From birth to maturity: midline Tessier 0-14 craniofacial cleft patients who have completed protocol management at a single craniofacial unit

Abstract

INTRODUCTION: The rare craniofacial clefts form an important component of craniofacial pathology, but little has been written regarding the definitive management of affected patients. This report describes the presentation, management, and outcomes in a group of patients who have completed their protocol management for treatment of midline Tessier 0-14 clefts. METHODS: A retrospective review of the clinical, photographic, and radiographic records of all midline cleft patients treated at a single center was performed. Data describing each patient's presenting features, surgical management, and final outcomes are presented. RESULTS: Four patients were identified as having completed protocol management for Tessier 0-14 midline clefting at the unit. The age range at the most recent follow-up was 19.3 to 36.3 years. Three patients had entered protocol management during infancy, and the remaining patient presented to the unit at 13.8 years of age. The surgical management regimen is described in detail. Outcomes for development, hearing, speech, and vision at maturity were all acceptable. Three patients attained a respectable educational and social status. With respect to facial aesthetics scores, the only significant difference after management was a significant worsening of deformity in the region of the orbits. The Whitaker grade for repeat surgery improved after management (3.25 before to 2.63 postmanagement), but this improvement was not statistically significant. CONCLUSION: Presented are the results of the first cohort of midline Tessier 0-14 cleft patients to have completed protocol management at a single craniofacial unit. As more patients complete their management in the future, further refinements to the protocol could be made.