Perioperative nonarteritic anterior ischemic optic neuropathy in Jehovah s Witnesses
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1 Perioperative nonarteritic anterior ischemic optic neuropathy in Jehovah s Witnesses CASE 1 A 62-year-old, female, African American, Jehovah s Witness experienced bilateral and simultaneous vision loss 2 days after hip arthroplasty. Her 5-hour supine hip replacement surgery resulted in 1 L of blood loss; given her religious status, she refused replacement blood products. Her hemoglobin was 6.3 g/dl preoperatively and 4.7 g/dl postoperatively. The etiology of her preoperative anemia was not known. Her lowest postsurgical blood pressure was 75/ 45 mm Hg. Her hypotensive medications were discontinued, she was kept in Trendelenburg position, and was treated with intravenous (IV) iron and darbepoetin alfa. Her medical history was significant for hypertension treated with bisoprolol, hydrochlorothiazide, and valsartan. Her vision was normal postoperatively. After 2 days, she was assisted out of bed and into a chair for the first time and noticed light-headedness with a slow, bilaterally progressive visual blurring over the next day. This transitioned into a dark curtain coming down over her vision, with her right eye affected more than her left. She could see only dark/blurred shapes and figures. On postoperative day 2, her vision was no light perception (NLP) OD and counting fingers (CF) at 6 OS. Fig Humphrey visual field (V stimulus) OS showed diffuse vision loss (case 1). e54 CAN J OPHTHALMOL VOL. 52, NO. 2, APRIL 2017
2 Fig. 2 (A) OD and (B) OS fundus photographs at 2-month follow-up significant for bilateral optic nerve pallor (case 1). Confrontation visual field testing showed preservation of the inferotemporal quadrant OS only, and Humphrey visual field testing (V stimulus) showed diffuse loss and superior/ inferior nerve fibre defects with central depression OS (Fig. 1). Her pupils were mildly anisocoric (6 and 5.75 mm) and poorly reactive to light; the right pupil was amaurotic with a right relative afferent pupillary defect (RAPD). Fundoscopy showed bilateral optic disc edema and few scattered flame/intraretinal blot hemorrhages. Erythrocyte sedimentation rate (ESR) was 144 mm/hr, but she denied headache, scalp tenderness, and jaw claudication. Cranial magnetic resonance imaging (MRI) was negative for any evidence of intracranial mass lesions and magnetic resonance angiography showed no evidence of vascular occlusive disease. Her bilateral vision loss and optic neuropathy were thought to be either nonarteritic/postsurgical or arteritic ischemic optic neuropathy (ION). Her antihypertensives were held and she was started on IV methylprednisolone 1 g and fludrocortisone. She received IV albumin and was administered 100% oxygen by mask. Temporal artery biopsy performed after 14 days (allowing her hemoglobin to rise above 8.0 g/dl) was negative. She received 3 doses of IV methylprednisolone 1 g and was maintained on oral prednisone 1 mg/kg/day throughout this period. Despite normal IOP, topical brimonidine OU was given to provide theoretical neuroprotective effects and to lower IOP, thereby increasing ocular perfusion pressure. A follow-up ESR normalized to 7 mm/hr; prednisone was tapered and discontinued. The optic nerves developed bilateral optic atrophy (Fig. 2) and vision remained NLP OD and CF OS. CASE 2 A 47-year-old, African American Jehovah s Witness witha history of hypertension underwent her fifth recurrent skull base meningioma resection and then presented with worsening bilateral/simultaneous vision loss. She originally presented with similar symptoms 3 years previously and was found to have bilateral optic atrophy. MRI showed meningioma centred on the cribriform plate and planum sphenoidale. All resections demonstrated World Health Organization grade I meningioma. The patient underwent intra- and extracranial debulking for intracranial/intranasal/intrasinus involvement, her fourth debulking. Her vision first improved and then deteriorated, leading to additional debulking, which lasted 10 hours and resulted in 1.2 L of blood loss. Hemoglobin decreased from 10.2 g/dl preoperatively to 8.2 g/dl postoperatively. She was treated with EPO and IV iron but refused blood products. Her medical history included hypertension and osteoarthritis. Her medications were lisinopril, venlafaxine, diphenhydramine, butalbital, and levetiracetam. The patient s vision was 20/40 OD and CF at 6 OS; preoperative values were 20/20 and 20/50. Her pupils measured 4 mm in dark and 2 mm in light with a left RAPD. Her motility was full. Her IOP was 29 OD and 19 OS; she was started on brimonidine OU. Her optic nerves demonstrated a cup-to-disc ratio of 0.6 OU, peripapillary atrophy, and global thickness of 43 μm OD and 54 μm OS on OCT (Fig. 3). Humphrey visual fields (Fig. 4) showed diffuse depression OU. Ultrasonography was performed, which showed no change on 30-degree testing. Her vision loss and optic neuropathy were multifactorial but included a presumed component of ischemia, likely precipitated by ocular hypertension coupled with perioperative anemia. Her vision remains stable but decreased, with significant optic atrophy bilaterally. DISCUSSION Perioperative ION can occur most frequently after spine and cardiac surgery; 1 the prevalence after hip surgery is estimated at only 0.004%. 2 Although multiple risk factors have been proposed, none have been proven, CAN J OPHTHALMOL VOL. 52, NO. 2, APRIL 2017 e55
3 Fig. 3 Optical coherence tomography demonstrating postsurgical bilateral optic atrophy (case 2). including patient-related factors (e.g., hypertension, anemia, diabetes, obesity, male sex, smoking, hyperlipidemia) and intra- and postoperative factors (e.g., blood loss, hypotension, prone positioning, type and duration of surgery, Wilson frame use in spine surgery, vasopressors, crystalloid vs colloid). Jehovah s Witnesses, a restorationist Christian denomination who report a membership of 8 million, 3 have religious convictions against receiving blood s primary components because of their interpretation of several Biblical passages. Physicians preparing to treat a Jehovah s Witness should familiarize them- e56 CAN J OPHTHALMOL VOL. 52, NO. 2, APRIL 2017
4 Fig Humphrey visual field (A) OD and (B) OS preceding meningioma debulking, and (C) OD and (D) OS after debulking (case 2). selves with substitutes discussed in Table 1 and the following. Preoperative preparation should focus on informed consent (the risks and consequences of transfusion denial) and hemodynamic optimization. Medications, foods, and supplements that increase bleeding may be considered for discontinuation. 4 Iron, folate, B12, and erythropoietin supplementation may increase hemoglobin. 4 Preoperative hemodilution involves infusion of colloid/crystalloid with (normovolemic) or without (hypervolemic) blood removal. Hemodilution decreases red blood cell mass loss relative to blood volume loss, but studies have not uniformly demonstrated benefit. 5 Medications that potentially precipitate ION (e.g., amiodarone, antihypertensives, analgesics, sedatives, phosphodiesterase inhibitors, 6 interferon alpha 7 ) may be reduced or discontinued. Risk factors for ION should be optimized before surgery (e.g., sleep apnea, 8,9 obesity, smoking, hypertension, hyperlipidemia, diabetes 9 ). Intraoperatively, meticulous hemostasis and surgical technique are imperative. Hemostasis can occur locally (i.e., synthetic sealants) or systemically (i.e., aminocaproic acid/ factor VIIa). 4 Volume resuscitation can occur via crystalloid or colloid. Discussion about the use of albumin or autologous blood kept in continuity with the patient may be options. Although cell saver autotransfusion is unacceptable because of batch processing, the tubing used in dialysis, cell salvage, and cardiopulmonary bypass may be considered continuous with the intravascular compartment. 5 Hemoglobin-based oxygen carriers and perfluorocarbons are newer transfusion alternatives in development. CAN J OPHTHALMOL VOL. 52, NO. 2, APRIL 2017 e57
5 Table 1 Medical treatments acceptable/unacceptable to Jehovah s Witnesses Generally Not Acceptable Autologous whole blood Allogeneic whole blood Red blood cells White blood cells Platelets Plasma Cell saver Generally Acceptable Crystalloids Colloids Recombinant erythropoietin Recombinant factor VIIa Hemodilution Cell salvage Dialysis Cardiopulmonary bypass Iron, vitamin B12, folate Supplemental oxygen Hemoglobin-based oxygen carriers Perfluorocarbons Postoperative countermeasures include supplemental oxygen, minimization of routine blood draws, vitamin and mineral supplementation, and erythropoietin. 9 In addition to blood pressure optimization, IOP reduction and recumbent positioning could be considered. We believe that increased sensitivity to and clinical awareness of the special needs of Jehovah s Witness patients may reduce the risk of perioperative ION in the setting of nonocular surgery in this unique population. Disclosure: The authors have no proprietary or commercial interest in any materials discussed in this article. Stacy Smith and Andrew Lee provided original text, editing assistance, and review of manuscript. Alec L. Amram, MD, * StacyV.Smith,MD, Andrew G. Lee, MD *,,,, * Department of Ophthalmology, University of Texas Medical Branch (UTMB), Galveston, Texas; Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery, Weill Cornell Medicine, New York, New York; Section of Ophthalmology, University of Texas MD Anderson Cancer Center, Houston, Texas; Department of Ophthalmology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa. Correspondence to: Alec Lee Amram, MD: alec.amram.md@gmail.com REFERENCES 1. Holy SE, Tsai JH, McAllister RK, Smith KH. Perioperative ischemic optic neuropathy: a case control analysis of 126,666 surgical procedures at a single institution. Anesthesiology. 2009;110: Shen Y, Drum M, Roth S. The prevalence of perioperative visual loss in the United States: a 10-year study from 1996 to 2005 of spinal, orthopedic, cardiac, and general surgery. Anesth Analg. 2009;109: Jehovah s Witnesses 2015 Grand Totals. Available at jw.org/en/publications/books/2016-yearbook/2015-grand-totals/ [accessed March 23, 2016]. 4. Tanaka A, Ota T, Uriel N, et al. Cardiovascular surgery in Jehovah s Witness patients: the role of preoperative optimization. J Thorac Cardiovasc Surg. 2015;150: e3. 5. Hughes DB, Ullery BW, Barie PS. The contemporary approach to the care of Jehovah s Witnesses. J Trauma. 2008;65: Pomeranz HD, Smith KH, Hart WM, Egan RA. Sildenafil-associated nonarteritic anterior ischemic optic neuropathy. Ophthalmology. 2002;109: Berg KT, Nelson B, Harrison AR, Mcloon LK, Lee MS. Pegylated interferon alpha-associated optic neuropathy. J Neuroophthalmol. 2010;30: Aptel F, Khayi H, Pépin JL, et al. Association of nonarteritic ischemic optic neuropathy with obstructive sleep apnea syndrome: consequences for obstructive sleep apnea screening and treatment. JAMA Ophthalmol. 2015;133: Bilgin G, Koban Y, Arnold AC. Nonarteritic anterior ischemic optic neuropathy and obstructive sleep apnea. J Neuroophthalmol. 2013;33: Can J Ophthalmol 2017;52:e54 e /17/$-see front matter & 2017 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. Doxycycline injection in orbital cyst associated to microphthalmos The use of percutaneous sclerotherapy (e.g., bleomycin, tetradecyl sulfate, ethanol) has been described for the treatment of cystic lymphatic malformations of the orbit. 1 4 We present an interesting case in which doxycycline was used for the treatment of a congenital orbital cyst in a child with bilateral microphthalmos. CASE REPORT The patient was a 4-year-old Caucasian male who was born by Cesarean section and weighed 4.46 kg at birth. Examinations by 2 pediatric ophthalmologists 4 days and 2 months after birth noted bilateral microphthalmia, microcornea, iris coloboma, iridocorneal angle dysgenesis, esotropia, and significant proptosis OS. At 2 months of age, an examination under anaesthesia revealed that the intraocular pressure was 28 OD and 12 OS and the pachymetry was 747 mm OD and 780 mm OS, but the axial length was unmeasurable. The posterior pole was remarkable for bilateral persistent fetal vasculature and a right optic nerve coloboma. Retcam pictures of both eyes were taken but refraction could not be measured. At 3 months of age, a visual evoked potential yielded light perception vision OD but was inconclusive OS. At 4 days of life, ultrasonography and orbital computed tomography (CT) scan showed a left orbital unilocular cyst measuring 27 mm 21 mm 26 mm. There was no evidence of tumour or lymphatic malformation. At 1 month of age, magnetic resonance imaging (MRI) confirmed these findings and showed that the left orbital cyst had not changed in size (28 mm 21 mm 26 mm) e58 CAN J OPHTHALMOL VOL. 52, NO. 2, APRIL 2017
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