Orbital Varix

Orbital varix is a tumour (vascular hamartoma) which comprises of collection of venous channels. Primary varix may be a congenital lesion or it may be acquired following trauma. Orbital varix may be distensible or non-distensible. It presents with intermittent proptosis which increases with straining, crying or stooping.

Any recent thrombosis or haemorrhage is associated with rapid development of proptosis, pain and limitation of ocular movement. On the other hand, atrophy of orbital fat may produce enophthalmos in some cases. Pulsations may be present with an associated arteriovenous malformation or with transmitted intracranial pulsations due to a mass adjacent to a bony defect.

Orbital varix is usually diagnosed between the ages of 10 to 30 years. However, it may be seen in any age group, including neonates. Varix usually affects superior ophthalmic vein. Other veins of the orbit may also be involved.

 

References

Basak Samar K. Atlas of Clinical Ophthalmology Second Edition. Jaypee Brothers Medical Publishers (P) Ltd 2013. P 418.

Garg Ashok, Alio Jorge L. Surgical Techniques in Ophthalmology- Oculoplasty and Reconstructive Surgery. Jaypee Brothers Medical Publishers (P) Ltd 2010. P 158.

Kalevar V. Clinical Ophthalmology. Ane Books India 2008. P 411- 412.

Selvakumar Ambika, Naronha Veena, Sundaram Padmaja Minakshi. Sankara Nethralaya Atlas of Imaging in Ophthalmology. Jaypee Brothers Medical Publishers (P) Ltd 2014. P 208- 209.

Ahmed E. Comprehensive Manual of Ophthalmology. Jaypee Brothers Medical Publishers (P) Ltd 2011. P 138.

Haaga John R, Dogra Vikram S, Forsting Michael, Gilkeson Robert C, Ha Hyun Kwon, Sundaram Murali. CT and MRI of the Whole Body Volume-1 Fifth Edition. Mosby Elsevier 2009. P 490. 

Karcioglu Zeynel A. Orbital Tumors- Diagnosis and Treatment Second Edition. Springer Science+Business Media New York 2015. P 166- 168.

Shields Jerry A, Shields Carol L. Eyelid, Conjunctival, and Orbital Tumors- An Atlas and Textbook Second Edition. Lippincott Williams & Wilkins, a Wolters Kluwer business 2008. P 542.

Garrity James A, Henderson John Warren, Cameron J Douglas. Henderson’s Orbital Tumors Fourth Edition. Lippincott Williams & Wilkins, a Wolters Kluwer business 2007. P 230.

Wright Kenneth W, Spiegel Peter H. Pediatric Ophthalmology and Strabismus Second Edition. Springer-Verlag New York Inc. 2003. P 325.

Midyett F Allan, Mukherji Suresh K. Orbital Imaging. Elsevier Saunders 2015. P 139- 142.

Heegaard Steffen, Grossniklaus Hans. Eye Pathology- An Illustrated Guide. Springer-Verlag Berlin Heidelberg 2015. P 559- 560. 

Kupersmith MJ, Berenstein A. Neurovascular Neuro-ophthalmology. Springer-Verlag Berlin Heidelberg 1993. P 156.

Yousem David M, Grossman Robert I. Neuroradiology Third Edition. Mosby Elsevier 2010. P 336.

Gonzalez Carlos F, Becker Melvin H, Flanagan Joseph C. Diagnostic Imaging in Ophthalmology. Springer-Verlag New York, Inc. 1986.

Harrie Roger P, Kendall Cynthia J. Clinical Ophthalmic Echography- A case Study Approach Second Edition. Springer Science+Business Media New York 2014. P 315.

Bowling Brad. Kanski’s Clinical Ophthalmology- A Systematic Approach Eighth Edition. Elsevier 2016. P 51.

http://eyewiki.aao.org/Orbital_varices

http://www.ijo.in/article.asp?issn=0301-4738;year=1984;volume=32;issue=4;spage=235;epage=237;aulast=Kulshrestha

http://onlinelibrary.wiley.com/doi/10.1034/j.1600-0420.1999.770420.x/pdf

Wright JE. Trans. Am Acad. Ophthalmol. Otolaryngol., 1974; 78: 606.

Llyod GA, Wright JE, Morgan G. Br. J. Ophthalmol., 1971; 55: 505.

Wright (1974) has classified initial symptoms of orbital varices into five groups as

  • Variable proptosis, associated with dilated veins in the eyelids and episcleral tissue.
  • Dilated veins in the eyelid and anterior orbit.
  • Variable proptosis without any visible lesions.
  • Acute orbital haemorrhage.
  • Acute thrombophlebitis.

In addition, venous varices may be associated with

  • Recurrent orbital pain.
  • Diminution of vision due to involvement of optic nerve.
  • Ocular movement disorders.

Venous varices are rare orbital lesions which causes characteristic intermittent proptosis.

Llyod (1971) divided orbital varices into two types

  • Varices not associated with an arterio-venous malformation within orbit or cranium. These represent primary congenital venous malformations which are not secondary to arterialisation of venous system.
  • Varices produced secondary to an intra-orbital or intra-cranial arterio-venous communication. These angiomas are usually found in the middle cranial fossa with venous communication through superior orbital fissure.

Although varix is most probably congenital in origin, the clinical signs may not become apparent until later in life, most cases presenting themselves during first three decades of life.

Clinical features

Orbital varix is usually unilateral, vascular malformation (commonly venous in origin).

There is non-pulsatile, intermittent axial proptosis which is not associated with bruit. There may be associated prominent conjunctival vessels. Proptosis may be accentuated or precipitated by dependent head posture or by Valsalva manoeuvre.

It may be associated with vascular lesions of eyelids or conjunctiva, and may be of arterial or venous origin.

There may be enophthalmos due to fat atrophy.

It may show occasional bruising. There may be extensive involvement of peri-orbital or intra-cranial regions.

Histopathology

Varix is seen as one or more dilated veins, frequently with thrombosis and hyalinisation. Thrombosed varix may lead to pathologic reaction showing intravascular papillary endothelial hyperplasia.

 

Investigations

  • X-ray: X-ray may give evidence of calcification due to phlebolith.
  • CT Imaging: An orbital varix has globular or smooth fusiform appearance, which enhances strongly with contrast medium. It increases in size on Valsalva manoeuvre on dynamic CT scanning.
  • MRI Imaging: MRI signal depends upon presence of flow within varix. Patent lumen appears as an area of flow void on both T1 and T2-weighted scans. Contrast enhancement is variable and may be absent. Slow flow or thrombus in varix will give it a slight heterogeneous appearance.
  • A and B-scan ultrasonography: There is increased size of the lesion on ultrasonography.
  • Venography: This may demonstrate abnormal saccular vessels flowing out through venous channels.
  • Colour Doppler Imaging: This may be used to demonstrate an orbital varix. This is a non-invasive method of investigation.

 

Differential diagnosis

Orbital varix should be distinguished from other vascular lesions of the orbit such as

  • Haemangioma.
  • Lymphangioma.
  • Idiopathic orbital inflammation.
  • Carotid-cavernous fistula.
  • Dural shunt.

Management of orbital varix is difficult.

Most orbital varices are left as such and are observed over time.

Surgical therapy

Surgical excision is rather difficult. Intraoperative venography and glue embolisation may be helpful. This may be followed by excision.

 

Prognosis

Prognosis is good in most patients with primary orbital varix. Recurrence after subtotal excision is an issue of concern. Most patients remain stable without evidence of re-bleeding.

  • PUBLISHED DATE : Dec 26, 2017
  • PUBLISHED BY : NHP Admin
  • CREATED / VALIDATED BY : Dr. S. C. Gupta
  • LAST UPDATED ON : Dec 26, 2017

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