Retinal vein occlusion is the second most common vascular retinopathy leading to visual impairment.1,2 Central retinal vein occlusion (CRVO) is characterized by acute, painless loss of vision and fundus findings of diffuse intraretinal hemorrhages and venous tortuosity.3 The most common risk factors for CRVO are systemic hypertension, diabetes mellitus, thrombophilia or hypercoagulable states, and glaucoma.3–6 Dehydration has also been identified as a potential risk factor in young patients.2,7–10 We report a case of CRVO in a young patient associated with prolonged vomiting and repeated Valsalva maneuvers due to acute gastroenteritis with dehydration.
A 44-year-old man was referred for acute, painless decreased vision for 1 week in his right eye. Ophthalmic records indicated that his previous best corrected visual acuity (BCVA) was 20/20. He had no significant medical history; he denied a history of diabetes, hypertension, migraines, or thrombophilia. He had an episode of prolonged vomiting and repeated Valsalva maneuvers associated with acute gastroenteritis and dehydration the day preceding the onset of decreased vision.
The patient’s BCVA was 20/70 in the right eye and 20/30 in the left. The intraocular pressure by applanation tonometry was 9 mm Hg bilaterally. Diffuse intraretinal hemorrhages and dilated retinal veins were present in all four quadrants in the right eye (Figure 1), and prominent cystoid macular edema (CME) was present. Spectral-domain optical coherence tomography (SD-OCT) of the right eye demonstrated severe CME and subretinal fluid in the right eye (Figure 2). The left eye was unremarkable.
Color fundus photograph of the right eye at presentation. There are diffuse intraretinal hemorrhages in all quadrants, dilated retinal veins, and prominent macular edema.
Spectral-domain optical coherence tomography of the macula at presentation demonstrates marked cystoid macular edema and subretinal fluid in the right eye.
The patient underwent a hypercoagulable laboratory work-up, which included a complete blood count, complete metabolic profile, prothrombin time, partial thromboplastin time, lipid profile, lupus anticoagulant antibodies, factor V Leiden mutation analysis, protein C and S activity, and antithrombin III activity. All of the patient’s laboratory values were within normal ranges.
The patient was managed with monthly intravitreal bevacizumab injections over 6 months, which was initiated on the day of initial diagnosis. His CME gradually improved over this period (Figure 3), and his BCVA improved to 20/20 in the right eye, which was maintained at his 8-month follow-up examination.
Spectral-domain optical coherence tomography of the macula in the right eye at the 7-month follow-up visit. There is a marked improvement in the intraretinal fluid and resolution of subretinal fluid. The inner segment/outer segment junction appears to be intact.
CRVO occurs more frequently in older individuals and is associated with hyperlipidemia, hypertension, diabetes, and glaucoma.3 In younger patients, CRVO is associated with hematologic coagulopathies and prothrombotic states.5 None of these risk factors were identified in this patient. The onset of the patient’s CRVO coincided with prolonged vomiting and repeated Valsalva maneuvers due to acute gastroenteritis with dehydration. Previous reports have suggested that dehydration plays a role in the pathogenesis of CRVO, particularly in younger individuals with no other identifiable risk factors.2,7–10 The previously reported cases included dehydration secondary to intense exercise, fasting, and alcohol intake.2,7–12 To our knowledge, based on a PubMed search, this is the first reported case of a CRVO associated with prolonged vomiting with repeated Valsalva maneuvers due to gastroenteritis and secondary dehydration.
Further support for the association of dehydration and CRVO comes from a Saudi Arabian study that found significantly higher rates of CRVO during the month of Ramadan, in which observers fast from dawn until sunset.11 Labriola and colleagues postulated that increased blood viscosity secondary to dehydration could lead to abnormal erythrocyte aggregation and potentially cause venous thrombosis.8 In another study, an association was identified between increased blood concentration of urea and incidence of CRVO.12
Repeated episodes of emesis are associated with Valsalva retinopathy and may have also contributed to the pathogenesis of the patient’s CRVO. A study by Dickerman et al showed that a sudden elevation of intra-abdominal or intrathoracic pressure causes a corresponding rise in intracranial pressure, which can obstruct ocular venous outflow and lead to hemorrhages and elevated intraocular pressure.13 Similarly, Sbeity and Mansour reported a case of recurrent RVO secondary to repeated Valsalva from playing a high-resistance wind instrument.14 These studies suggest that in this case, the patient’s prolonged vomiting in conjunction with his dehydration may have played a role in the development of his CRVO.
Large, multicenter studies have established the role of intravitreal anti-vascular endothelial growth factor (VEGF) agents in the management of CRVO- associated CME.15–17 Although the patient was significantly younger and had a different etiology for his RVO than the population included in those studies, the CME responded to intravitreal anti-VEGF therapy. Of the 15 previously reported cases of dehydration- associated CRVO, only two patients who presented with a BCVA worse than 20/20 subsequently improved to 20/20.2,7–10 Both of those patients, along with our patient, received intravitreal anti-VEGF agents. These findings support the use of anti-VEGF agents in CME from dehydration-associated CRVO.
This case demonstrates that prolonged vomiting and Valsalva maneuvers associated with gastroenteritis and secondary dehydration are another potential risk factor for CRVO. CME caused by dehydration-associated CRVO is responsive to intravitreal anti-VEGF agents. In addition to being assessed for standard risk factors, young patients who present with CRVO should also be questioned about a history of vomiting and Valsalva maneuvers.
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- Jacobs DJ, Ahmad F, Pathengay A, Flynn HW Jr, . Central retinal vein occlusion after intense exercise: response to intravitreal bevacizumab. Ophthalmic Surg Lasers Imaging. 2011;42:e59–62.
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- Sbeity ZH, Mansour AM. Recurrent retinal vein occlusion after playing a wind instrument. Graefes Arch Clin Exp Ophthalmol. 2004;242(5):428–431. doi:10.1007/s00417-003-0848-9 [CrossRef]
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