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Year : 2008  |  Volume : 18  |  Issue : 4  |  Page : 310-312
Extraconal cavernous hemangioma of orbit: A case report


Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India

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   Abstract 

Cavernous hemangioma is the most common benign noninfiltrative neoplasm of the orbit. Most cavernous hemangiomas are intraconal and lateral in location. We present a case of a cavernous hemangioma with an unusual extraconal and superomedial location.

Keywords: Extraconal; hemangioma; orbit

How to cite this article:
Anand R, Deria K, Sharma P, Narula M K, Garg R. Extraconal cavernous hemangioma of orbit: A case report. Indian J Radiol Imaging 2008;18:310-2

How to cite this URL:
Anand R, Deria K, Sharma P, Narula M K, Garg R. Extraconal cavernous hemangioma of orbit: A case report. Indian J Radiol Imaging [serial online] 2008 [cited 2014 Oct 22];18:310-2. Available from: http://www.ijri.org/text.asp?2008/18/4/310/43849
Cavernous hemangioma is the most common benign noninfiltrative neoplasm of the orbit. Most cavernous hemangiomas are intraconal and lateral in location. They result from the new formation of vessels, proliferation of tissue components of the vessel wall, and hyperplasia of cellular elements ordinarily concerned with the genesis of vascular tissue. [1] A case of cavernous hemangioma with an unusual extraconal superomedial location is presented.


   Case Report Top


An 18-year-old woman presented with gradually progressive proptosis of the left eye with swelling of the superomedial quadrant of the left orbit. The swelling did not change in size with the Valsalva maneuver, coughing, straining, or change in head position. The cornea and sclera were normal.

USG revealed a well-encapsulated, compressible, echogenic mass lesion (approximately 2.8 1.7 cm in size) situated superomedially in the extraconal space of the left orbit. On Doppler, the mass showed multiple vascular channels (both arterial and venous channels) suggestive of a vascular lesion [Figure 1]. Small low-flow feeding arteries were seen. MRI showed an oval, encapsulated, superomedial, and extraconal mass in the left orbit. The mass appeared isointense to orbital muscle on T1W [Figure 2] and mildly hyperintense to orbital muscle on T2W [Figure 3] images, with intense enhancement on post-gadolinium scans [Figure 4a] and [Figure 4b]. There was lateral displacement of the medial rectus muscle anteriorly with inferolateral displacement of the globe. The intraocular muscles and optic nerve sheath complex showed normal signal intensity.

The tumor was excised using an anterior orbitotomy approach. Histopathology confirmed the diagnosis of a cavernous hemangioma.


   Discussion Top


Hemangiomas (benign vascular neoplasms) are classified as capillary and cavernous. A capillary hemangioma usually presents in the first year of life and often increases in size for 6-10 months before slowly involuting. Cavernous hemangiomas are the most common benign noninfiltrative neoplasms of the orbit and have a slowly progressive mass effect. [2] They are usually present in the second to fourth decades of life and are more frequent in females. [3],[4]

A slowly progressive proptosis is the typical presenting symptom. Extraocular muscle impairment and impaired visual function are seen with large lesions and with lesions located at the orbital apex. Clinically, these tumors are soft and do not change in size with the Valsalva maneuver or with coughing, straining, or change in the head position. [3],[4]

Most cavernous hemangiomas are typically intraconal and lateral in location. Extraconal and medial locations are uncommon. [3],[5] USG, CT scan, and MRI are useful imaging techniques for the evaluation of cavernous hemangiomas. Angiography is rarely required. [5],[6]

The tumors are round to oval in shape with well-defined borders and a specific 'honeycomb' pattern of alternating weak and strong echoes corresponding to their structure, with flow on color Doppler. [3],[5] They may show a negative Doppler phenomenon which is attributable to the stagnant blood within the vascular spaces. [7]

CT scan shows discrete lesions with varying degrees of enhancement. On MRI, the lesions are homogenous, isointense to muscle on T1W images, and hyperintense to muscle on T2W images, as was seen in the present case. Variable homogenous or inhomogeneous contrast enhancement is the rule. [3],[5] These lesions have a small arterial input with small venous outflow channels and very slow flow within. [6]

Histopathology reveals a fine capsule that surrounds a tumor consisting of large endothelium-lined channels with abundant, loosely distributed smooth muscle in the vascular wall and stroma. [3],[6]

Treatment of the tumor is surgical excision. Complete excision is generally accomplished as the tumor is well encapsulated with relatively few feeding vessels. [6],[8]

 
   References Top

1.Rosca TI, Pop MI, Curca M, Vladescu TG, Tihoan CS, Serban AT, et al . Vascular tumors of the orbit-cavernous and capillary hemangiomas. Ann Diag Pathol 2006;10:13-9.  Back to cited text no. 1    
2.Rodgers R, Grove AS Jr. Vascular lesions of the orbit. Principles and practice of ophthalmology. 2nd ed. p. 3144-55.   Back to cited text no. 2    
3.Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: An analysis of 66 cases. J Neurosurg 1979;51;219-28.   Back to cited text no. 3    
4.Bilaniuk LT. Orbital vascular Lesions: Role of Imaging. Radial Clin North Am 1999;37:169-83.   Back to cited text no. 4    
5.Davis KR, Hesselink JR, Dallow RL, Grove AS Jr. CT and ultrasound in the diagnosis of cavernous hemangiomas of the orbit. J Comput Tomogr 1980;4:98-104.   Back to cited text no. 5  [PUBMED]  
6.Rootman J. Diseases of the orbit - A multi disciplinary Approach. 2nd ed. 2003.  Back to cited text no. 6    
7.Lerine RA. Orbital ultrasonography. Radiol Clin North Am 1987;25:447-69.   Back to cited text no. 7    
8.Missori P, Tarantino R. Delfini R, Lunardi P, Cantore G. Surgical management of Orbital cavernous angiomas: Prognosis for visual function after removal. Neurosurgery 1994;35:34-8.  Back to cited text no. 8    

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Correspondence Address:
Kavita Deria
Flat No. 3583, Sector-D, Pocket-3, Vasant Kunj, New Delhi-110 070
India
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DOI: 10.4103/0971-3026.43849

PMID: 19774188

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4a], [Figure 4b]

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