Ophthal Plast Reconstr Surg, Vol. 31, No. 4, 2015
Case Reports
anti-proteinase 3 antineutrophil cytoplasmic antibodies, rheumatoid factor, angiotensin converting enzyme, and immunoglobulin-G4) was negative. A rheumatology review showed no signs of underlying systemic autoimmune disease or vasculitis. An otolaryngology review revealed no perceivable nasal pathology. Biopsy of the intraorbital mass was performed via the transcaruncular approach. We simultaneously arranged for an order of the previous histopathologic slices from the prior hospital. After the biopsy, antibiotics and antimycotics were restarted. A steroid was not used at this time because orbital cellulitis could not be ruled out. Twelve days after biopsy of the intraorbital mass, the patient noticed acute visual loss, periocular edema, and proptosis on the right side (Fig. C). His visual acuity decreased to 0.08 OD. Measurement of the right intraocular pressure and exophthalmometry could not be performed because of high eyelid tension. T2-weighted MRI showed a heterogeneous lesion spreading over the orbit and right globe tenting with an angle of 90° between the posterior sclera on the right side of the optic nerve (Fig. D). Emergency lateral canthotomy and cantholysis were performed under local anesthesia. On the same day, the histopathological results of the orbital and nasal samples revealed perivascular fibrosis with eosinophil-rich inflammatory infiltration (Fig. E) and “onion-skin” angiocentric fibrous lesions (Fig. F). There were few IgG4-positive plasma cells with no evidence of granulomas, necrosis, vasculitis, or infection. The diagnosis was EAF. The yellowish eyelids histopathologically corresponded to xanthoma. Treatment with intravenous methylprednisolone (125 mg/ day) and oral diaminophenyl sulfone (75 mg/day) was started immediately after the emergency surgery. Five days later, the right visual acuity improved to 1.0. The diaminophenyl sulfone was discontinued 1 month after initial administration, and cyclophosphamide (50 mg/day) was started at that point. The doses of methylprednisolone and cyclophosphamide were tapered over 9 months and 7 months, respectively. At the 1.5-year follow-up examination at our hospital, the patient showed a maintained right visual acuity of 1.0. The right intraocular pressure was normal. The Hertel exophthalmometry measurement improved to 12 mm OU. After the development of orbital compartment syndrome, his eye position deviated in the inferomedial direction, and binocular single vision was lost. However, both were restored after recession of the medial rectus and inferior rectus muscles. The lesions did not grow in size again.
DISCUSSION This is the first reported case of orbital compartment syndrome caused by the rapid spread of an EAF lesion over the orbit. Because the lesion developed abruptly after biopsy of the intranasal mass at another hospital and the intraorbital mass at our hospital, we suggest that these surgeries may have triggered the rapid progression of the lesion. Treatment of EAF is challenging, and anti-inflammatory and immunosuppressive medications are mostly ineffective.1–3 Although EAF demonstrates mixed findings of inflammation and fibrosis,1 most EAF lesions are associated with fibrotic changes with little inflammation at the time of first diagnosis,3 and medical therapy for this fibrosis is usually unsuccessful. However, because rapidly progressing lesions are thought to be associated with inflammation rather than fibrosis, our case was responsive to medical treatment.2 A heterogeneous lesion with hyperintensity was present on T2-weighted images taken on first examination at our
e100
hospital and before lateral canthotomy and cantholysis. EAF generally presents as a hypointense lesion on T2-weighted images because of fibrosis in the late phase.5 However, periocular edema and the progressive responsive to medical treatment in our case indicated considerable orbital inflammation, which may have caused the hyperintensity on the T2-weighted images. The visual outcome is commonly poor in cases of globe tenting with a posterior globe angle of
Case Reports
anti-proteinase 3 antineutrophil cytoplasmic antibodies, rheumatoid factor, angiotensin converting enzyme, and immunoglobulin-G4) was negative. A rheumatology review showed no signs of underlying systemic autoimmune disease or vasculitis. An otolaryngology review revealed no perceivable nasal pathology. Biopsy of the intraorbital mass was performed via the transcaruncular approach. We simultaneously arranged for an order of the previous histopathologic slices from the prior hospital. After the biopsy, antibiotics and antimycotics were restarted. A steroid was not used at this time because orbital cellulitis could not be ruled out. Twelve days after biopsy of the intraorbital mass, the patient noticed acute visual loss, periocular edema, and proptosis on the right side (Fig. C). His visual acuity decreased to 0.08 OD. Measurement of the right intraocular pressure and exophthalmometry could not be performed because of high eyelid tension. T2-weighted MRI showed a heterogeneous lesion spreading over the orbit and right globe tenting with an angle of 90° between the posterior sclera on the right side of the optic nerve (Fig. D). Emergency lateral canthotomy and cantholysis were performed under local anesthesia. On the same day, the histopathological results of the orbital and nasal samples revealed perivascular fibrosis with eosinophil-rich inflammatory infiltration (Fig. E) and “onion-skin” angiocentric fibrous lesions (Fig. F). There were few IgG4-positive plasma cells with no evidence of granulomas, necrosis, vasculitis, or infection. The diagnosis was EAF. The yellowish eyelids histopathologically corresponded to xanthoma. Treatment with intravenous methylprednisolone (125 mg/ day) and oral diaminophenyl sulfone (75 mg/day) was started immediately after the emergency surgery. Five days later, the right visual acuity improved to 1.0. The diaminophenyl sulfone was discontinued 1 month after initial administration, and cyclophosphamide (50 mg/day) was started at that point. The doses of methylprednisolone and cyclophosphamide were tapered over 9 months and 7 months, respectively. At the 1.5-year follow-up examination at our hospital, the patient showed a maintained right visual acuity of 1.0. The right intraocular pressure was normal. The Hertel exophthalmometry measurement improved to 12 mm OU. After the development of orbital compartment syndrome, his eye position deviated in the inferomedial direction, and binocular single vision was lost. However, both were restored after recession of the medial rectus and inferior rectus muscles. The lesions did not grow in size again.
DISCUSSION This is the first reported case of orbital compartment syndrome caused by the rapid spread of an EAF lesion over the orbit. Because the lesion developed abruptly after biopsy of the intranasal mass at another hospital and the intraorbital mass at our hospital, we suggest that these surgeries may have triggered the rapid progression of the lesion. Treatment of EAF is challenging, and anti-inflammatory and immunosuppressive medications are mostly ineffective.1–3 Although EAF demonstrates mixed findings of inflammation and fibrosis,1 most EAF lesions are associated with fibrotic changes with little inflammation at the time of first diagnosis,3 and medical therapy for this fibrosis is usually unsuccessful. However, because rapidly progressing lesions are thought to be associated with inflammation rather than fibrosis, our case was responsive to medical treatment.2 A heterogeneous lesion with hyperintensity was present on T2-weighted images taken on first examination at our
e100
hospital and before lateral canthotomy and cantholysis. EAF generally presents as a hypointense lesion on T2-weighted images because of fibrosis in the late phase.5 However, periocular edema and the progressive responsive to medical treatment in our case indicated considerable orbital inflammation, which may have caused the hyperintensity on the T2-weighted images. The visual outcome is commonly poor in cases of globe tenting with a posterior globe angle of
