514 Wolfgang Schrader and Ferenc Kuhn
3.3.2Evaluation
The most important diagnostic tool is the ophthalmoscope; FLAG plays more of a role in these injuries than in a typical, direct eye injury. Specific diagnostic information is provided below with each condition (see Chap. 1.9 for further details).
3.3.3Specific Injuries
3.3.3.1Heavy Blood Loss
Massive or recurrent blood loss, especially from the gastrointestinal system, can lead to anemia and secondary arterial hypotension. AION is the consequence that is responsible for the visual loss [12] via, presumably, a sudden decrease of the arterial blood pressure and release of endogenous factors [10].
3.3.3.1.1 Symptoms
Following a serious bleeding, the patient may notice a temporary visual loss, with a recovery after several minutes. A few days later (occasionally weeks) the patient notices bilateral, acute, irreversible visual loss, ranging from a small, usually inferior, visual field defect to complete blindness.
3.3.3.1.2 Evaluation
On ophthalmoscopy, the typical appearance of an AION is seen, with disc edema in the acute phase and an optic atrophy in the late phase. The FLAG is not very helpful; the peripapillary choroidal and retinal perfusion may be decreased [10].
3.3.3.1.3 Treatment
There is no effective therapy for AION treatment; further vasoconstriction should be avoided or medically reversed (with angiotensin-converting enzyme inhibitors).
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3.3.3.2High-altitude Retinopathy
Hypoxia at high altitudes (typically above 4000 m ) causes increased retinal blood flow and blood volume possibly via autoregulatory mechanisms. Physical strain with Valsalva maneuvers (e.g., during mountain climbing) compounds the condition via increased retinal venous pressure. A hypoxic retinal capillary bed exposed to increased retinal venous pressure predisposes to intraretinal hemorrhage.
3.3.3.2.1 Symptoms
Although up to 60% of mountaineers develop high-altitude retinopathy in altitudes above 4000 m [20], usually no symptoms are noted unless vitreous hemorrhage occurs.
3.3.3.2.2 Evaluation
On ophthalmoscopy, a marked increase of retinal vessel diameters with tortuosity of arterioles and venules are seen, along with hyperemia or edema of the disc. The intraor preretinal hemorrhages often spare the macular area.
3.3.3.2.3 Treatment
The retinal changes disappear within weeks. To prevent high altitude retinopathy, ascending slowly and the use of supplemental oxygen are recommended.
3.3.3.3Hyperbaric Trauma
Not only a hypobaric, but also a hyperbaric, environment can cause ocular damage, usually during diving, but also following hyperbaric oxygen
Approximately 13,000 feet.
Those sensitive to the decreased atmospheric pressure may also have difficulty in flying airplanes at high altitudes or in unpressurized airplanes: vasodilatation and intraocular hemorrhages may occur. The low pressure must also be taken into consideration when intraocular gas tamponade is used: gas expansion occurs with a spike in the IOP.
516 Wolfgang Schrader and Ferenc Kuhn
therapy. The term “hyperbaric trauma” summarizes three different injuries: barotrauma; decompression disease; and air embolism.
The eyes are directly exposed to pressure changes with the diver wearing an air-filled head gear. When the diver is deep in the water, a negative pressure in the mask may cause lid edema and conjunctival hemorrhages. Conversely, a diver may suffer from Caisson disease after too fast a decompression. Because the intracorporal air pressure may decrease by more than one bar, air bubbles can develop in the vessels and cause vascular occlusions [2]. If lung alveoli rupture, air bubbles may directly enter the vessels and cause occlusions in the central artery or in the intracranial arteries.
3.3.3.3.1 Evaluation
Lid edema and subconjunctival hemorrhages may be visible at the slit lamp. On ophthalmoscopy, signs of retinal vascular occlusions can be found.
3.3.3.3.2 Treatment
The vaso-occlusive changes may be irreversible and unresponsive to medical treatment.
3.3.3.4Purtscher’s Retinopathy
Described in 1868, this distinctive retinal pathology can be caused by head trauma, chest compression , fracture of a (long) bone, orbital and liver trauma, angiography, and surgery .
3.3.3.4.1 Evaluation
Although unilateral cases have also been reported [27], the symptoms are usually bilateral, even if asymmetrical. They present within a few hours, but no later than 4 days, after the trauma. The patients complain about decreased vision, typically between CF and 20/200.
On ophthalmoscopy (Fig. 3.3.1), the following symptoms are found:
The most common etiology is an MVC. Carotid, thoracic, renal.
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•Superficial retinal hemorrhages
•Serous macular detachment
•Dilated and tortuous vasculature
•Numerous white patches or confluent cotton wool spots around the optic disc
•Disc edema
On FLAG the characteristic signs are:
•Focal areas of arteriolar obstruction
•Patchy capillary nonperfusion
•Disc edema
•Dye leakage from retinal arterioles, capillaries, and venules [27]
•Blockage of background choroidal fluorescence but usually normal choroidal filling in the acute stage
•Late perivenous staining and/or partial vein obstruction
•Disc edema
Fig. 3.3.1 Purtscher’s retinopathy. Hemorrhages and multiple cotton-wool spots are visible in the posterior pole in this patient who was injured in an MVC
518 Wolfgang Schrader and Ferenc Kuhn
3.3.3.4.2 Treatment
Observation is sufficient. The retinal lesions resolve over a period of weeks to a few months. After resolution, the fundus may appear normal, but pigment migration and optic atrophy can occur [3]. Visual acuity returns to normal or near normal in most eyes. Initially, visual field defects may occur, including central, paracentral, segmental, or annular scotomas. The visual field defects usually resolve completely, although, particularly if optic atrophy has occurred, they may be permanent [3]. Careful documentation is needed to be able to subsequently prove that the damages are trauma related.
3.3.3.5Shaken Baby Syndrome
3.3.3.5.1 Introduction
The traumatic sequelae of nonaccidental injuries occurring in infants and young children as a consequence of violent shaking are called shaken baby syndrome. The reported mortality rate of 15% underscores the importance of recognizing this form of child abuse [19]. In central Europe, 3.5% of parents confessed to having used violence against their children so that it might have resulted in severe injury and 10% of children admitted to hospital due to injury showed evidence of physical violence; less than 5% of abused children become known to the authorities. The typical victim of shaken baby syndrome is a male infant younger than 6 months of age who is alone with the perpetrator at the time of injury [16]. The injury is unrelated to race, gender, socioeconomic status, or education [16].
An infant is more likely to suffer from intracranial and intraocular bleeding as a result of shaking [7] because the head is proportionately larger and heavier relative to the body than that of an older child or adult, and the still weak neck muscles provide less stability and protection.
Synonyms include whiplash shaken infant syndrome, battered child syndrome, and child abuse syndrome.
Two-thirds of the abused children are babies.
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3.3.3.5.2 Evaluation
The diagnosis of child abuse requires a high index of suspicion. The history of shaking is characteristically lacking, and there may be minimal external signs of trauma.
ZCave
The leading sign of child abuse is ocular in 4−6% of cases, but the vast majority of abused children have some eye involvement.
While after accidental head injuries nearly all children under 3 years of age have no abnormality on fundus examinations, most babies with nonaccidental head injuries show retinal hemorrhages of varying degree, occurring in 11−23% of all physically abused children and in 50−80% of shaken babies [9, 18, 24].
On ophthalmoscopy, intraocular hemorrhage can be present in various locations: subretinally; intraretinally; preretinally (subhyaloidally); and intravitreally [17]. The blood is concentrated in the posterior pole region and is usually bilateral. The amount of intraocular blood correlates with the degree of the acute neurological damage [29]. Cotton-wool spots, whitecentered hemorrhages (Roth’s spot), macular edema, disc edema, and retinoschisis are less common [6, 30].
On CT or MRI, the intracranial pathology includes subarachnoid or intracerebral hemorrhage, cerebral edema, and cerebral atrophy. Elevated intracranial pressure is often present. A variety of neurological symptoms can occur: irritability; lethargy; seizures; and coma. Death should not be an unexpected consequence [31].
3.3.3.5.3 Differential Diagnosis
In infants retinal hemorrhages are most common post partum or in shaken babies: 19−32% of eyes show retinal hemorrhage in one or both eyes 24 h after a normal birth. By 72 h the rate drops to ~13%, and these hemorrhages usually completely resolve within 6 weeks. If intravitreal or retinal hemorrhages are found in a child older than 6 weeks, this is most likely due to abuse.
520 Wolfgang Schrader and Ferenc Kuhn
3.3.3.5.4 Management Strategy
Suspicion of a child abuse requires a multidisciplinary approach and a careful documentation (e.g., photography and ultrasonography) of all findings. If violence is suspected, a detailed systemic examination has to be performed. The ophthalmologist has a key role because of the pathognomic appearance of the fundus (other causes of retinal hemorrhages have to be carefully ruled out; Table 3.3.1).
Table 3.3.1 Differential diagnosis of traumatic retinopathy. (Modified after [3])
|
Contusion |
Purtscher’s |
Purtscher’s |
Purtscher’s |
Valsalva |
|
retinopathy |
retinopathy |
retinopathy |
retinopathy |
retinopathy |
|
|
|
(fat embo- |
(traumatic |
|
|
|
|
lism) |
asphyxia) |
|
Type of |
Directly to |
Chest |
Fracture of |
Chest com- |
Valsalva ma- |
trauma |
the eye |
compres- |
long bones, |
pression |
neuver |
|
|
sion, head |
multiple |
|
|
|
|
injury |
injuries |
|
|
Accom- |
None |
None |
Pulmonary |
Blue-black |
None |
panying |
|
|
and cerebral |
discolor- |
|
systemic |
|
|
signs, |
ation of up- |
|
picture |
|
|
petechial |
per body |
|
|
|
|
hemorrhages |
|
|
Onset of |
None |
None |
Symptom- |
Immediate |
None |
systemic |
|
|
free interval |
|
|
picture |
|
|
for a few |
|
|
|
|
|
days |
|
|
Initial |
Normal |
Variable |
Normal |
Normal to |
Normal to |
vision |
to CF |
|
|
NLP |
CF |
Duration |
Several |
Several |
Several days |
Several |
Several |
of re- |
days |
weeks |
|
weeks |
weeks |
duced vi- |
|
|
|
|
|
sion |
|
|
|
|
|
|
Contusion |
Purtscher’s |
Purtscher’s |
Purtscher’s |
Valsalva |
|
retinopathy |
retinopathy |
retinopathy |
retinopathy |
retinopathy |
|
|
|
(fat embo- |
(traumatic |
|
|
|
|
lism) |
asphyxia) |
|
Final vi- |
Usually |
Usually |
Normal |
Normal to |
Usually |
sion |
normal, |
normal, |
|
NLP |
normal, |
|
sometimes |
sometimes |
|
|
sometimes |
|
impaired |
impaired |
|
|
impaired |
Eye’s |
Normal to |
Normal |
Normal to |
Subcon- |
Normal to |
external |
contused |
|
petechial |
junctival |
conjunctival |
appear- |
|
|
conjunctival |
hemor- |
hemor- |
ance |
|
|
hemorrhages |
rhages |
rhages |
Fundus |
Retinal |
Exudates |
Exudates |
Normal or |
Retinal |
picture |
whitening |
and hemor- |
and hemor- |
hemor- |
hemor- |
acutely |
|
rhages |
rhages, reti- |
rhages, |
rhage, sub- |
|
|
|
nal edema |
rarely |
ILM blood |
|
|
|
|
exudates |
|
Time from |
Within a |
Within |
After 1 or |
Immediate |
Acutely |
trauma |
few hours |
4 days |
2 days |
or 2 days |
|
to fundus |
|
|
|
|
|
changes |
|
|
|
|
|
sorb within a few months. Late manifestations include perimacular retinal folds, chorioretinal atrophy or scarring, optic atrophy, and retinal detach-
522 Wolfgang Schrader and Ferenc Kuhn
ment [8, 17]. A retinal detachment or a nonclearing vitreous hemorrhage requires surgical treatment (see Chaps. 2.9, 2.16).
3.3.3.5.5 Prognosis and Outcome
The clinical course of shaken baby retinopathy ranges from complete resolution to severe visual loss due to optic atrophy or macular scarring [21]. Postmortem examination of the optic nerves in shaken babies often reveal perineural hemorrhage, which may contribute to the poor outcome in survivors through nerve fiber compression and optic atrophy [15, 22]. A 50% incidence of gazing disorders is reported in shaken baby syndrome, reflecting nervous system insults [19].
3.3.3.6Terson Syndrome
Terson syndrome (Fig. 3.3.2) is defined as a vitreous hemorrhage occurring in association with any form of acute intracranial hemorrhage; the incidence reaches 8% [14]. Other types of intraocular bleeding is found in up to 20% of eyes [5, 30]. Subarachnoid bleeding from a cerebral aneurysm, in particular an aneurysm of the anterior communicating artery, is the most common underlying cause in adults [5], but traumatic subdural bleeding in children (see Chap. 2.16) [14].
3.3.3.6.1 Evaluation
On ophthalmoscopy, multiple preretinal, intraretinal, and subretinal hemorrhages can be seen if the vitreous hemorrhage allows inspection of the retina. A dome-shaped accumulation of blood is found at the macula in 39% of eyes, two-thirds of which is underneath the ILM [14]. Ultrasono graphy allows preoperative identification of the hemorrhagic macular cyst, and surgical intervention may have to be performed more urgently in such cases.
Subarachnoidal or subdural, occasionally intracerebral.
Hemorrhagic macular cyst.
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Fig. 3.3.2 Terson syndrome. Intraoperative image of an eye with a submembranous hemorrhagic cyst in the macula. The cyst’s border is clearly visible, delineated by a white line representing the ILM’s reinsertion into the retina. The cyst still contains blood, which is degenerated and has turned white. The scissors is used to open the ILM for complete blood evacuation (R. Morris, Birmingham, Alabama)
ZCave
The amount of intraocular hemorrhage directly correlates with the rapidity and magnitude of intracranial pressure elevation. The presence of intraocular hemorrhage is associated with increased morbidity and mortality [4, 28].
3.3.3.6.2 Management
The final visual outcome may be good without pars plana vitrectomy [25], but surgery is able to achieve rapid visual rehabilitation and prevent PVR and retinal detachment [14, 28]. Vitrectomy is especially useful if a hemorrhagic macular cyst is present or if the patient is in the amblyopic age.
The decision whether to observe or operate should be discussed with the patient, family, neurologist, and the physical therapist (see Chap. 1.4).
This is true even if the prognosis is much worse in children than in adults.
