Photo Essay Multiple Retinal Vein Occlusions Associated with Anti-thrombin III Deficiency Ramin Nourinia, MD; Talie Montahaei, MD; Bahareh Behdad, MD; Ali Aghajani, MD Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
J Ophthalmic Vis Res 2015; 10 (4): 493-494.
PRESENTATION A 23-year-old man was presented with slowly progressive bilateral decreasing vision over the past 3 years. He had no remarkable medical, surgical or family history of ocular or systemic disease. Best-corrected visual acuity (BCVA) was 20/200 in both eyes with -1-0.75×90 and -1.50-0.50×90 correction in the right and left eyes, respectively. Anterior segment examination revealed diffuse neovascularization of iris (NVI) in both eyes [Figure 1]. Diffuse capillary non-perfused areas associated with occluded retinal vessels in both eyes, and fibrovascular membrane with traction on the macula in the left eye were present in dilated fundus examination [Figures 2 and 3]. Moreover, on wide field fluorescein angiography (FAG), extensive capillary non-perfused areas, occluded vessels, and retinal artery narrowing were observed [Figures 4 and 5]. A complete systemic workup including detailed thrombophilia factors was requested among which a reduced level of circulating anti-thrombin III was significant. The patient was managed by pan-retinal laser photocoagulation (PRP) bilaterally in addition to intravitreal bevacizumab injection in the right eye.
DISCUSSION Anti-thrombin III is a potent inhibitor of reactions in the coagulation cascade. Its main duty is to virtually inhibit all coagulation enzymes to some extent. Congenital anti-thrombin III deficiency is an autosomal dominant disorder in which an individual inherits one copy of a defective gene. This condition leads to an increased
risk of venous and arterial thrombosis with onset of clinical manifestations typically appearing in young adulthood. [1,2] Acquired anti-thrombin III (AT III) deficiency has been reported in metabolic acidosis, hyper-lipoproteinemias, chronic liver diseases especially liver cirrhosis, active inflammatory bowel diseases, nephrotic syndrome, hemodialysis and disseminated intravascular coagulation.[3] Anti-thrombin deficiency is associated with a 5 to 50-fold increased risk for venous thrombosis.[4] Since the average age in patients affected by retinal vein occlusion (RVO) is 65, [5] the majority of RVO cases are mainly secondary to underlying cardiovascular diseases. [6] Although there is no Access this article online
Correspondence to:
Ramin Nourinia, MD. Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Boostan 9 St., Paidarfard St., Pasdaran Ave., Tehran 16666, Iran. E‑mail: [email protected] Received: 06-07-2014
Figure 1. Slit lamp photography shows diffuse iris neovascularization around the pupil.
Accepted: 14-03-2015
Journal of Ophthalmic and Vision Research 2015; Vol. 10, No. 4
Quick Response Code:
Website: www.jovr.org
DOI: 10.4103/2008-322X.176906
493
Photo Essay; Nourinia et al
Figures 2. Color fundus photograph of the right eye shows pale optic disc, severe arterial narrowing, occluded vessels, shunt vessels and mild fibrosis.
Figures 3. Fundus photograph of the left eye shows a fibrovascular membrane on the optic disc with macular traction, retinal arterial narrowing occluded vessels, shunt vessels.
Figures 4. Wide-field FAG of the right eye shows diffuse hypo fluorescence (capillary non-perfused area), occluded vessels and shunt vessels.
Figures 5. Wide-field FAG of the left eye shows significant hypo fluorescence due to occluded vessels, disc leakage, peripapillary shunt and neovascular capillary networks.
consensus, it seems rational to refer patients with central RVO for thrombophilia screening if they are younger than 50 years of age.[7] Herein, we reported a patient with severe retinal ischemia secondary to multiple RVO with anti-thrombin III deficiency to emphasize the importance of detection of thrombophilia in cases of vein occlusion particularly in young subjects. Ophthalmologists play a key role in approaching such cases by uncovering systemic associations and preventing vision and life-threatening conditions.
Lechner K, et al. Homozygous antithrombin deficiency type II (99 Leu to Phe mutation) and childhood thromboembolism. Thromb Haemost 2001;86:1007‑1011. Müller G. Acquired antithrombin III deficiency. Z Gesamte Inn Med 1992;47:74‑77. Khor B, Van Cott EM. Laboratory tests for antithrombin deficiency. Am J Hematol 2010;85:947‑950. Hattenbach LO, Klais C, Scharrer I. Heparin cofactor II deficiency in central retinal vein occlusion. Acta Ophthalmol Scand 1998;76:758‑759. Kuhli C, Hattenbach LO, Scharrer I, Koch F, Ohrloff C. High prevalence of resistance to APC in young patients with retinal vein occlusion. Graefes Arch Clin Exp Ophthalmol 2002;240:163‑168. Pierre‑Filho Pde T, Pierre AM, Nascimento MA, Marcondes AM. Central retinal vein prethrombosis as an initial manifestation of protein S deficiency. Sao Paulo Med J 2004;122:134‑135.
REFFERENCES 1.
2.
494
Beauchamp NJ, Pike RN, Daly M, Butler L, Makris M, Dafforn TR, et al. Antithrombins Wibble and Wobble (T85M/K): Archetypal conformational diseases with in vivo latent‑transition, thrombosis, and heparin activation. Blood 1998;92:2696‑2706. Kuhle S, Lane DA, Jochmanns K, Male C, Quehenberger P,
3. 4. 5. 6.
7.
How to cite this article: Nourinia R, Montahaei T, Behdad B, Aghajani A. Multiple retinal vein occlusions associated with anti-thrombin III deficiency. J Ophthalmic Vis Res 2015;10:493-4. Source of Support: Nil. Conflict of Interest: None declared.
Journal of Ophthalmic and Vision Research 2015; Vol. 10, No. 4
J Ophthalmic Vis Res 2015; 10 (4): 493-494.
PRESENTATION A 23-year-old man was presented with slowly progressive bilateral decreasing vision over the past 3 years. He had no remarkable medical, surgical or family history of ocular or systemic disease. Best-corrected visual acuity (BCVA) was 20/200 in both eyes with -1-0.75×90 and -1.50-0.50×90 correction in the right and left eyes, respectively. Anterior segment examination revealed diffuse neovascularization of iris (NVI) in both eyes [Figure 1]. Diffuse capillary non-perfused areas associated with occluded retinal vessels in both eyes, and fibrovascular membrane with traction on the macula in the left eye were present in dilated fundus examination [Figures 2 and 3]. Moreover, on wide field fluorescein angiography (FAG), extensive capillary non-perfused areas, occluded vessels, and retinal artery narrowing were observed [Figures 4 and 5]. A complete systemic workup including detailed thrombophilia factors was requested among which a reduced level of circulating anti-thrombin III was significant. The patient was managed by pan-retinal laser photocoagulation (PRP) bilaterally in addition to intravitreal bevacizumab injection in the right eye.
DISCUSSION Anti-thrombin III is a potent inhibitor of reactions in the coagulation cascade. Its main duty is to virtually inhibit all coagulation enzymes to some extent. Congenital anti-thrombin III deficiency is an autosomal dominant disorder in which an individual inherits one copy of a defective gene. This condition leads to an increased
risk of venous and arterial thrombosis with onset of clinical manifestations typically appearing in young adulthood. [1,2] Acquired anti-thrombin III (AT III) deficiency has been reported in metabolic acidosis, hyper-lipoproteinemias, chronic liver diseases especially liver cirrhosis, active inflammatory bowel diseases, nephrotic syndrome, hemodialysis and disseminated intravascular coagulation.[3] Anti-thrombin deficiency is associated with a 5 to 50-fold increased risk for venous thrombosis.[4] Since the average age in patients affected by retinal vein occlusion (RVO) is 65, [5] the majority of RVO cases are mainly secondary to underlying cardiovascular diseases. [6] Although there is no Access this article online
Correspondence to:
Ramin Nourinia, MD. Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Boostan 9 St., Paidarfard St., Pasdaran Ave., Tehran 16666, Iran. E‑mail: [email protected] Received: 06-07-2014
Figure 1. Slit lamp photography shows diffuse iris neovascularization around the pupil.
Accepted: 14-03-2015
Journal of Ophthalmic and Vision Research 2015; Vol. 10, No. 4
Quick Response Code:
Website: www.jovr.org
DOI: 10.4103/2008-322X.176906
493
Photo Essay; Nourinia et al
Figures 2. Color fundus photograph of the right eye shows pale optic disc, severe arterial narrowing, occluded vessels, shunt vessels and mild fibrosis.
Figures 3. Fundus photograph of the left eye shows a fibrovascular membrane on the optic disc with macular traction, retinal arterial narrowing occluded vessels, shunt vessels.
Figures 4. Wide-field FAG of the right eye shows diffuse hypo fluorescence (capillary non-perfused area), occluded vessels and shunt vessels.
Figures 5. Wide-field FAG of the left eye shows significant hypo fluorescence due to occluded vessels, disc leakage, peripapillary shunt and neovascular capillary networks.
consensus, it seems rational to refer patients with central RVO for thrombophilia screening if they are younger than 50 years of age.[7] Herein, we reported a patient with severe retinal ischemia secondary to multiple RVO with anti-thrombin III deficiency to emphasize the importance of detection of thrombophilia in cases of vein occlusion particularly in young subjects. Ophthalmologists play a key role in approaching such cases by uncovering systemic associations and preventing vision and life-threatening conditions.
Lechner K, et al. Homozygous antithrombin deficiency type II (99 Leu to Phe mutation) and childhood thromboembolism. Thromb Haemost 2001;86:1007‑1011. Müller G. Acquired antithrombin III deficiency. Z Gesamte Inn Med 1992;47:74‑77. Khor B, Van Cott EM. Laboratory tests for antithrombin deficiency. Am J Hematol 2010;85:947‑950. Hattenbach LO, Klais C, Scharrer I. Heparin cofactor II deficiency in central retinal vein occlusion. Acta Ophthalmol Scand 1998;76:758‑759. Kuhli C, Hattenbach LO, Scharrer I, Koch F, Ohrloff C. High prevalence of resistance to APC in young patients with retinal vein occlusion. Graefes Arch Clin Exp Ophthalmol 2002;240:163‑168. Pierre‑Filho Pde T, Pierre AM, Nascimento MA, Marcondes AM. Central retinal vein prethrombosis as an initial manifestation of protein S deficiency. Sao Paulo Med J 2004;122:134‑135.
REFFERENCES 1.
2.
494
Beauchamp NJ, Pike RN, Daly M, Butler L, Makris M, Dafforn TR, et al. Antithrombins Wibble and Wobble (T85M/K): Archetypal conformational diseases with in vivo latent‑transition, thrombosis, and heparin activation. Blood 1998;92:2696‑2706. Kuhle S, Lane DA, Jochmanns K, Male C, Quehenberger P,
3. 4. 5. 6.
7.
How to cite this article: Nourinia R, Montahaei T, Behdad B, Aghajani A. Multiple retinal vein occlusions associated with anti-thrombin III deficiency. J Ophthalmic Vis Res 2015;10:493-4. Source of Support: Nil. Conflict of Interest: None declared.
Journal of Ophthalmic and Vision Research 2015; Vol. 10, No. 4
