Managing Bilateral Oro-Sino-Orbital Fistulae in the Setting of Bilateral Tessier IV Clefts

The number IV orofacial cleft is extremely rare, and reports on long-term outcomes are limited. Here we present the management of a child born with bilateral Tessier number IV clefts and microphthalmia, who developed bilateral oro-sino-orbital fistulae. He presented 8 years after his index procedure with persistent ocular drainage and radiographic evidence of bilateral oro-sinoorbital fistulae. His globes maintained light perception, and in order to protect them from chemical reflux he underwent fistula obliteration with demineralized bone matrix and acellular dermal matrix. His orbits were reconstructed by Imre and conjunctival flaps. In the presence of marginally functioning globes, the importance of timely intervention is critical in order to preserve residual ocular function. Light perception vision assists in the maintenance of circadian rhythm and physiologic functions that are critical to the developing pediatric patient. This case also emphasizes the importance of a multi-disciplinary team in the correction of complex craniofacial deformities.