American Academy of Optometry Irreversible Vision Loss during Pregnancy: Clinical Picture of Diabetic Macular Ischemia

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1 American Academy of Optometry Irreversible Vision Loss during Pregnancy: Clinical Picture of Diabetic Macular Ischemia First Author: Caryn Jones Burns Street Apt C 3 Forest Hills, NY carynsmail1@yahoo.com Second Author: Jaqueline Armani jarmani@gmail.com Third Author: Gulshan Karamchandani gkaramchandani@sunyopt.edu

2 Abstract: During pregnancy, the progression of diabetic retinopathy is greatly accelerated. Macular ischemia coupled with florid neovascularization creates the potential for devastating vision loss. A unique and careful approach must be taken in managing these patients. Case History: Patient Demographics: 21 year old white female with history of type 1 diabetes for approximately ten years, presents to the Brooklyn VAMC eye clinic for an initial consult. Patient is currently 6.5 months pregnant. Chief complaint: Blurry vision OD for 1.5 years. Ocular history: Last seen by private ophthalmologist 1 month ago (July 2010) who diagnosed severe diabetic retinopathy. The patient s previous ophthalmologist recommended urgent laser by a retinal specialist. The patient did not follow up with additional care until August 2010, more than 1 month later. Medical history: Type 1 diabetes, anemia, 6.5 months pregnant. Patient reports that blood sugar has been fluctuating during the course of her pregnancy and last blood sugar reading that she can recall was greater than 200. Last Hemoglobin A1C is unknown. Medications: Insulin, Nitrofurantoin mono/macro 100mg PO for infection Pertinent Findings: Blood pressure: 120/78 Heart Rate: 87 BPM Uncorrected Visual acuity: OD: Count Fingers at 5 feet with eccentric viewing, no improvement with pinhole OS: 20/20-2 Confrontation visual fields: FTFC OU Extra-ocular Motility: full and smooth OU; (-)diplopia, (-)pain Pupils: ERRL APD

3 Slit lamp examination: Lids: clear OU Conjunctivae: clear OU Corneas: clear OU Anterior Chamber: deep and quiet OU Iris: flat, clear OU, (-)NVI OU Lenses: clear OU Goldmann tonometry: OD: 15 mm/hg OS: 16 mm/hg at 2:00pm Patient refused dilation at initial visit. Fundus photos were taken at initial visit. Non-dilated fundus examination: Vitreous: clear OU Optic disc:.3/.3 OD;.35/.35 OS Macula: dot/blot hemes with retinal whitening in all 4 quadrants, possible thickening over fovea with surrounding whitening OD; dot/blot hemes throughout superior and inferior arcades with whitening surrounding fovea, irregular blood vessel appearance, possible neovascularization elsewhere OU; neovascularization of the disc OS Vessels: irregular tortuous appearance; NVD, NVE OS Periphery: Not viewed OU Patient will return in 1 week for dilated exam with retinal specialist. Dilated examination findings: Macular ischemia OD>OS; neovascularization elsewhere OU. Differential diagnosis Primary diagnosis: Proliferative diabetic retinopathy with diabetic macular ischemia Other conditions included in the differential: Eales Disease, a diagnosis of exclusion (2) Sickle Cell Retinopathy Central/branch retinal artery occlusion (1) Central/Branch retinal vein occlusion (1) Neovascularization following crystalline or talc retinopathy (1) Ocular ischemic syndrome (1) Radiation Retinopathy (1)

4 Sarcoidosis (1) Systemic Lupus Erythematosus (3) Duchenne's muscular dystrophy (3) Linear scleroderma "en coup de sabre" (3) Parry-Romberg (3) Chronic Myelogenous Leukemia (3) Diagnosis and Discussion With the patient s systemic history of uncontrolled type 1 diabetes, and evidence from the dilated retinal examination, it was concluded that the patient has diabetic macular ischemia and proliferative diabetic retinopathy in both eyes. Diabetic macular ischemia accounts for the reduction in vision of the right eye. The diagnosis of macular ischemia was made through clinical observation during the dilated fundus exam. Fluorescein angiography was not used to determine the extent of diabetic macular ischemia in attempt to limit any potential harm to the patient s child during pregnancy. The decision to avoid fluorescein angiography may be controversial to some doctors as fluorescein angiography has safely been completed in numerous pregnant individuals over time. However, according to a compilation of data from 424 retina specialists, there have been several reports of birth complications following fluorescein angiography, but it has not shown to cause a high rate of complications during pregnancy (4). In attempt to minimize any potential risk to the patient and the fetus, we elected not to utilize fluorescein angiography for this case. The cycle of edema and ischemia add to the level of pathology present. This is because edema and exudation decreases the ease of diffusion of oxygen and nutrients between the inner retina and the small capillaries of the perifoveal zone. While edema was not evident during the patient s initial presentation, it is possible that it was present prior to the onset of macular ischemia. A compounding systemic condition present in this particular patient is anemia. Anemia is known to increase the level of ischemia, as it decreases the amount of oxygen supplied to tissues (8). Macular ischemia progresses over time, according to the 5 year ETDRS, and clinically there has been little to no evidence of regression (5). The prognosis for improvement of visual acuity in the patient s right eye is therefore quite poor. Neovascularization of the retina and disc increase the potential for reduced vision as a vitreous hemorrhage or tractional retinal detachments are possible complications. Unfortunately, pregnant females are at an increased risk for a retinal or vitreous hemorrhage during the process of delivery. Laser treatment is therefore highly indicated in pregnant individuals with proliferative diabetic retinopathy, clinically significant macular edema, and under certain circumstances, non-proliferative diabetic retinopathy (6). Immediate treatment with pan retinal photocoagulation is imperative to this treatment plan to reduce the risk of further vision reduction. Several factors were found to increase the difficulty of diagnosing and effectively treating this patient. The patient had symptoms of reduced vision for more than a year before seeking medical attention. Another month elapsed after receiving the diagnosis of severe diabetic retinopathy, with instructions to have urgent laser treatment, before she was seen at the Brooklyn VAMC. The time between the onset of symptoms and her initial consult may have severely limited the prognosis, and thusly for visual improvement. Additionally, the patient is twenty six weeks pregnant, and extreme caution must be taken when beginning any treatment regime.

5 Lastly, fluorescein angiography was not performed as it poses a potential risk to the patient s child. All of the conditions unique to this patient made a meticulous and conservative treatment plan a necessity to attempt to maintain the level of vision the patient currently has. Treatment and Management The patient has a very poor prognosis for regaining vision in her right eye as the reduced vision is due to macular ischemia. Treatment aimed to prophylactically reduce the risk of vitreous or retinal hemorrhages, or a retinal detachment. Since the patient is over six months pregnant, laser therapy was indicated as the immediate treatment, as the threat of vitreous or retinal hemorrhage is greatly enhanced during delivery. Progression of diabetic retinopathy also occurs twice as commonly during pregnancy (6). After consulting with a retinal specialist, the patient received pan retinal photocoagulation OU. The patient has had multiple short treatments with pan retinal photocoagulation since diagnosis to date. The potential for reduced vision in the left eye remains high as the patient has florid neovascularization and macular ischemia in the left eye as well. Results of laser are pending: patient s next appointment is on 9/10/10. Conclusion All diabetics should have a dilated eye exam at least once per year. Optimally, patients should control their glycemic levels prior to conception. This should be done, ideally, at least six months before the pregnancy. Patients should work with their physicians to slowly lower their blood sugar levels over a period of at least six months prior to conception when diagnosed with severe non-proliferative diabetic retinopathy or proliferative diabetic retinopathy. A dilated exam should be completed within the first trimester and third of the pregnancy, and follow up care should continue throughout the pregnancy and postpartum. With moderate retinopathy, follow up care needs to occur more frequently and patients should return on a monthly basis to ensure resolution of the retinopathy. Any neovascularization or clinically significant macular edema present should be treated with laser photocoagulation immediately to reduce the risk of decreased vision. Vaginal delivery has shown to increase the risk of retinal or vitreous hemorrhages and therefore it may be necessary for the retinal specialist to consult with the patient s obstetrician regarding methods of delivery available to reduce these risks, such as a cesarean section (6). Clinical Pearls: The risks and benefits of using 1% tropicamide and 2.5% phenylephrine on a pregnant individual must be discussed with patients before dilation.

6 It is imperative that diabetic patients receive thorough eye examinations to detect retinopathy and vision threatening conditions as quickly as possible because the risk of progression of diabetic retinopathy doubles during pregnancy (6). It is therefore highly recommended that all diabetic individuals have a dilated exam during their pregnancy (7). Laser treatment is indicated for all pregnant individuals with neovascularization. An immediate referral for PRP is necessary in these cases (6). Prompt referral for treatment of diabetic retinopathy is imperative to reduce the risk of irreversible vision loss, such as with macular ischemia. Care must be taken in patients who develop macular edema to reduce the chance of developing macular ischemia (5). The patient s history is a key part of diagnosing diabetic macular ischemia and proliferative diabetic retinopathy. Masqueraders of diabetic retinopathy must still be considered in the differential as many life threatening conditions can present in a similar fashion to diabetic retinopathy (3).

7 Bibliography (1) Ehlers JP. Shah CP, eds. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 5 th Ed. New York: Wolters Kluwer Lippincott Williams & Wilkins; 2008: (2) Roth, Daniel B., Eales Disease. EMedicine. February Available at: Accessed August 20, (3) Barbazetto IA. Diabetic retinopathy: the masqueraders. Retinal Physician [serial online]. July Available at: Accessed August 21, (4) Halperin LS, Olk RJ, Soubrane G, Coscas G. Safety of fluorescein angiography during pregnancy. Am J Ophthalmol. May ;109 (5): (5) Paulter SE. Diabetic Ischemia. In: Browning DJ, ed. Diabetic Retinopathy: Evidence- Based Management. New York: Springer; 2010: (6) Kitzmiller JL, Block JM, Brown FM, et. al. Managing Preexisting Diabetes for Pregnancy: Summary of evidence and consensus recommendations for care. Diabetes Care. 2008;31: (7) Portello JK, Krumholz DM. Dilation of the Pupil. In: Bartlett JD, Jaanus SD. Clinical Ocular Pharmacology. 5 th Ed. Burlington, MA: Butterworth-Heinemann; 2001: 335. (8) Mehdizadeh M. Macular edema-ischemia makes a vicious cycle in diabetic retinopathy: Role of retinal hypoxia in diabetic macular edema. Graefe's Arch for Clin and Exp Ophthalmol. August 2008; 246 (8): 1203.

The Human Eye. Cornea Iris. Pupil. Lens. Retina

The Human Eye. Cornea Iris. Pupil. Lens. Retina The Retina Thin layer of light-sensitive tissue at the back of the eye (the film of the camera). Light rays are focused on the retina then transmitted to the brain. The macula is the very small area in