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Bilateral cavernous haemangiomas of the orbit
  1. JERRY A SHIELDS
  1. Oncology Service,Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA, 19107, USA
  2. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  3. Oncology Service
  4. Department of Pathology
  5. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  6. Oncology Service
  1. R NICK HOGAN
  1. Oncology Service,Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA, 19107, USA
  2. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  3. Oncology Service
  4. Department of Pathology
  5. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  6. Oncology Service
  1. CAROL L SHIELDS
  1. Oncology Service,Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA, 19107, USA
  2. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  3. Oncology Service
  4. Department of Pathology
  5. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  6. Oncology Service
  1. RALPH C EAGLE, JR
  1. Oncology Service,Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA, 19107, USA
  2. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  3. Oncology Service
  4. Department of Pathology
  5. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  6. Oncology Service
  1. ROBERT H KENNEDY
  1. Oncology Service,Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA, 19107, USA
  2. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  3. Oncology Service
  4. Department of Pathology
  5. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  6. Oncology Service
  1. ARUN D SINGH
  1. Oncology Service,Wills Eye Hospital, 900 Walnut Street, Philadelphia, PA, 19107, USA
  2. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  3. Oncology Service
  4. Department of Pathology
  5. Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA and the Department of Ophthalmology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
  6. Oncology Service
  1. Dr Jerry A Shields

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Editor,—The great majority of orbital tumours are unilateral. However, some conditions, such as idiopathic orbital inflammation (“pseudotumour”), granulomatous inflammation, lymphoid tumours, or metastases sometimes can be bilateral. Orbital cavernous haemangioma is almost always unilateral.1 2 We report a patient with bilateral orbital cavernous haemangiomas that caused unilateral proptosis and visual distortion.

CASE REPORT

A 37 year old man developed distorted vision in the inferior field of his left eye and was found to have mild left proptosis. There was mild swelling of the left optic disc and a small inferonasal visual field defect. Orbital computed tomography (CT) revealed bilateral orbital masses and the patient was referred to the oncology service for another opinion and management. His visual acuity was 6/6 in each eye and colour vision was normal in both eyes. The only positive findings were 3 mm of left proptosis, mild swelling of the left optic disc, and a subtle inferonasal field defect. The orbital CT showed a round, soft tissue mass in the left orbit temporally that displaced the retrobulbar portion of the optic nerve nasally. A similar but smaller mass was present in the right orbit lateral to the medial rectus muscle (Fig 1). Both masses showed slight contrast enhancement. The clinical diagnosis was bilateral cavernous haemangioma. The left orbital mass was removed intact by a superolateral extraperiosteal approach. Gross and microscopic studies revealed a benign tumour composed of large endothelial lined vascular channels separated by fibrous stroma, consistent with cavernous haemangioma (Fig 2). The patient had an unremarkable postoperative course with good visual acuity.

Figure 1

Orbital computed tomograms with axial cut (A) and coronal cut (B), showing bilateral circumscribed orbital soft tissue masses, with a larger tumour in the left orbit and a small tumour in the right orbit.

Figure 2

Pathology. (A) Gross photograph of pseudoencapsulated reddish blue mass. (B) Photomicrograph, showing large cavernous vascular channels typical of orbital cavernous haemangioma (haematoxylin and eosin ×100).

COMMENT

Cavernous haemangioma of the orbit is a common, benign tumour that almost always occurs unilaterally. There are rare reports of bilateral orbital cavernous haemangiomas. Fries and Char reported a case that was very similar to ours.3 They excised the tumour in one orbit and elected to follow the asymptomatic tumour in the opposite orbit. Sullivan and associates reported an unusual case of multiple bilateral orbital haemangiomas.4 Johnson and co-workers reported bilateral orbital haemangiomas as part of Maffucci's syndrome, a condition characterised by enchondromatosis and multiple systemic haemangiomas.5 Our patient was otherwise normal with no evidence of Maffucci's syndrome. There was also a recent report of bilateral orbital vascular masses, presumed orbital varices, that caused intravascular papillary endothelial hyperplasia.6

The widespread use of CT and magnetic resonance imaging to evaluate unrelated problems, such as headache, has led to the recognition of asymptomatic lesions compatible with orbital cavernous haemangioma. We have seen several such cases and have elected to follow without treatment these small, asymptomatic tumours, presumed to be orbital cavernous haemangiomas. Consequently, it is possible that orbital cavernous haemangioma may be more common than previously believed. If so, additional cases of bilateral orbital cavernous haemangioma will probably be recognised. In our case, the asymptomatic tumour in the right orbit most likely is a cavernous haemangioma, but periodic observation, rather than surgical excision, would seem to be the best management. In summary, the ophthalmologist should include orbital cavernous haemangioma in the differential diagnosis of bilateral, as well as unilateral, orbital tumours.

Acknowledgments

Supported by the Eye Tumor Research Foundation, Philadelphia, PA (J Shields, C Shields), the Noel T Sara L Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital (RC Eagle). and from Research to Prevent Blindness (RN Hogan).

References

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