Tessier number 4 facial cleft

Tessier number 4 facial cleft is an extremely rare congenital craniofacial malformation classified within the Tessier cleft system, which categorizes rare facial clefts based on their anatomical position relative to the orbit. The Tessier number 4 cleft extends from the upper lip (lateral to the philtrum, medial to the oral commissure) upward through the cheek to the lower eyelid, typically passing medial to the infraorbital foramen. This oro-ocular cleft disrupts the soft tissue and sometimes the bony structures between the mouth and the orbit, affecting the maxilla, the inferior orbital rim, and the orbital floor. It may also be referred to as a medial oro-ocular cleft. Key clinical features include a cleft or groove extending from the lateral upper lip toward the medial aspect of the lower eyelid, coloboma (notching) of the lower eyelid, disruption of the lacrimal drainage system, and hypoplasia or clefting of the maxillary bone. The nose and lip may appear distorted, and dental anomalies are common in the affected region. The cleft can be unilateral or bilateral and may occur in isolation or in association with other Tessier clefts or craniofacial syndromes such as Treacher Collins syndrome or amniotic band syndrome. Treatment is primarily surgical and requires a multidisciplinary craniofacial team including plastic surgeons, oral and maxillofacial surgeons, ophthalmologists, orthodontists, and speech therapists. Surgical reconstruction is typically staged and may involve soft tissue repair of the lip and cheek, reconstruction of the orbital floor and inferior orbital rim using bone grafts, lower eyelid reconstruction, and correction of the lacrimal system. Early intervention is important to optimize functional outcomes including feeding, vision, and facial growth. Long-term follow-up is necessary as additional corrective procedures may be needed during growth. Psychosocial support is also an important component of care for affected individuals and their families.

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