Shenelle Wickramarathna

Introduction

Orbital varices are characterized by thin-walled, distensible venous channels that communicate with the normal orbital venous system. These rare venous malformations account for approximately 1.3% of all orbital masses (1). Varices can be classified as primary or secondary; primary varices are congenital, while secondary orbital varices are acquired, often resulting from arteriovenous malformations or arteriovenous fistulas (2). Due to their rarity, orbital varices present diagnostic and management challenges. However, timely identification is crucial, as they can lead to significant complications, including thrombosis (4,5) and haemorrhage (5, 6). 

Presentation 

While patients with orbital varices may be asymptomatic, the hallmark clinical feature is a transient proptosis induced by actions that increase venous pressure, such as the Valsalva manoeuvre, coughing, or bending forward (1). This occurs due to the engorgement of the varices, as venous hypertension is easily transmitted through these interconnected, valveless veins. 

In addition to proptosis, orbital varices may present with unilateral periorbital pain (3, 4) or diplopia (7) resulting from the compression of extraocular muscles. 

Orbital varices are particularly prone to thrombosis. The absence of valves in the orbital veins facilitates increased blood volume and slowed venous return, predisposing to thrombus formation. Thrombosis within an orbital varix may occur due to venous stasis, endothelial damage, or hypercoagulability. A thrombosed varix may compromise the optic nerve via mass effect, leading to severe vision impairment (8), or extraocular muscle compression, causing diplopia. In extreme cases, spontaneous haemorrhage may occur, and there have been reported cases of orbital compartment syndrome—a sight-threatening emergency that necessitates urgent intervention (6). 

Diagnosis 

Clinical examination, including slit-lamp evaluation, may be unremarkable even in the presence of an orbital varix. Therefore, imaging is essential for diagnosis. 

Ultrasound and Doppler can demonstrate venous flow and detect variceal distensibility (9, 10, 12). While CT and MRI are preferred imaging techniques, particularly with dynamic manoeuvres like the Valsalva manoeuvre, which reveal the distensibility of the varix—a hallmark diagnostic feature. Imaging also aids in ruling out differential diagnoses, such as arteriovenous fistulas or orbital lymphangioma (9, 10).

Management 

Due to the rarity of thrombosed orbital varices, standardized treatment guidelines are lacking. Management is typically individualized based on the severity of symptoms and complications. 

Conservative Management

Asymptomatic or mildly symptomatic varices are often managed with observationand serial imaging. Low-dose anticoagulation may be employed to prevent further thrombosis. (3, 12) 

Medical Management

Corticosteroids and analgesia can be used to manage pain and inflammation in thrombosed varices without acute vision-threatening complications (4, 5). 

Surgical Intervention

Generally reserved for cases with extreme orbital pressure, functional deficits, intractable pain, or cosmetic disfigurement (6, 7, 11). 

Emergent Procedures

In rare cases, lateral canthotomy may be required to relieve orbital compartment syndrome caused by a thrombosed varix (6). 

Other Treatment Options

Sclerotherapy and embolization have been described in the literature as alternative treatment modalities for symptomatic or recurrent cases (13). 

Conclusion

Orbital varices, though rare, are clinically significant entities that can lead to vision-threatening complications when thrombosed. Early recognition through appropriate imaging and individualized management are crucial for optimizing patient outcomes. Further research and case studies are needed to develop standardized guidelines for the diagnosis and management of thrombosed orbital varices. 

References 

(1) Karcioglu ZA. Berlin: Springer; 2015. Orbital Tumors: Diagnosis and Treatment.  

(2) Wright JE, Sullivan TJ, Garner A, Wulc AE, Moseley IF. Orbital venous anomalies. Ophthalmology. 1997;104:905-13.  

(3) Bullock, John D., Stuart H. Goldberg, and Patrick J. Connelly. “Orbital varix thrombosis.” Ophthalmology 97.2 (1990): 251-256.  

(4) Wade, Ryckie George, Thomas B. Maddock, and Srinivasan Ananth. “Orbital varix thrombosis: a rare cause of unilateral proptosis.” Case Reports 2013 (2013): bcr2012007935.  
(5) Haizul, I. M., et al. “Spontaneous Retrobulbar Haemorrhage Secondary to Orbital Varices of Inferior Ophthalmic Vein-A Case Report.” Journal of Surgical Academia 2.2 (2012): 49-51.  

(6) Ma, Shang-Te, Shu-Lang Liao, and Yi-Hsuan Wei. “Orbital compartment syndrome and irreversible blindness related to orbital varix thrombosis: a case report.” The Journal of Emergency Medicine 60.3 (2021): 377-379.  

(7) Pappas A, Araque JM, Sarup V. Orbital Venous Varices: A Rare Bilateral Asymptomatic Presentation. Cureus. 2018 Sep 14;10(9):e3302. doi: 10.7759/cureus.3302. PMID: 30705795; PMCID: PMC6349571. 

(12) Secil M, Soylev M, Ada E, Saatci AO. Orbital varices: imaging findings and the role of color Doppler sonography in the diagnosis. Comput Med Imaging Graph. 2001 May-Jun;25(3):243-7. doi: 10.1016/s0895-6111(00)00080-x. PMID: 11179700. 

(8) Go JL, Fasula V, Sirotkin I. Orbital Varix Masquerading as an Intraorbital Lymphoma. Fed Pract. 2021 Sep;38(9):431-434. doi: 10.12788/fp.0182. Epub 2021 Sep 15. PMID: 34737541; PMCID: PMC8562897. 

(9) Menon, Smita V., et al. “Thrombosed orbital varix–a correlation between imaging studies and histopathology.” Orbit 23.1 (2004): 13-18.  

(10) Ulus, Ozden Sila, and Ercan Kararaslan. “Orbital varix: CT and MRI findings.” European Journal of Radiology Extra 75.2 (2010): e51-e53.  

(11) Pichayawat C, Chokthaweesak W, Leelawongs S, Chanthanaphak E, Tritanon O, Putthirangsiwong B. Acute unilateral orbital varix thrombosis in preexisting bilateral orbital varices: illustrative case. J Neurosurg Case Lessons. 2023 Jun 19;5(25):CASE23132. doi: 10.3171/CASE23132. PMID: 37354429; PMCID: PMC10550533. 

(12) Howells MS, Sharma R. Orbital varices. BMJ Case Rep. 2019 Dec 8;12(12):e232887. doi: 10.1136/bcr-2019-232887. PMID: 31818898; PMCID: PMC6904158. 

(13) Arat, Y. O., Mawad, M. E., & Boniuk, M. (2004). Orbital venous malformations: current multidisciplinary treatment approach. Archives of Ophthalmology, 122(8), 1151-1158.