IgG4-related disease: a systematic review of this unrecognized disease in pediatrics
and the anterior chamber was deep (Figure 1). Despite treatment with maximal topical antihypertensives and intravenous acetazolamide, IOP remained 44 mm Hg. The patient was admitted for management of the pain and the raised IOP. In the next 24 h she developed a 2 mm right-sided proptosis, a subtle relative afferent pupillary defect, mild limitation of right eye ocular movements, increasing pain, and a spike in temperature (38 1C). Based on the clinical findings of chemosis, congestion, a developing proptosis, limited ocular motility, a spike in temperature, and a subtle relevant afferent pupillary defect, an orbital cellulitis was suspected. An urgent CT scan of the orbits showed no signs of orbital haemorrhage, infection, or inflammation, but there were extra-scleral and subconjunctival loculated spaces with an appearance similar to that of the gas in the vitreous cavity (Figures 2 and 3). The CT scan raised suspicion of extra-ocular gas. Owing to the progressive proptosis and uncontrolled IOP despite maximum medical therapy, we decided to release and exchange the gas tamponade. The patient underwent 23-gauge vitrectomy with gas to fluid and then fluid to 1000 centistoke silicone oil exchange. Postoperatively the chemosis, congestion, and proptosis resolved completely and the patient was asymptomatic with a flat retina and a normal IOP. One month postoperatively, the retina is flat under oil; however, visual acuity is limited to hand movements owing to optic atrophy presumed to be secondary to the raised IOP and compression of the nerve from the intra-orbital gas.