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Management Dilemma : Intra-orbital Haemangioma
Chinmaya Sahu*, Hemkala Trivedi**, Sujit Murade*
 
 

We would like to present a case of a patient who presented to us in the OPD with the chief complaints of the diplopia. At the outset the case looked insignificant and as most often is the case we thought that the patient is malingering. The patient did not have proptosis neither was there any restriction of ocular movements, so why should she have diplopia? But what unfolded was quite unsual not only because of the rarity of the condition but also for the management dilemma it presented to us

Case Report
Tajrunissa, a 28 yr old married female came with the history of sudden onset diplopia and diminution of vision since 3 months. The patient was apparently alright 3 months back when she first started experiencing headache, slight diminution of vision and seeing objects double with both the eyes open and more so on the downward and outward gaze (Fig. 1). The patient had no complaints of redness, watering, headache, vomiting or any significant medical or surgical illness.



Fig. 1 : Normal looking patient.
Her examination findings were as follows,
LE
Vn: 6/9 with pin hole no improvement
Lid : N
Conjunctiva : N
Cornea : Clear
AC/ Iris : N
Pupil : Reacting to light
Lens : Clear
Fundus : Posterior Choroidal Folds, otherwise N
Tension : 17.3 mm Hg
Movements : Full
Proptosis : Absent
   

Fig. 2 : Choroidal folds.

Fig. 3 : Intra-orbital haemangioma.

Diplopia Charting
Following this a, Presumptive diagnosis of posterior Choroidal Folds secondary to some intra-orbital pathology was arrived at (Fig. 2).

Also it was hypothesized that the diplopia was due to the slight displacement of the fovea leading to Anomalous Retinal Correspondence.

The patient was advised to undergo a USG B- Scan.

The B-Scan showed the presence of an Intra-Orbital Haemangioma (Fig. 3).

She was referred to our Neuro-Surgery Department where she underwent removal of the mass after careful dissection.

The patient was fine following the surgery and showed improvement in her vision with abatement of diplopia.

The patient again came back to us 1.5 months back with the chief complaint of recurrence of Double vision on downward and outward gaze.

A detailed Ophthalmic examination was done which showed presence of Choroidal folds and presence of Diplopia on downward and outward gaze.

A CT scan was done for the patient which showed a recurrence of the intra-orbital haemangioma.

Management Dilemma

  1. Was the resection warranted?
  2. What can be done for her diplopia? Is surgery the answer for this?

Treatment
The patient underwent a local resection of the mass but the diplopia has recurred .

So, we are considering the further management options for this patient.

Should we prescribe prism glasses for her?

Should she undergo another surgery to release the traction in the posterior pole region?

Or should we just patch her left eye and follow her up routinely after adequately counseling her? If so for how long can we keep patching her?

Discussion

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.

Pathophysiology : 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. Lagophthalmos can result in exposure keratopathy, keratitis, and corneal perforation.

Frequency
  • Internationally: Henderson (1984) reported an incidence of 4.3% among orbital neoplasms.

    Sex : Harris and Jakobiec (1978) found a 7:3 occurrence ratio of women to men, while Henderson (1984) reported an almost equal ratio, 8:7 in women and men.

    Age : Patients usually manifest symptomatically during the third to fifth decades of life.

    History : Patients who present with clinically significant cavernous haemangiomas usually are middle-aged. Some of the more salient clinical symptoms are listed below.
  • Patients commonly describe a painless, slowly progressive protrusion 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.
  • Extraocular muscle dysfunction and the resultant diplopia secondary to extraocular muscle impingement can cause a patient to seek consultation.
  • Some patients may describe the feeling of something next to or behind their eye and describe swelling or fullness of their upper lid.
  • Rarely, a patient harbouring a cavernous angioma may describe gaze-evoked amaurosis fugax or headache.
  • Zauberman and Feinsod described a pregnancy-induced increase in symptomatology.

Physical : Performing a complete ophthalmologic examination on patients is beneficial.

  • As with any examination, a thorough history and review of symptoms is paramount in formulating a comprehensive list of differential diagnoses.
  • Examination of patients should commence with an observation of facial features, noting any asymmetry or scarring. Palpation of the lids and globes allow one to assess differences in lid fullness and increased resistance to retropulsion. Hertel exophthalmometry can detect axial proptosis and should be documented for comparison on follow-up visits.
  • Visual and color acuities, as well as visual fields, should be assessed, followed by testing of pupillary and extraocular muscle function. Decreased colour vision, visual field deficits, and relative afferent pupillary defects warrant immediate imaging to rule out a compressive optic neuropathy. Additionally, any extraocular motility disturbance should be quantitated with prismatic measurements.
  • Rarely, slit lamp or penlight evaluation may find dilated and tortuous epibulbar vessels, an epibulbar cherry-red spot, or a darkening over insertions of extraocular muscles.
  • Dilated fundoscopic examination may elucidate choroidal folds secondary to compression of the globe by the mass. If the tumefaction is in close proximity to the optic nerve, visible changes may include oedema, elevation, pallor, and even atrophy in severe cases.
  • Fluorescein angiography :
    • Alternating hypo- and hyper-fluorescent lines correseponding to the folds orientation that persist throughout all phases of the study

    • The hypofluorescent lines correspond to the dark bands where the RPE cells are compressed in the valleys or troughs of the folds

    • The hyperfluorescein lines correspond to the light bands where the Bruch’s membrane and RPE cells are stretched on the peak of the folds

Age: Diplopia is encountered almost exclusively in adults or in those with mature visual systems because of the following :

  • Young children may not be able to express this symptom. More importantly, the immature visual system deals with diplopia by suppressing the poorer image, possibly resulting in irreversible amblyopia. Children with obvious and marked ocular malalignment from strabismus are comfortable and content because the visual image from the deviating eye is suppressed and not noticed.
  • In contrast, adults who have mature visual processing pathways cannot easily ignore the second image. Wearing a patch over one eye to prevent visual confusion sometimes is the only option for these patients.

An orbital haemangioma is typically a slow-growing tumour.

If there is no eye damage, they can be observed for growth (with serial radiographic studies) prior to considering intervention.

Indications for treatment :

  1. There is evidence of growth.
  2. Optic nerve compression.
  3. Corneal exposure (with secondary keratitis sicca)
  4. Evidence of vision loss.

Goal of local resection for choroidal haemangioma :

Complete removal of the tumour. This usually involves careful dissection of the tumour to protect the tumour’s capsule (as possible). Connecting vascular feeder vessels should be identified and cauterized. A lateral orbitotomy is usually required to keep the large tumour intact.

References

  1. Vascular malformations of the orbit: hemodynamic concepts. J Rootman - Orbit, 2003 - taylorandfrancis.metapress.com
    ... 22 No. 2, pp. 103–120 © Swets and Zeitlinger 2003 Accepted 25 November 2002 Vascular malformations of the orbit: hemodynamic concepts Jack Rootman, MD ...
  2. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg1982; 69: 412-20.
    Simpson JR. Natural history of cavernous haemangiomata.
  3. Ellis PP. Occlusion of the central retinal artery after retrobulbar corticosteroid injection. Am J Ophthalmol 1978; 85: 352-6.
  4. Gangopadhyay AN, Sinha CK, Gopal SC, Gupta DK, Sahoo SP, Ahmad M. Role of steroid in childhood haemangioma: a 10 years review. Int Surg 1997; 82: 49-5 .
  5. Ezekowitz RAB, Mulliken JB, Folkma J. Interferon alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med 1992; 326: 1456-63.
  6. Stigmar G, Crawford JS, Ward CM, Thomson HG. Ophthalmic sequelae of infantile hemangiomas of the eyelids and orbit. Am J Ophthalmol 1978; 85 : 806-13.
    Boyd MJ, Collin JRO. Capillary haemangiomas: an approach to their management. Br J Opthalmol 1991; 75: 298-300.
  7. Haik GR, Jakobiak FA, Ellsworth RM, Jones IS. Capillary hemangioma of the lids and orbit: an analysis of the clinical features and therapeutic results in 101 cases. Ophthalmology 1979; 86 : 760-89.

SCREENING FOR DEPRESSION IN PRIMARY CARE WITH TWO VERBALLY ASKED QUESTIONES : CROSS SECTIONAL STUDY
1. During the past month have you often been bothered by feeling down, depressed, or hopeless?
2. During the past month have you often been both ered by little interest or pleasure in doing things?

BMJ, 2003; 327 : 1144.

 

 

*Resident; **Assoc. Prof. Acting Head, Department of Ophthalmology, TNMC and BYL Nair Hospital, Mumbai Central, Mumbai 400 008.

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