title: Congenital Ocular Dystopia from Orbitofrontal Bone Dysplasia.
abstract: BACKGROUND
  Several patients with ocular dystopia and craniofacial differences have been found to have orbital dysplasia and a frontal bone defect. This deformity is characteristically different from differential diagnoses of encephalocele, sphenoid dysplasia, craniofacial dysostoses, or atypical clefting.
  
  
  METHODS
  The authors retrospectively reviewed the craniofacial registries of two pediatric centers for patients presenting with ocular dystopia or orbitofrontal anomalies between 2000 and 2014. The features and treatment of these patients were analyzed.
  
  
  RESULTS
  Four patients with congenital orbitofrontal bone dysplasia were identified, three with unilateral and one with bilateral frontal bone defects. Clinical signs of hypoglobus and vertical ocular dystopia of an average of 5.0 mm on the affected side were noted shortly after birth. The transversely oriented bony defect had an average surface area of 3.9 cm in unilateral cases and 10.7 cm in bilateral cases. Patients showed a characteristic orbital vertical elongation with an average orbital height-to-width ratio of 1.30-in excess of the average normal 1.14 by 14 percent-and inferior rim displacement. Cranial contour demonstrated frontal bossing and borderline dolichocephaly. Fronto-orbital reconstruction was performed in three patients, using cranial bone grafting to obliterate the orbital roof defect and elevate the orbital floor, which was successful in reducing ocular dystopia and preserving vision in each patient. One patient followed for 11 years postoperatively has a durable result with no surgical revision.
  
  
  CONCLUSIONS
  Orbitofrontal bone dysplasia has not been previously reported, and includes a frontal bone defect and ocular dystopia. Single-stage fronto-orbital reconstruction appears to adequately correct it.
  
  
  METHODS
  Therapeutic, IV.
authors.affiliation: Philadelphia, Pa. From the Division of Plastic Surgery, Perelman School of Medicine at the University of Pennsylvania, The Children's Hospital of Philadelphia.
authors.forename: Jordan W
authors.initials: JW
authors.lastname: Swanson
authors.forename: Scott P
authors.initials: SP
authors.lastname: Bartlett
mesh_terms: D001848:Bone Diseases, Developmental / Q000000981:diagnostic imaging / Q000601:surgery*; D016025:Bone Transplantation / Q000379:methods*; D003394:Craniofacial Dysostosis / Q000000981:diagnostic imaging / Q000601:surgery*; D005124:Eye Abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005624:Frontal Bone / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009915:Orbit / Q000000981:diagnostic imaging / Q000601:surgery*; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies; D013100:Sphenoid Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D014057:Tomography, X-Ray Computed