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Coronoid-Temporalis Pedicled Flap for Orbital Floor Defect Reconstruction
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875
Congenital Ocular Dystopia from Orbitofrontal Bone Dysplasia
Jordan W Swanson1, Scott P Bartlett
1Philadelphia, Pa. From the Division of Plastic Surgery, Perelman School of Medicine at the University of Pennsylvania, The Children's Hospital of Philadelphia.
Plastic and Reconstructive Surgery
|December 29, 2015
Summary
Orbitofrontal bone dysplasia, a newly identified condition causing ocular dystopia and frontal bone defects, can be effectively treated with single-stage fronto-orbital reconstruction, preserving vision.
Area of Science:
- Craniofacial Surgery
- Pediatric Plastic Surgery
- Congenital Abnormalities
Background:
- Orbital dysplasia and frontal bone defects are observed in patients with ocular dystopia and craniofacial differences.
- This condition is distinct from other diagnoses like encephalocele or craniofacial dysostoses.
Purpose of the Study:
- To identify and characterize a previously unreported condition of orbitofrontal bone dysplasia.
- To evaluate the efficacy of fronto-orbital reconstruction for this condition.
Main Methods:
- Retrospective review of craniofacial registries from 2000-2014.
- Analysis of features and treatment outcomes in four patients with congenital orbitofrontal bone dysplasia.
Main Results:
- Four patients identified with unilateral or bilateral frontal bone defects and ocular dystopia.
- Fronto-orbital reconstruction using cranial bone grafting successfully reduced ocular dystopia and preserved vision in three patients.
Conclusions:
- Orbitofrontal bone dysplasia is a novel condition characterized by frontal bone defects and ocular dystopia.
- Single-stage fronto-orbital reconstruction is an effective treatment for this condition.
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