Anatomical classification of Tessier craniofacial clefts number 3 and number 4 in a South African population.

Abstract

The craniofacial clefts are rare defects of the face with an incidence of 1.43 to 4.85 per 100,000 live births. In 2016, WHO reported a death rate of 303,000 new-borns before 4 weeks of age due to congenital anomalies of which craniofacial clefts are one. Surviving the defect is associated with long term disabilities which impacts the individual, families, the healthcare system and society. How much we know about these clefts is seriously hampered by the rarity and the variations of these defects, so much so, that its treatment and communication amongst researchers is affected. The understanding of the skeletal defects occurring in the clefts has long been postulated as a key to any successive reconstruction of the face. This study aimed to reveal the extent of our understanding of these clefts, document the anatomical basis for the craniofacial cleft number 3 and number 4 and generating a sub-classification based on this and also document the clinical presentation as well as associated clefts of these craniofacial clefts in our select South African population. The methods used to achieve these included conducting a scoping review of the literature on patients with Tessier cleft number 3 and number 4 using relevant identified studies from 1976 sourced from PubMed, Medline, EBSCOhost, Google Scholar and the Cochrane libraries. The result of the study was reported using the Preferred Reporting Items for Systematic and Meta-analyses (PRISMA). Likewise, CT scans of patients who had been treated for Tessier clefts number 3 and 4 at Inkosi Albert Luthuli Central Hospital in Durban South Africa between 2003 and 2017 were analysed. Measurements of the expected defects in each cleft were taken and compared with the unaffected side as reference points. Emerging patterns of their analysis were then used to generate a sub-classification for these clefts. Lastly the records of 8 patients who had been treated for either Tessier cleft number 3 or number 4 were reviewed and compared with 9 studies sourced from the literature. In addition to the defects recorded, associated clefts and other congenital malformations were also documented, and findings were compared.

The scoping review had 33 studies that met the inclusion criteria. The majority were conducted in middle income countries (54.5%) while none were recorded in low income countries. Only 12.1% of the included studies reported on anthropometry. In understanding the skeletal defects, the presence of an alveolar cleft, the emerging patterns of comparison of the measurements of the maxilla and the orbits of the cleft side and the non-cleft side as well as absence of the bone were used to arrive at a sub-classification system using (a), (b). (c), (M+ O+), (M- O-), and (0). Clinical presentation of the patients who had been treated as cases of Tessier cleft number 3 and number 4 were compared to the reviewed literature and the different parameters were documented. In addition, associated clefts were also recorded, and this study found that the association pattern noted for Tessier cleft number 4 did not conform to its traditional counterpart. In conclusion, this study found that the knowledge of Tessier clefts number 3 and number 4 exist albeit not fully documented. Also, the study proposed a sub-classification for Tessier clefts number 3 and number 4 that will allow physicians to anticipate the extent and form of skeletal defect present before even seeing the patient. Lastly, it was concluded that however variable these clefts appear; they have a similar presentation worldwide and also that associated clefts do not conform to the original Tessier classification system.