Purtscherʼs Retinopathy as a Cause of Postoperative Blindness

Abstract

Sir: The most frightening sequela of managing periorbital trauma is acute postoperative blindness. As such, surgeons should be aware of the causes, diagnosis, and management options for patients who develop visual loss following surgery. A 16-year-old boy with no medical history or prior visual problems sustained trauma to the head and chest in a motor vehicle accident. The orbitozygomatic trauma required open reduction and internal fixation, including the use of cranial bone grafts to the orbital floor. Postoperatively, he was followed with head elevation, iced compresses, and close observation. Light perception was recorded serially. Gentle separation of the eyelids confirmed intact vision. He initially did well and was discharged to home on postoperative day 2. At home on the fourth postoperative day, he noted a rapid decline in vision in the left eye. No tenderness, bleeding, or swelling was noted. A computed tomographic scan failed to demonstrate involvement of the nerve either by incomplete reduction or external compression. A retinal specialist determined the cause to be Purtscher's syndrome based on the history and funduscopic findings. A course of high-dose steroids was prescribed. Purtscher's syndrome is a retinopathy of vascular origin first described in the early 1900s.1,2 The hallmark is a history of severe head or chest trauma, but a similar appearance has been described with acute pancreatitis,3 fat embolism, renal failure, childbirth, and connective tissue disorders. The pathogenesis was originally thought to be extravasation of lymph from retinal vessels following a sudden increase in pressure. Since then, several causes have been suggested, including raised intrathoracic pressure, vasculitis resulting from free fatty acids, and vascular occlusion caused by emboli. Purtscher's retinopathy is characterized by multiple white patches, hemorrhages, and optic disc edema on funduscopy (Fig. 1). The white lesions (cotton-wool spots) represent retinal microinfarcts within the layer of nerve fibers. The findings may be unilateral or bilateral, and the effect on vision may be delayed for up to 48 hours after onset of the condition. Late physical findings include pigment migration and optic atrophy. The diagnosis is based on both the appearance of the fundus and the history of associated systemic injury (Fig. 2).Fig. 1.: Preoperative axial computed tomographic scan demonstrating fracture of the left zygomaticomalar complex.Fig. 2.: Funduscopic appearance of Purtscher's syndrome. (From the University of Iowa Department of Ophthalmology and Visual Sciences.)On follow-up examination at 3 months, the patient was noted to have 20/20 vision in the right eye and 20/200 in the left. A significant afferent pupillary defect was noted on the affected side. Dilated ophthalmoscopy revealed a return to a relatively normal retinal appearance despite some optic nerve pallor. At 1-year follow-up, he was noted to have a central acuity of 20/25 with persistent peripheral visual field damage. The significant recovery of partial vision over the first 6 months is consistent with the course of Purtscher's syndrome. No specific treatment for Purtscher's syndrome has been delineated. However, the use of early, parenteral, high-dose steroids and/or fenestration of the optic nerve sheath may be beneficial.4 An initial loss of vision in the range of 20/200 to hand movement visual acuity can improve over a period of months to a range of 20/30 to 20/200. It is important for plastic surgeons who manage facial trauma to be aware of this condition because early, high-dose, steroid therapy may be beneficial. Justin Lipper, B.A. Joseph Pazkowski, M.D. Peter J. Taub, M.D. Mount Sinai Medical Center New York, N.Y.