Archives ofDisease in Childhood 1990; 65: 1369-1372 1369

CURRENT TOPIC Arch Dis Child: first published as 10.1136/adc.65.12.1369 on 1 December 1990. Downloaded from

Retinal haemorrhages

Baljit Kaur, David Taylor

Site and appearance of retinal haemorrhages turbance of the underlying retinal pigment epithe- Retinal haemorrhages occur when there is extra- lium. vasation of blood, either into the itself Haemorrhage beneath the retinal pigment (intraretinal), between the retina and the retinal epithelial layer is usually confmed, and related to a pigment epithelium (subretinal), or between the rupture (for example, trauma or pseudoxanthoma retina and the hyaloid face of the vitreous body elasticum) in Bruch's membrane, which is nor- (subhyaloid or preretinal). The shape and colour of mally adherent to it. This results in diffuse, the haemorrhages are determined by the level or roughly linear haemorrhages of variable- levels of the retina affected, and they are often sometimes great-extent. mixed. Often the hyaloid face ruptures and the Haemorrhage within the choroid expands freely haemorrhage spreads into the vitreous (intra- and forms pool like areas the ophthalmoscopic vitreal). Richman in 1936 classified retinal appearance of which is governed by the density of haemorrhages into four groups according to the the overlying pigmentation. Generally, choroidal morphological appearances,' and their location can haemorrhages appear as dark reddish blue areas, be determined by their clinical appearance. obviously deep in the fundus, and they usually The ophthalmoscopic appearance of preretinal absorb leaving a normal fundus. blood depends on its age and the size and shape of Some intraretinal and preretinal haemorrhages the blood filled space. Fresh preretinal blood have white centres. Such lesions (or Roth's spots) usually looks like a dome shaped, semilunar, cres- are not specific for any single underlying disorder. centic, or geographic accumulation of dark red The white centre can be focal ischaemia, an blood overlying and obscuring its source. With inflammatory infiltrate, a colony of infective time the same accumulation of blood can look organisms, fibrin and platelets, or an accumulation semilunar or crescentic in shape, with a fluid level of neoplastic cells. It is easy to mistake the bright caused by gravitational settling of the formed ele- light reflex from the internal limiting membrane

ments. The plasma-erythrocyte interface may shift over the apex of a retinal haemorrhage for the http://adc.bmj.com/ with changes in the position of the head. A faint small white centre of a haemorrhage. line of residual blood usually marks the superior Intravitreal haemorrhages are relatively uncom- extent of the space originally filled with whole mon in children; they usually resolve slowly but blood. completely,2 but in young children may cause Intraretinal haemorrhages may be splinter warranting early vitrectomy. shaped, flame shaped, or 'dot and blot' rounded haemorrhages. Splinter haemorrhages are small, linear, dull red, and lie superficially about the disc Aetiology on September 24, 2021 by guest. Protected copyright. margin parallel to the major vessels, their shape NEONATAL RETINAL HAEMORRHAGES being determined largely by the nerve fibre layer Jaeger was probably the first to have reported this ofthe retina and the vessels. They may be single or condition.3 The exact mechanism of neonatal reti- multiple, and they clear rapidly. nal haemorrhages is not clear, but it has been Flame shaped haemorrhages are bright red, and suggested that blood viscosity,4 prostaglandins in lozenge shaped with a striated or serrated outline the fetal circulation,5 and the unequal pressure dis- on at least one margin, and they follow the course tribution during birth-the sudden release of of the retinal nerve fibre layer. They occur in the pressure with a rapid change in intracranial posterior pole of the fundus and are characteristi- pressure-may be partly responsible.6 Direct cally associated with disorders of. the superficial trauma is not implicated in most cases. radial peripapillary capillaries. This usually benign form of haemorrhagic Dot and blot haemorrhages are dense, dark red, retinopathy usually presents as flame shaped or and sharply outlined. They are located within the blot haemorrhages located in the posterior pole or compact middle layers of the retina and are seen in periphery.7 This type of haemorrhage has been a association with disorders that predominantly subject of a monograph8 and the reported inci- affect the deep retinal capillary system. Occasio- dence varies enormously, from 2-6% to 50%.4-- nally several discrete blot haemorrhages may The reason for this is the variable time before The Hospitals for coalesce and appear as an irregular geographic fundus examination after birth, the technique of Sick Children, accumulation of blood. examination, and whether this is histological or Great Ormond Street, as by direct or indirect . Von London WC1N 3JH Subretinal haemorrhages typically appear Baljit Kaur irregular red plaques or opaque reddish circular Barsewisch pointed out that whether the examina- David Taylor lesions deep to the retina and retinal vessels tion was done by an experienced or inexperienced Correspondence to: (between the photoreceptors and pigment epithe- ophthalmologist or a paediatrician or other, signifi- Mr Taylor. lial layer). On resolution they can result in a dis- cantly influenced the results8; the method of 1370 Kaur, Taylor

delivery of the baby is also important. Retinal Retinal haemorrhages from crush injuries to the haemorrhages occur in about one third of babies chest are usually accompanied by retinal infarcts Arch Dis Child: first published as 10.1136/adc.65.12.1369 on 1 December 1990. Downloaded from born by occipital presentation, increasing in inci- ('cotton wool spots') and exudates in the so called dence with prolonged labour and obstetric Purtscher's retinopathy,33 the mechanisms for procedures,'2 and decreasing in breech presenta- which may be air embolism,3m an acute rise in tions and birth by caesarean section. Retinal intraocular venous pressure, or hypoxia from haemorrhages are more common in babies of angiospasm of the retina.33 elderly primiparas,7and mothers with toxaemia. 13 The sudden rise in intrathoracic pressure as a Retinal haemorrhages in newborn infants result of forceful contraction of the thoracoabdo- usually resolve within a few days.4 Follow up stu- minal muscles against a closed glottis causes an dies have reported normal visual development. 14 15 acute rise in central venous pressure, which in turn increases the intracranial pressure as the intra- cranial venous sinuses are in direct communica- SUBARACHNOID HAEMORRHAGE tion with the superior vena cava and there are no When bleeding from any cause occurs into the intervening valves in the large veins above the subarachnoid space there may be increased intra- heart. This acute raised central venous pressure cranial pressure, optic nerve sheath haemorrhage, occurs with the Valsalva manoeuvre,31 in cases of and an increase in the pressure within the optic trauma when there is forced compression of the nerve sheath because of raised central retinal chest (usually anteroposteriorly) against a closed venous pressure and obstruction of the retinocho- glottis,35 and also in epileptic seizures,36 37 vomit- roidal anastomosis 6; to this may be added raised ing, or spells of coughing.37 central venous pressure as a result of epileptic Appreciable rises in cerebral venous pressure seizures.17 The incidence of retinal haemorrhages (10-20 cm H20) have been reported38 in neonates in subarachnoid haemorrhage is between 20%18 during episodes of crying, abdominal straining, and 32%,19 and they usually occur simultaneously and convulsions.38 Values of cerebral venous or are noted within a few days.20 Streak and pressure above 100 cm H20 have been shown preretinal haemorrhages occur mainly around the experimentally during a tonic seizure.39 The optic disc and often accompany papilloedema. increase in muscle tone and intrathoracic pressure These haemorrhages often resolve spontaneously.2' makes an important contribution to this. These Occasionally large preretinal haemorrhages may transient rises in cerebral venous pressure are break into the vitreous (Terson's syndrome). This probably responsible for the small cerebral is often a delayed phenomenon. The pathogenesis haemorrhages seen after experimental seizures.40 of haemorrhages in Terson's syndrome is controv- Retinal haemorrhages may occur under these cir- ersial. One view is that blood from the subarach- cumstances but only rarely do they have more than noid space is forced into the optic nerve sheath and transient effect.37 41 The coincident rise in penetrates the lamina cribrosa to appear within the intraocular pressure at such times reduces the globe.22 23 Intraocular and optic nerve sheath pressure difference across the vessel wall and may haemorrhages have, however, been reported in give a protective effect against more profuse cases of sudden intracranial in which bleeding.37 there had been no intracranial bleeding.24 Other This mechanism could explain the occurrence of authors'6 18 25 believe that intraocular haemor- retinal haemorrhages after clumsy attempted rhage is the result of rupture of retinal veins resuscitation of infants by inexperienced adults. http://adc.bmj.com/ secondary to retinal venous hypertension, which in turn is the result of obstruction of both the central retinal vein and the retinochoroidal NON-ACCIDENTAL INJURY anastomosis.16 18 25 For this to occur the intra- Ocular complications of non-accidental injury cranial pressure must rise much higher than it does were first described in 1964.42 The most common to cause nerve sheath finding is retinal haemoffhage,43 44 which occur in optic haemorrhage alone.2' up to 900/o of such children." These haemorrhages In subdural haemorrhage the rise in intracra- can last for several years, in contrast to those seen on September 24, 2021 by guest. Protected copyright. nial pressure is less acute; they are usually at birth.43 The cause cannot be determined by fun- post-traumatic,26 but acute subdural haematoma dal exaniination alone or by the morphology of the has been described in association with Purtscher's retinal haemorrhages. retinopathy (which is normally associated with In non-accidental injury there are several thoracic compression) in an infant with non- mechanisms that cause the haemorrhage. accidental injury,27 and in infants with pre-existing asymptomatic chronic subdural haematoma or subdural effusion with occult Raised intraocular venous pressure craniocerebral disproportion.28 Retinal and pre- This occurs by two mechanisms, often occurring retinal haemorrhages are consistently present in all together. Firstly, raised intracranial pressure is infants with acute subdural haematoma.29 transmitted to the subarachnoid space, either through clear cerebrospinal fluid or by haemorrhage.30 The raised optic nerve sheath RAISED CENTRAL VENOUS PRESSURE pressure compresses the central retinal vein, and Raised central venous pressure from crying, sei- the chorioretinal anastomosis thus raising the zures, or squeezing of the chest, by any mechan- pressure upstream to the capillary bed.'6 ism where there is forced contracture of the chest In non-accidental injury the intraocular haemor- against a closed or partially closed glottis,30 may rhages are often accompanied by intracranial cause the so called Valsalva's haemorrhagic haemorrhages with associated damage.46 The retinopathy,3' which is usually associated with severity ofintraocular haemorrhage correlates with superficial retinal haemorrhages. Haemorrhages the severity of acute neurological injury.47 Diffuse into all layers of the retina may be more common fundal impairment, vitreous haemorrhage, or large in non-accidental injury, at least in pathological subhyaloid haemorrhages are associated with more studies.30 32 severe acute neurological injury.47 Retinal haemorrhages 1371

The second mechanism is raised central venous INFECTIONS pressure, as already discussed. Viral infectims Arch Dis Child: first published as 10.1136/adc.65.12.1369 on 1 December 1990. Downloaded from In cytomegalovirus infection the retinal haemor- rhages are usually flame shaped, and occur after the white lesions that look similar to 'cotton wool Rapid deceleratum or cycles ofrapid acceleratin and spots' and retinal necrosis; they may resemble reti- deceleration nal branch vein occlusions. The infective process Ober and Lyle et al have implied that the shear spreads slowly along the course of the retinal blood forces generated by acceleration or deceleration, or entire fundus. Retinal both, have been a factor in the production of vessels to encompass the 48 have haemorrhages are seen in acquired cytomegalo- haemorrhages.32 Duhaimi et al, however, virus after cytotoxic and corticosteroid reported that the -at least renal and in in its most severe form-is not usually caused treatment in recipients of grafts by non-impact acceleration-deceleration forces patients with neoplastic disease.56 alone.49 The tendency for more severe retinopathy to occur in infants than in older children could be explained by clinical and experimental evidence Other infections that suggests that intraocular haemorrhages may Rickettsial infections,57 subacute bacterial endo- be caused by abrupt increase in retinal venous carditis, and herpes simplex are other known pressure secondary to an acute rapid rise in intra- causes of retinal haemorrhages. cranial pressure.'6 30 Infants weigh less, have a greater head size in relation to body size, and have less developed neck musculature to support the FAMILIAL RETINAL ARTERIOLAR TORTUOSITY head during shaking of the thorax. These factors The progressive autosomal dominant inherited may contribute to the susceptibility of infants to syndrome of retinal arteriolar tortuosity may be acceleration-deceleration forces and more severe complicated by spontaneous retinal haemorr- increases in retinal venous pressure.50 Commonly, hages.58 The retinal arteriolar tortuousity is often however, there are no external signs of head subtly increased during adolescence and affects injury, but bilateral infantile subdural haematomas small retinal arterioles, mainly in the macular area. and bilateral retinal haemorrhages are present.46 It is easily overlooked, but even when haemorr- The latter are seen particularly in the posterior hages affect the fovea spontaneous clearing with interhemispheric fissure, presumably as a result of recovery of normal vision is the rule. tearing of the immature and fragile bridging veins. In shaken infants, retinal haemorrhages typically extend through all the layers of the retina and into MISCELLANEOUS CAUSES AND PREDISPOSING the subretinal space. In Purtscher's and haemor- FACTORS rhagic Valsalva's retinopathy, exudates and super- Retinal diseases ficial haemorrhages, respectively, are seen.30 Coats' disease (telangiectasis of retinal vessels), Eale's disease (retinal periphlebitis), radiation retinopathy, juvenile mellitus, hyperten- sion, and high predispose to retinal or BLOOD DYSCRASIAS

vitreous haemorrhage. http://adc.bmj.com/ Blood dyscrasias with anaemia, pancytopenia, and coagulopathy may give rise to retinal haemorr- hages-for example, in leukaemia and sickle cell Blood hyperviscosity disease. Retinal haemorrhages occur in patients with Retinal haemorrhages occur in both acute (more hyperviscosity of the blood such as in cystic fibro- common)5' and chronic leukaemia, but their pre- sis,59 macroglobulinaemia,60 cryoglobulinaemia,61 sence does not necessarily imply a grave progno- and paraproteinaemia.6' The mechanism by which sis.52 The retinopathy is characterised by multiple these scattered retinal haemorrhages develop is not on September 24, 2021 by guest. Protected copyright. intraretinal haemorrhages, subhyaloid haemorr- has been found in hages, Roth's spots, exudates, and abnormalities clear. In cystic fibrosis it of retinal vasculature. patients with moderate to severe pulmonary In sickle cell disease retinal haemorrhages are disease.59 the result of extravasation of blood through the danmged arteriolar walls after transient arteriolar occlusions and subsequent reperfusions.53 Prereti- Carotid-cavernous fistula nal or intraretinal haemorrhages may evolve into Especially when traumatic in origin, this is consid- schisis cavities. erably less common in infancy than adults.62 The retinal fistula produces haemodynamic changes resulting in lowered arterial pressure and raised venous pressure in the eye and orbit. The resultant reduc- HYPOTENSION AND ANOXIA tion in perfusion pressure for a long time, with Sudden reduction of systemic blood pressure ischaemia, results in widespread intraretinal and because of cardiac decompensation, or traumatic superficial haemorrhages.63 shock, or treatment for arterial hypertension54 may cause retinal and optic nerve ischaemia and haemorrhages. Sudden surgical lowering of Other predisposing factors intraocular pressure (for example, in fil- These include drugs (for example, aspirin and tering surgery) in the presence of a healthy retinal anticoagulants) and hypercapnia. vessel seldom produces retinal bleeding, but ano- xia together with increased transmural pressure is likely to produce bleeding-for example, after Conclusion difficult breech deliveries or where the cord is There are many causes of retinal haemorrhages in strangulating the neck at birth.55 infancy, and there are several causative mechan- 1372 Kaur, Taylor

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