Hallermann-Streiff Syndrome in Concordant Monozygotic Twins With Congenital Cataracts, Exudative Retinal Detachments, and One Case of Corneal Perforation Requiring Keratoplasty.

PURPOSE: We describe the management of Hallermann-Streiff syndrome in monozygotic female twins with congenital cataracts, exudative retinal detachments, and 1 case of corneal descemetocele with associated dellen and subsequent perforation. METHODS: This study was a case report and review of the literature. RESULTS: Twins 1 and 2 exhibited all 7 cardinal characteristics of Hallermann-Streiff syndrome, presenting with spontaneous lenticular resorption, anterior uveitis, and glaucoma. They underwent bilateral cataract extraction with near total capsulectomy. Both twins experienced recurrent glaucoma, for which twin 1 underwent successful endocyclophotocoagulation in both eyes and twin 2 in the left eye alone. The fellow eye developed 2 sites of perilimbal corneal descemetoceles with associated dellen at the inferotemporal limbal corneal junction leading to spontaneous perforation of 1 site, requiring a full-thickness corneal graft. Both twins developed recurrent bilateral exudative retinal detachments unresponsive to oral prednisolone. Twin 1's last best-corrected visual acuity with aphakic spectacles was 20/260 in the right eye and 20/130 in the left eye at age 4 years and 8 months. Twin 2's last best-corrected visual acuity was 20/130 in each eye at age 4 years and 11 months, over a year after right eye penetrating keratoplasty. CONCLUSIONS: We describe 2 rare cases of Hallermann-Streiff syndrome in monozygotic twins complicated by corneal perforation requiring penetrating keratoplasty in 1 eye of 1 twin. Although corneal opacities have been described in this condition, this is the first case of corneal descemetocele in Hallermann-Streiff syndrome. The cornea was stabilized with a relatively favorable visual outcome over 1 year later.

Factors Associated with Ocular Injury in Orbital Fracture Patients: Who Requires Urgent Ophthalmic Evaluation?

Importance: Create validated criteria to identify orbital fracture patients at higher risk for significant ocular injuries. Objective: Determine history and physical examination findings in orbital fracture patients who are associated with ocular injury and warrant urgent assessment by an ophthalmologist. Design, Setting, and Participants: Retrospective chart review of 535 adult orbital fracture patients evaluated at a Level I emergency department between 2014 and 2017, without prior history of orbital fracture, ocular injury, or ocular/orbital surgery. Main Outcomes and Measure: Presence of ocular injury. Results: In total, 195 (36%) patients had an ocular injury. Those with and without ocular injury were compared in a multivariate logistic regression model including demographics, fracture characteristics, injury mechanism, and physical examination findings. Visual acuity change, radiographic retrobulbar hemorrhage, abnormal pupillary reaction, and inability to open the injured eye all had significant associations with ocular injury when other findings were controlled. Conclusion: This study shows a significant association between ocular injury and visual acuity change, retrobulbar hemorrhage, abnormal pupillary reaction, and inability to open the injured eye. These factors can help triage when to obtain an urgent ophthalmology consult.