Abstract

Haemangioma is the most common intraorbital tumour in adults. Asymptomatic in its early stage it usually becomes symptomatic only when it becomes quite big, at which time it generally presents with proptosis. We present here a rare case of an intraorbital haemangioma which over a period of time caused total phthisis bulbi leaving behind only an area of intraocular ossification covered with scleral coat. Such extreme phthisis bulbi as a complication of orbital haemangioma has never been reported in the English literature to the best of our knowledge.

Introduction

Cavernous haemangiomas are the most common intraorbital tumours found in adults. These benign, vascular lesions are slow growing and can manifest as a painless, progressively proptotic eye. Most of these tumefactions are exceedingly unilateral. Bilateral cases have been reported but are rare.

Case Report

A 38 year old man presented with a painless, protruding mass of the left orbit with proptosis and visual loss. He had been noticing progressively increasing proptosis since the past five years and visual diminution since 2 years.

On examination it was a very unsightly view. There was a large mass covered with chemotic conjunctiva, protruding about six centimeters outwards. The lids were gaping and could not completely cover the mass. On retracting the upper eyelid flattened collapsed scleral coat was seen with a scarred nebulous cornea. There was no light perception in the eye. Fundoscopy was not possible and a CT scan was performed which showed a large, homogenously enhancing mass without adjacent bony destruction.The most surprising abnormality was however the globe which was completely collapsed, flattened with dense ossification (CT density > 1500 H.U) within it (Fig. 1).

Colour Doppler USG revealed the mass to have homogenous internal echoes with patchy, slow blood flow consistent with a haemangioma (Fig. 2). Total enucleation with artificial implant was placed in the orbit. Histopathology of the excised mass showed it to be a benign cavernous haemangioma.

Discussion

Cavernous haemangiomas are the most common intraorbital tumours found in adults. These benign, vascular lesions are slow growing and can manifest as a painless, progressively proptotic eye. Most of these tumefactions are exceedingly unilateral. Some studies have shown left sided predominance (1.5:1). Bilateral cases have been reported but are rare and seen with Maffucci syndrome.

Haemangiomas are relatively common benign neoplasms with capillary neoplasms occurring primarily in infants and cavernous haemangiomas primarily in adult.1 Cavernous haemangioma occurs in the young and middle aged and is described to be more common in females. Though said to be common, Harris and Jacobeic found only 66 cases over a forty year period.2 Maroon and Kennerdell have reported only 17 haemangiomas from among 300 cases of orbital tumours over a period of seven years.3 Nath et al have reported 12 cases among 120 primary orbital tumours.4

Orbital cavernous angiomas can increase intraorbital volume with a resultant mass effect. Although cavernous haemangiomas are histologically benign, they can encroach on intraorbital or adjacent structures and can be considered anatomically or positionally malignant. Visual acuity or field compromise, diplopia, and extraocular muscle or pupillary dysfunction can result from compression of intraorbital contents by the angioma.3 Lagophthalmos can result in exposure keratopathy, keratitis, and corneal perforation. The morbidity associated with cavernous haemangiomas is the threat of compressive optic neuropathy, extraocular muscle dysfunction, and cosmetic disfigurement.5

Patient usually presents as painless progressive proptosis or bulging of their globe accompanied by mild eyelid fullness. A change in visual acuity secondary to induced hyperopia or reduction of the myopic refractive error can result from an anteriorly directed mass effect. In some cases, a compressive optic neuropathy can be the aetiological basis for the visual acuity or field disturbance. A change in visual acuity secondary to induced hyperopia or reduction of the myopic refractive error can result from an anteriorly directed mass effect. In some cases, a compressive optic neuropathy can be the aetiological basis for the visual acuity or field disturbance.6

Ultrasound study can find a uniform high-echogenicity on A-scan. These reflections are secondary to the septae found within the lesion. Doppler flow study may reveal subdued blood flow within the angioma.6
CT scan detects an oval or round shaped, sharply marginated, homogenous lesion. Uptake of contrast medium by this tumefaction is highly variable and has limited diagnostic value. Computed tomography should not be solely relied upon since it does not allow one to make a definitive diagnosis.

Most cavernous haemangiomas remain stable throughout a patient’s life and cause no visual compromise. If surgical intervention is warranted, most lesions excised in the hands of an experienced surgeon have an excellent prognosis and result in a low morbidity. There is no recurrence following excision or risk of malignant transformation. The tumours are removed successfully by standard lateral orbitotomy or anterior orbitotomy.7

Fig. 1 : Axial CT scan shows large homogenous exophytic mass occupying and expanding entire left orbit.Phthitic proptotic eyeball with only the residual scleral coat covering foci of hyperdense ossification seen.		Fig. 2 : High resolution USG image with color Doppler shows homogenous, hyperechoic mass with scattered slow flow consistent with a hemangioma.

References

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3. Maroon JC, Kennerdell JS. Tumours of the orbit. In : Wilkins RH and Rengachay SS, editors. Neurosurgery New York. McGraw Hill Co. 1985; 1 : 964-76.
   
4. Nath K, Gopi R, Khan AA, et al. Vascular hamartoma and vascular tumours of the orbit. Ind J Ophthal 1977; 25 : 18-23.
   
5. Yan J, Wu Z. Cavernous hemangioma of the orbit: analysis of 214 cases. Orbit 2004; 23 (1) : 33-40.
   
6. Mercandetti M, Cohen AJ. Exophthalmos. eMedicine Journal [serial online]. 2001. Available at: http://www.emedicine.com/oph/topic616.htm.
   
7. Henderson GW. Vascular hamartomas, hyperplasias, and neoplasms. In: Henderson, ed. Orbital Tumours. New York: Raven Press; 1994: 94-100.
   

*Associate Professor, **Resident, Department of Ophthalmology, T.N.M.C and B.Y.L. Nair Hospital, Mumbai - 400 008.