Surgery. John Wiley & Sons, Ltd; 2015:255-263. doi:10.1002/9781118655412.ch20
  2. 2. Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg. 1976;4(2):69-92. doi:10.1016/s0301-0503(76)80013-6
  3. 3. Eppley B, van A, Robey A, Havlik R, Sadove A. The spectrum of orofacial clefting. Plast Reconstr Surg. 2005;115(7):101e-114e. doi:10.1097/01.prs.0000164494.45986.91
  4. 4. van der, Mazzola R, Vermey-Keers C, Stricker M, Raphael B. A morphogenetic classification of craniofacial malformations. Plast Reconstr Surg. 1983;71(4):560-572. doi:10.1097/00006534-198304000-00022
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