Unusual extraconal presentation of orbital cavernous hemangioma

Harikrishnan Marappan; Deepika Makam; Keerthi Ballela

doi:10.4103/jocr.jocr_28_21

CASE REPORT

Year : 2022 | Volume : 2 | Issue : 1 | Page : 51-53

Unusual extraconal presentation of orbital cavernous hemangioma

Harikrishnan Marappan, Deepika Makam, Keerthi Ballela
Department of Oculoplastics, Lotus Eye Hospital and Institute, Coimbatore, Tamil Nadu, India

Date of Submission	19-Sep-2021
Date of Decision	22-Sep-2021
Date of Acceptance	13-Nov-2021
Date of Web Publication	05-Oct-2022

Correspondence Address:
Dr. Harikrishnan Marappan
Lotus Eye Hospital and Institute, Coimbatore, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jocr.jocr_28_21

Abstract

A 27-year-old male patient complained of dull aching pain in the right eye (RE) for 3 months. On examination, the patient had mild proptosis of RE with downward displacement and fullness in superotemporal quadrant. No palpable mass was felt, and finger insinuation was possible, the swelling did not change in size on bending, coughing, and not pulsatile, and no bruit was heard. Visual acuity, color vision, and confrontation were normal. Mild ptosis with minimal abduction and elevation restriction was noted. MRI orbits revealed well defined fusiform, lobulated, extraconal soft tissue lesion in the superotemporal aspect causing abaxial proptosis with scalloping and thinning of bony orbital roof. Histopathology reveals features suggestive of cavernous hemangioma. Even though the most common location of cavernous hemangioma is intra-conal, its extra- conal occurrence also possible and needs high suspicion.

Keywords: Cavernous hemangioma, extraconal, proptosis

How to cite this article:
Marappan H, Makam D, Ballela K. Unusual extraconal presentation of orbital cavernous hemangioma. J Ophthalmol Clin Res 2022;2:51-3

How to cite this URL:
Marappan H, Makam D, Ballela K. Unusual extraconal presentation of orbital cavernous hemangioma. J Ophthalmol Clin Res [serial online] 2022 [cited 2023 Oct 4];2:51-3. Available from: http://www.jocr.in/text.asp?2022/2/1/51/357892

Introduction

Cavernous hemangiomas are venous malformations and are the most common benign orbital tumor in adults. It presents as a slowly progressive tumor of the orbit seen mostly in middle-aged population with female preponderance. The most common occurrence of the tumor is (intraconal), i.e., within the muscle cone lateral to the optic nerve. In this case report, we present an extraconal and superotemporal location of cavernous hemangioma.

Case Report

A 27-year-old male patient presented with complaints of dull aching pain, more like pressure sensation in the right eye (RE) for 3 months. On examination, the patient had mild proptosis with downward displacement and fullness in the superotemporal quadrant. No palpable mass was detected, and finger insinuation was possible [Figure 1]. The swelling did not change in size on bending, coughing, or straining. The swelling was not pulsatile, and no bruit was heard. His unaided visual acuity was 6/6 in both eyes. His color vision and confrontation visual fields were normal, extra-ocular movements revealed mild abduction and elevation restriction in right eye. Right eye evaluation showed mild upper eyelid ptosis of 1 mm with good levator function of 16 mm, good bells phenomenon and adequate lid closure.

Figure 1: Clinical image – right eye mild proptosis with inferior dystopia

Click here to view

Magnetic resonance imaging (MRI) of the orbits with contrast revealed well-defined fusiform (lobulated) extraconal soft tissue lesion measuring 29 mm anteroposterior (Ap) × 25 mm transverse (trans) × 18 mm craniocaudal (cc) in the superotemporal aspect of the right orbit. It is causing mass effect over the eyeball, causing mild abaxial proptosis with scalloping and thinning of the bony orbital roof suggestive of benign neoplasm [Figure 2]a. The lesion is hypointense on T1 and hyperintense on T2 with mild heterogeneous contrast enhancement [Figure 2]b.

Figure 2: Radiological images. (a) Coronal T1-weighted magnetic resonance imaging image shows a well-defined extraconal lesion in superotemporal aspect, isointense to extraocular muscle causing globe compression and inferior dystopia, (b) T1-weighted magnetic resonance imaging image in sagittal section

Click here to view

The patient underwent an excisional biopsy through anterolateral orbitotomy.

The operation and postoperative course were uneventful.

Histopathology

Histopathological examination shows a circumscribed lesion with proliferating large thick-walled blood vessels filled with blood and few organizing thrombi [Figure 3]a. Perivascular hyalinisation with intervening fibromyxoid stroma and lymphoplasmacytic infiltration also noted [Figure 3]b. All these features are suggestive of cavernous hemangioma.

Figure 3:Histopathological images. (a) (White arrow) dilated vessel wall with red blood cells (black empty arrow) lined by flattened endothelial cells. Thrombi in the vessel wall (black filled arrow) (H and E, ×10), (b) (Black filled arrow) cyst-like dilated vessels filled with red blood cell separated by fibrous stroma (black empty arrow) and myxoid stroma (white filled arrow) (H and E, ×4)

Click here to view

Discussion

Cavernous hemangioma is the third most common benign orbital tumor in adults, the first being lymphoid tumors followed by orbital inflammatory tumors and is the most common vascular lesion of the orbit.^[1] Sixty percent of cases occur in women because of the influence of estrogen and progesterone, which seem to have an effect on progression.^[2],[3]>

The most common presentation is exophthalmos in 70%–95% of cases, and proptosis is axial when the lesion is located intraconal. Extraconal location of tumor displaces the eyeball in the opposite direction of the location of tumor. Deterioration of vision is another common symptom seen in 50% of cases when tumor compresses on the optic nerve. Choroidal folds and optic disc changes may also be present when the lesion is situated in the vicinity of the globe. Other manifestations such as motility deficits and visual field changes are present in 20% cases^[4] and 6.5% patients present with pain as a presenting complaint,^[3] mostly described as pressure sensation in the eye like in our case.

Less common manifestations including double vision or gaze-evoked transient loss of vision (amaurosis) are seen with intraconal or extraconal lesions caused by optic nerve ischemia and transitory axonal inhibition.^[4]

Cavernous hemangiomas most frequently occur intraconally, i.e., within the cone in the lateral part.^[5] Extraconal occurrence of hemangioma is very rare, and so only a few cases have been reported in the literature. One such case was reported by Hentati et al. having a bilateral cavernous hemangioma with extraconal superomedial location of lesion in RE with intraconal lesion in left eye.^[5] They are usually solitary, but multifocal lesions have been reported and 5%–10% of lesions extend intracranially through a superior orbital fissure.

On MRI, it appears homogeneous and isointense to muscle on T1 and hyperintense to muscle on T2 imaging with a well-defined capsule.^[1]

Histopathological examination reveals a well-defined capsule enclosing multiple large and dilated vascular channels lined by endothelial cells and abundant stroma. The lumen contains blood at various stages of thrombi, indicating slow flowing of blood or stasis.^[6]

Surgical treatment is indicated in the case of symptomatic hemangioma, optic nerve compression, and cosmetically disfiguring proptosis, and the type of approach to the surgery depends on the anatomical location of the tumor.^[7]

Conclusion

Cavernous hemangiomas are most commonly seen in the intraconal space lateral to the optic nerve. However, it can also have an extraconal occurrence in few cases and needs high suspicion. CT and MRI can efficiently demonstrate extraconal location.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Young SM, Kim YD, Lee JH, Woo KI. Radiological analysis of orbital cavernous hemangiomas: A review and comparison between computed tomography and magnetic resonance imaging. J Craniofac Surg 2018;29:712-6.
2.	Jayaram A, Lissner GS, Cohen LM, Karagianis AG. Potential correlation between menopausal status and the clinical course of orbital cavernous hemangiomas. Ophthalmic Plast Reconstr Surg 2015;31:187-90.
3.	Yan J, Wu Z. Cavernous hemangioma of the orbit: Analysis of 214 cases. Orbit 2004;23:33-40.
4.	Costan, Victor Vlad. Histopathological and Clinical Characteristics of Surgically Removed Cavernous Venous Malformations (so-Called Cavernous Hemangiomas) of the Orbit. Revista de Chimie, vol. 70, no. 1, Revista de Chimie SRL, Feb. 2019, p. 350-54. Crossref, https://doi.org/10.37358/rc.19.1.6914.
5.	Hentati A, Matar N, Dridi H, Bouali S, Jemel H. Bilateral orbital cavernous hemangioma. Asian J Neurosurg 2018;13:1222-4. [PUBMED] [Full text]
6.	Rootman DB, Heran MK, Rootman J, White VA, Luemsamran P, Yucel YH. Cavernous venous malformations of the orbit (so-called cavernous haemangioma): A comprehensive evaluation of their clinical, imaging and histologic nature. Br J Ophthalmol 2014;98:880-8.
7.	Calandriello L, Grimaldi G, Petrone G, Rigante M, Petroni S, Riso M, et al. Cavernous venous malformation (cavernous hemangioma) of the orbit: Current concepts and a review of the literature. Surv Ophthalmol 2017;62:393-403.