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Case of the season
Case of the Season By Ay-Ming
HIS 53-year-old black woman had a tenyear history of right retroorbital mass. She was referred to the hospital for evaluation of progressive vision loss in the right eye. Two years ago she had been diagnosed clinically as having pseudotumor of her right orbit. Iridocyclitis was found in the right eye. Vision had improved significantly after intravenous steroid treatment. Chest radiographs and laboratory examinations were normal one year ago. Figure 1A is an axial CT scan of the orbits after contrast administration, obtained one year ago. A sagittal reformed
T
Seminars in Roentgenology,
Vol XIX, No 3 (July), 1984
Wang
(computer reconstructed) CT image was taken along the right (Fig 1B) and left optic nerve (Fig IC). From the Department of Radiology, Harvard Medical School, Brigham and Women’s Hospital, Boston, Mass. Ay-Ming Wang: Assistant Professor of Radiology, Harvard Medical School. Address reprint requests IO Ay-Ming Wang, MD, Department of Radiology, Harvard Medical School, 25 Shattuck Street, Boston, MA 02115. o I984 by Crune & Stratton, Inc. 0037-I 98X/84/1 903~003$05.00/0
163
A’i-MING
164
Fig 1A
Fig 2
(labelled)
DIAGNOSIS: SARCOIDOSIS OF THE RIGHT ORBIT
The initial CT scan of this patient was taken first without and then with intravenous administration of contrast. Figure IA (labelled) shows a soft-tissue mass involving the lacrimal gland (arrows) and retrobulbar region (open arrows) of the right orbit, extending to the enlarged superior orbital fissure (small arrows). No intracranial extension is noted. There is enophthalmos of the right eye. The sagittal computer reformed CT image demonstrates a diffusely infiltrating, soft-tissue mass in the retrobulbar region which cannot be separated from the right optic nerve (Fig 1B). The left optic nerve is normal (Fig 1C). Surgical biopsy of the right retrobulbar mass revealed a noncaseating granuloma consistent with sarcoidosis. The patient received steroid treatment, and vision improved in her right eye. One year later she complained of dizziness. On followup CT scan (Fig 2), the right retrobulbar mass had decreased in size and could now be separated from the right optic nerve (arrows). The soft-tissue mass in the right lacrimal gland also appeared to be smaller. No intracranial abnormality was noted. Sarcoidosis is a multisystem granulomatous disorder of unknown cause. The disease shows a predilection for blacks in the 20- to 40-year age group. lmpairment in cell-mediated immunity is suggested by depression of delayed hypersensitivity. Ocular involvement is present in 20% to 50% of all patients with sarcoidosis; it is equally common in males and females and is the reason for initial presentation in 10% to 25% of cases.
WANG
Chest x-ray is positive in nearly all of these patients. Iridocyclitis is the most common ocular manifestation of sarcoidosis; it is seen in 70% to 80% of patients with ocular disease. The lacrimal gland contains sarcoid granuloma in 7% to 10% of sarcoid patients.’ Involvement of other orbital areas is exceedingly rare. Systemic corticosteroids may be effective. Concentric widening of the optic canal due to sarcoid involvement of the optic nerve has been reported.2 An isolated sarcoidal optic nerve mass must be distinguished from optic nerve glioma, neurofibroma, meningioma, orbital pseudotumor, and metastasis. None of the first three possibilities is associated with both retrobulbar soft-tissue mass and lacrimal gland involvement. Orbital pseudotumor and metastasis can mimic orbital sarcoidosis. Immediate and dramatic response to steroid treatment in orbital pseudotumor may help in differential diagnosis.3 Enophthalmos, as in the right eye in this patient, is unusual for orbital pseudotumor. Orbital CT provides detailed information about the characteristics and extension of a soft-tissue mass, as well as bony involvement, but it may not provide a definite diagnosis. When the latter is in question, surgical biopsy should be considered. REFERENCES 1. Moseley IF, Sanders MD: Disease of the orbit. In: Moseley IF, Sanders MD (eds): Computerized Tomography in Neuro-ophthalmology. Philadelphia: Saunders, 1982;2082 2. Statton R, Blodi FC, Hanigan J: Sarcoidosis of the optic nerve. Arch Ophthalmol 1964;71:834-836 3. Nugent RA, Rootman J, Robertson WD, et al: Acute orbital pseudotumors: Classification and CT features. AJNR 198 1;2:43 l-436
HIS 53-year-old black woman had a tenyear history of right retroorbital mass. She was referred to the hospital for evaluation of progressive vision loss in the right eye. Two years ago she had been diagnosed clinically as having pseudotumor of her right orbit. Iridocyclitis was found in the right eye. Vision had improved significantly after intravenous steroid treatment. Chest radiographs and laboratory examinations were normal one year ago. Figure 1A is an axial CT scan of the orbits after contrast administration, obtained one year ago. A sagittal reformed
T
Seminars in Roentgenology,
Vol XIX, No 3 (July), 1984
Wang
(computer reconstructed) CT image was taken along the right (Fig 1B) and left optic nerve (Fig IC). From the Department of Radiology, Harvard Medical School, Brigham and Women’s Hospital, Boston, Mass. Ay-Ming Wang: Assistant Professor of Radiology, Harvard Medical School. Address reprint requests IO Ay-Ming Wang, MD, Department of Radiology, Harvard Medical School, 25 Shattuck Street, Boston, MA 02115. o I984 by Crune & Stratton, Inc. 0037-I 98X/84/1 903~003$05.00/0
163
A’i-MING
164
Fig 1A
Fig 2
(labelled)
DIAGNOSIS: SARCOIDOSIS OF THE RIGHT ORBIT
The initial CT scan of this patient was taken first without and then with intravenous administration of contrast. Figure IA (labelled) shows a soft-tissue mass involving the lacrimal gland (arrows) and retrobulbar region (open arrows) of the right orbit, extending to the enlarged superior orbital fissure (small arrows). No intracranial extension is noted. There is enophthalmos of the right eye. The sagittal computer reformed CT image demonstrates a diffusely infiltrating, soft-tissue mass in the retrobulbar region which cannot be separated from the right optic nerve (Fig 1B). The left optic nerve is normal (Fig 1C). Surgical biopsy of the right retrobulbar mass revealed a noncaseating granuloma consistent with sarcoidosis. The patient received steroid treatment, and vision improved in her right eye. One year later she complained of dizziness. On followup CT scan (Fig 2), the right retrobulbar mass had decreased in size and could now be separated from the right optic nerve (arrows). The soft-tissue mass in the right lacrimal gland also appeared to be smaller. No intracranial abnormality was noted. Sarcoidosis is a multisystem granulomatous disorder of unknown cause. The disease shows a predilection for blacks in the 20- to 40-year age group. lmpairment in cell-mediated immunity is suggested by depression of delayed hypersensitivity. Ocular involvement is present in 20% to 50% of all patients with sarcoidosis; it is equally common in males and females and is the reason for initial presentation in 10% to 25% of cases.
WANG
Chest x-ray is positive in nearly all of these patients. Iridocyclitis is the most common ocular manifestation of sarcoidosis; it is seen in 70% to 80% of patients with ocular disease. The lacrimal gland contains sarcoid granuloma in 7% to 10% of sarcoid patients.’ Involvement of other orbital areas is exceedingly rare. Systemic corticosteroids may be effective. Concentric widening of the optic canal due to sarcoid involvement of the optic nerve has been reported.2 An isolated sarcoidal optic nerve mass must be distinguished from optic nerve glioma, neurofibroma, meningioma, orbital pseudotumor, and metastasis. None of the first three possibilities is associated with both retrobulbar soft-tissue mass and lacrimal gland involvement. Orbital pseudotumor and metastasis can mimic orbital sarcoidosis. Immediate and dramatic response to steroid treatment in orbital pseudotumor may help in differential diagnosis.3 Enophthalmos, as in the right eye in this patient, is unusual for orbital pseudotumor. Orbital CT provides detailed information about the characteristics and extension of a soft-tissue mass, as well as bony involvement, but it may not provide a definite diagnosis. When the latter is in question, surgical biopsy should be considered. REFERENCES 1. Moseley IF, Sanders MD: Disease of the orbit. In: Moseley IF, Sanders MD (eds): Computerized Tomography in Neuro-ophthalmology. Philadelphia: Saunders, 1982;2082 2. Statton R, Blodi FC, Hanigan J: Sarcoidosis of the optic nerve. Arch Ophthalmol 1964;71:834-836 3. Nugent RA, Rootman J, Robertson WD, et al: Acute orbital pseudotumors: Classification and CT features. AJNR 198 1;2:43 l-436