Tessier number 5 facial cleft

Tessier number 5 facial cleft is an extremely rare congenital craniofacial malformation classified within the Tessier cleft classification system, which categorizes rare facial clefts based on their anatomical position relative to the orbit. The number 5 cleft extends from the oral cavity obliquely through the cheek toward the lateral aspect of the lower eyelid, running between the medial canthus and the infraorbital foramen. It is considered the cranial extension of the number 9 cleft in the Tessier system. This cleft primarily affects the soft tissues and bony structures of the midface, involving the maxilla, the cheek, and the lower eyelid region. Clinically, Tessier number 5 clefts may present with an oblique oro-ocular cleft through the cheek, coloboma (notching) of the lower eyelid, disruption of the lateral nasal region, and deficiency of the underlying maxillary bone. The upper lip is typically intact, distinguishing it from more common cleft lip presentations. Soft tissue findings can include a groove or fissure extending from the corner of the mouth or the premolar region of the upper jaw toward the lower eyelid. Bony involvement may include hypoplasia or clefting of the maxillary bone lateral to the infraorbital foramen. Associated features can include orbital dystopia and dental anomalies. Treatment is primarily surgical and requires a multidisciplinary craniofacial team approach. Surgical reconstruction aims to restore normal facial anatomy and function, typically involving soft tissue repair, bony reconstruction, and eyelid correction. Multiple staged surgeries may be needed throughout childhood. Supportive care may include dental and orthodontic management, speech therapy, and ophthalmologic monitoring. Due to the extreme rarity of this condition, management is highly individualized and based on the specific anatomical structures involved in each patient.

Common questions about Tessier number 5 facial cleft