Chapter 32
Retinopathy and Distant Extraocular Trauma
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The retinal manifestations of distant trauma may be asymmetric and vary among patients depending on the type of trauma. Discrepancies in retinal findings relate to an incompletely understood pathogenesis of the retina's response to distant trauma, as well as the type of trauma sustained. For example, retinal changes resulting from long bone fractures are manifest differently than the retinal changes resulting from whiplash injuries.

The pathophysiologic mechanisms of retinal damage after distant trauma have been debated. Three mechanisms have been proposed to explain the resulting fundus findings: (1) increased intraluminal pressure may damage the retinal vascular endothelial cells; (2) emboli from sources including air, blood products, or fat may also damage the retina, a theory that has been supported in experimental models; and (3) mechanical forces acting at the vitreoretinal interface may damage the retina.

In this chapter we describe six clinical entities: Purtscher's retinopathy, traumatic asphyxia, fat embolism retinopathy, Valsalva retinopathy, whiplash maculopathy, and shaken baby syndrome. With the exception of whiplash maculopathy, the five retinopathies have some overlap in either clinical presentation or pathophysiology and the categorization of the retinopathies relates more to the type of trauma than to a unique retinal appearance.

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Purtscher's retinopathy is characterized by retinal hemorrhages, exudates, and decreased vision associated with nonocular trauma (Fig. 1). In 1912, Otmar Purtscher described multiple, white retinal patches and retinal hemorrhages surrounding a normal-appearing optic disc in five patients with visual loss after severe head trauma.1 Most commonly, Purtscher's retinopathy develops as a sequela of chest-compressing trauma. The severity of the traumatic event is variable, ranging from minimal external trauma to crushing chest wall injuries. The onset of symptoms usually occurs within 2 days after trauma. Both eyes are typically involved, but unilateral cases have been reported.2,3 Patients complain of decreased vision, often from 20/200 (6/60) to counting fingers. Fundus examination usually reveals numerous white retinal patches or confluent cotton-wool spots around the disc, as well as superficial retinal hemorrhages. Other findings include serous macular detachments, dilated and tortuous vasculature, and disc edema. The peripheral retina is commonly spared. Fluorescein angiography can reveal focal areas of arteriolar obstruction, patchy capillary nonperfusion, disc edema, and dye leakage from retinal arterioles, capillaries, and venules.3 Purtscher1 originally proposed that the etiology of the white retinal lesions resulted from lymph extravasated from retinal vessels during a sudden increase in intracranial pressure. In 1962, Marr and Marr4 wrote that the retinopathy resulted from reflux venous shock waves produced from intrathoracic chest compression. Several authors have implicated arteriolar emboli including air and fat as the cause.3,5,6 Other authors have suggested an etiologic role for granulocyte or other blood product emboli formed after complement activation, arguing that microembolization is a mechanism common to the varied clinical settings of Purtscher's retinopathy.7,8 Interestingly, a Purtscher's-like fundus picture may occur in several nontraumatic settings, such as acute pancreatitis, chronic renal failure, thrombotic thrombocytopenic purpura lupus erythematosus, and childbirth.7,9–12 Clinically, the retinal lesions resolve over a period of weeks to a few months.4 After resolution, the fundus may appear normal, but pigment migration and optic atrophy can occur.13Although visual acuity can remain reduced, the acuity may return to normal or near normal.4

Fig. 1 Purtscher's retinopathy. Note cloudlike, white exudates; superficial hemorrhages; and dilated veins. (Wills Eye Hospital clinical archive)

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Traumatic asphyxia usually results from severe compression of the thorax and is characterized by a striking, ecchymotic mask, or blue discoloration of the upper chest and the face. External ocular involvement is virtually universal in cases of traumatic asphyxia. Patients have ecchymotic eyelids and hemorrhagic conjunctiva but retinal changes occur less commonly. Trampling, suicide attempts by hanging, seizures, vomiting, and childbirth have caused traumatic asphyxia.14 Visual acuity can be unaffected by traumatic asphyxia but may be reduced to no light perception.4 Fundus examination may reveal intraretinal hemorrhages, as well as cotton-wool spots and disc edema. Often the retina may be ophthalmoscopically normal.4,13 In one case of traumatic asphyxia, fluorescein angiography revealed in one eye blockage of fluorescence by retinal hemorrhage, blurring of the background choroidal pattern associated with cotton-wool spots, and hyperfluorescence with dye leakage associated with hemorrhage at the nasal edge of the disc. The other eye was angiographically normal.15 The fundus changes of this patient improved over a period of weeks, but the visual acuity of the right eye had decreased from 20/30 (6/9) to 20/100 (6/30), presumably as a result of mottling and disruption of the retinal pigment epithelium.15 The pathogenesis of traumatic asphyxia retinopathy and Purtscher's retinopathy may be similar but the ecchymotic appearance associated with traumatic asphyxia separates the two entities. Purtscher's retinopathy usually has no associated external findings.15 Also, the development of Purtscher's retinopathy may be slower than the retinopathy of traumatic asphyxia. Again, patients with traumatic asphyxia are treated supportively.
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Fat embolism retinopathy is usually secondary to fat embolism syndrome (FES). Retinal findings variably include cotton-wool spots, intraretinal hemorrhages, and visible emboli. FES was first described in 1861 in patients suffering fractures of medullated bones.16 The most likely fractures producing the syndrome are fractures of the lower extremities and the pelvis.17–19 Typically, the manifestations of FES ppear within 24 to 48 hours after the injury, but the syndrome is only recognized in approximately 5% of patients with long bone fractures. Some of these manifestations include petechial rashes, respiratory insufficiency, retinal lesions, and altered mental status.20 In patients with manifest FES, 50% to 60% may have retinal findings.19–21 Chuang et al.22 reported that of 100 consecutive long bone fracture cases, four patients demonstrated retinal pathology resulting from subclinical FES. In this series three patients had normal vision and one patient complained of a visual field defect. Fundus examination classically reveals cotton-wool spots and intraretinal hemorrhages. Additional fundus findings include intravascular fat emboli and central retinal artery occlusion.6,22–24 This retinopathy has occurred after facial autologous fat injection.25,26 Various alterations in lipid homeostasis are probably involved in the pathogenesis of FES. Retinal microinfarcts from fatty emboli have been demonstrated histopathologically, and this may reveal, at least in part, the etiology of the retinal findings.6 The retinal lesions resolve after resolution of the FES. With resolution, most patients are asymptomatic, although permanent scotomas can occur.21
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Valsalva retinopathy develops in response to the valsalva maneuver. A rapid rise in abdominal pressure especially against a closed glottis characterizes this maneuver (Fig. 2). Some common settings in which the Valsalva maneuver occurs include heavy lifting, coughing, vomiting, or bowel movement. A history of such activity is helpful in establishing the diagnosis of Valsalva retinopathy. Patients may be asymptomatic but usually complain of decreased vision. Fundus examination most commonly reveals a red, dome-shaped hemorrhage underneath the internal limiting membrane (ILM).27 A fluid level may be observed in the area of the hemorrhage as the blood settles inferiorly under the ILM.27 Valsalva retinopathy may cause decreased visual acuity if blood obscures the macula. Also, extramacular preretinal hemorrhage may diffuse into the vitreous, which may limit vision.3,21 Usually, the sub-ILM hemorrhage resorbs after several days to weeks. A serous detachment of the ILM may persist after the blood resolves but spontaneous reattachment usually occurs, leaving a normal-appearing fundus.27 Fluorescein angiography typically shows no retinal vascular alterations except in cases in which Valsalva retinopathy has been associated with other retinal disorders such as retinal artery macroaneurysm or diabetic retinopathy.27–29 Duane30 proposed that the pathophysiology might be related to the rapid rise in intravenous pressure caused by the Valsalva maneuver. With a rise in intraocular venous pressure, the Valsalva maneuver causes a rupture of the superficial retinal capillaries.27,30–32 For the usual cases of valsalva retinopathy, the preretinal hemorrhage clears spontaneously, though neodymium–yttrium-aluminum-garnet laser disruption of the internal limiting membrane has been used to allow a more rapid clearing of the preretinal hemorrhage.33

Fig. 2 Valsalva hemorrhagic retinopathy 1 day after extremely heavy lifting. Note preretinal hemorrhage obscuring the macula.

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Whiplash maculopathy occurs after flexion–extension head and neck injuries. Patients who have suffered whiplash injuries may complain of a wide range of ophthalmic disturbances. In one study, 26% of patients with whiplash injuries had ophthalmic complications, most commonly a loss of accommodation or convergence.34 Reports of retinal damage attributed to whiplash injury are less common. Whiplash maculopathy symptoms begin with a mild blurring of vision that occurs at the time of the injury, usually in the 20/30 range. The symptoms are bilateral and resolve over a period of a few days. Acutely, the fundus examination may reveal a minimal detachment of the posterior vitreous with a grayish appearance of the fovea. Later, a crater-like depression of less than 100 μmin diameter with slight retinal pigment epithelium disturbances may be found and may remain unchanged over time even though symptoms may resolve.35,36 Some foveal pits may be the result of remote whiplash injury. Small opercula in association with whiplash maculopathy suggest that a true retinal excavation exists and that the pathogenesis of whiplash maculopathy is related to vitreous traction on the macula.35
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Caffey37 described the first cases of a form of child abuse that he later termed the whiplash shaken infant syndrome. This syndrome is defined by violent shaking of an infant that commonly results in intraocular and intracranial hemorrhages (Fig. 3). Ocular complications occur commonly in child abuse cases. Several studies have found that 35% to 46% of abused children may have eye injuries.38–40 Importantly, the shaken infant may present with minimal external signs of trauma. Certainly, numerous cases have occurred in which the head has shown no signs of visible trauma. The mortality rate (15%) and the morbidity rate (50%) underscores the importance of recognizing this form of child abuse.41 Ocular examination usually reveals any combination of subretinal, intraretinal, preretinal, or vitreous hemorrhage. The presence of intraocular hemorrhage is a predictor of intracranial hemorrhage, and the severity of the intraocular hemorrhage correlates with the severity of the acute neurologic injury.42,43 The usual intracranial manifestation of the shaken baby syndrome is subdural hematoma. Bridging dural vessels may tear and bleed in response to repetitive acceleration and deceleration motion of the brain caused by shaking. Early views held that the pathogenesis of shaken baby retinopathy related to an acute rise in intracranial pressure.37 More recently, Greenwald and associates44 proposed that the same acceleration and deceleration forces that cause intracranial hemorrhage act on the vitreous. Vitreous forces perpendicular to the plane of the retina cause a separation of the ILM or a splitting of deeper retinal layers. Retinal hemorrhages result from tearing of small retinal vessels.44,45 The clinical course of shaken baby retinopathy ranges from complete clearing to severe visual loss secondary to optic atrophy or macular scarring.46 Treatment of the retinal manifestations of shaken baby syndrome is most commonly supportive, but the ophthalmologist must be wary of the possibility of significant intracranial trauma in the shaken infant.

Fig. 3 Shaken baby syndrome in a 4-month-old boy. Note scattered retinal hemorrhages and dome-shaped, hemorrhagic retinoschisis. (Spaide RF, Swengel RM, Scharre DW, et al: Shaken baby syndrome. Am Fam Physician 41:1145, 1990, with permission)

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1. Purtscher O: Angiopathia retinae traumatica, Lymphorrhagien des Augengrundes. Graefes Arch Ophthalmol 82:347, 1912

2. Fischbein F, Safir A: Monocular Purtscher's retinopathy. Arch Ophthalmol 85:480, 1971

3. Burton TC: Unilateral Purtscher's retinopathy. Ophthalmology 87:1096, 1980

4. Marr WG, Marr EG: Some observations on Purtscher's disease: Traumatic retinal angiopathy. Am J Ophthalmol 54:693, 1962

5. Roden D, Fitzpatrick G, O'Donoghue H, et al: Purtscher's retinopathy and fat embolism. Br J Ophthalmol 73:677, 1989

6. Kearns TP: Fat embolism of the retina. Am J Ophthalmol 41:1, 1956

7. Blodi BA, Johnson MW, Gass DM, et al: Purtscher's-like retinopathy after childbirth. Ophthalmology 97:1654, 1990

8. Behrens-Baumann W, Scheurer G, Schroer H: Pathogenesis of Purtscher's retinopathy. Graefes Arch Clin Exp Ophthalmol 230:286, 1992

9. Stoumbos VD, Klein ML, Goodman S: Purtscher's-like retinopathy in chronic renal failure. Ophthalmology 99:1833, 1992

10. Patel MR, Bains AK, O'Hara JP, et al: Purtscher retinopathy as the initial sign of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome. Arch Ophthalmol 119:1388, 2001

11. Power MH, Regillo MC, Custis PH: Thrombotic thrombocytopenic purpura associated with purtscher retinopathy. Arch Ophthalmol 115:128, 1997

12. Gass JDM: Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment, 4th ed., Traumatic Retinopathy St. Louis: CV Mosby, 1987:746–747

13. Duke-Elder S, MacFaul PA: System of Opthalmology, Vol. 14, part 1: Mechanical Injuries, St. Louis: CV Mosby, 1972:718–729

14. Fred HL, Chandler FW: Traumatic asphyxia. Am J Med 29:508, 1960

15. Ravin JG, Meyer RF: Fluorescein angiographic findings in a case of traumatic asphyxia. Am J Ophthalmol 75:643, 1973

16. Zenker FA: Beitrage zur Anatomie und Physiologie der Lunge, Dresden, Braunsdorf, 1861. p 20

17. Donnell JM: Observations of clinical fat embolism. Can Med Assoc 186:1060, 1962

18. Benatar SR, Ferguson AD, Goldschmidt RB: Fat embolism: some clinical observations and a review of controversial aspects. Q J Med 41:8, 1972

19. Gossling HR, Pellegrini VD Jr: Fat embolism syndrome: a review of the pathophysiology and physiological basis of treatment. Clin Orthop 165:68, 1982

20. Gurd AR, Wilson RI: The fat embolism syndrome. J Bone Joint Sur g 56:408, 1974

21. Thomas JE, Ayyar DR: Systemic fat embolism. A diagnostic profile in 24 patients. Arch Neural 26:517, 1972

22. Chuang EL, Miller FS, Kalina RE: Retinal lesions following long bone fractures. Ophthalmology 92:370, 1985

23. Adams CBT: The retinal manifestations of fat embolism. Injury 2:221, 1971

24. Evans JJ: Cerebral fat embolism with recovery: and involvement of the central retinal artery. Br J Ophthalmol 24:614, 1940

25. Danesh-Meyer HV, Savino PJ, Sergott RC: Case reports and small case series: ocular and cerebral ischemia following facial injection of autologous fat. Arch Ophthalmol 119:777, 2001

26. Teimourian B: Blindness following fat injections. Plast Reconstr Surg. 82:361, 1988

27. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment, 4th ed., Traumatic Retinopathy St. Louis: CV Mosby, 1987:752–754

28. Avins LR, Krummenacher TK: Valsalva maculopathy due to a retinal arterial macroaneurysm. Ann Ophthalmol 15:421, 1983

29. Kassoff A, Catalano RA, Mehu M: Vitreous hemorrhage and the Valsalva maneuver in proliferative diabetic retinopathy. Retina 8:174, 1988

30. Duane TD: Valsalva hemorrhagic retinopathy. Am J Ophthalmol 75:637, 1973

31. Duane TD: Valsalva hemorrhagic retinopathy. Trans Am Ophthalmol Soc 70:298, 1972

32. Williams DF, Mieler WF, Williams GA: Posterior segment manifestations of ocular trauma. Retina 10:S35, 1990

33. Gabel V-P, Birngruber R, Gunther-Koszka H, et al: Nd: YAG laser photodisruption of hemorrhagic detachment of the internal limiting membrane. Am J Ophthalmol 107:33, 1989

34. Burke JP, Orton HP, West J, et al: Whiplash and its effect on the visual system. Graefes Arch Clin Exp Ophthalmol 230:335, 1992

35. Kelley JS, Hoover RE, George T: Whiplash maculopathy. Arch Ophthalmol 96:834, 1978

36. Daily L: Macular and vitreal disturbances produced by traumatic vitreous rebound. South Med J 63:1197, 1970

37. Caffey J: The whiplash shaken infant syndrome: Manual shaking by the extremities with whiplash-induced intracranial and intraocular bleedings, linked with residual permanent brain damage and mental retardation. Pediatrics 54:396, 1974

38. Harley RD: Ocular manifestations of child abuse. J Pediatr Ophthalmol Strabismus 17:5, 1980

39. Friendly DS: Ocular manifestations of physical child abuse. Trans Am Acad Ophthalmol Otolaryngol 75:318, 1971

40. Jensen AD, Smith RE, Olson MI: Ocular clues to child abuse. J Pediatr Ophthalmol 8:270, 1971

41. Ludwig S, Warman M: Shaken baby syndrome: A review of 20 cases. Ann Emerg Med 13:104, 1984

42. Tomasi LG, Rosman NP: Purtscher's retinopathy in the battered child syndrome. Am J Dis Child 129:1335, 1975

43. Wilkinson WS, Han DP, Rappley MD, et al: Retinal hemorrhage predicts neurologic injury in the shaken baby syndrome. Arch Ophthalmol 107:1472, 1989

44. Greenwald MJ, Weiss A, Oesterle CS, et al: Traumatic retinoschisis in battered babies. Ophthalmology 93:618, 1986

45. Greenwald MJ: The shaken baby syndrome. Semin Ophthalmol 5:202, 1990

46. Mushin AS: Ocular damage in the battered-baby syndrome. BMJ 3:402, 1971

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