Ординатура / Офтальмология / Английские материалы / Moorfields Manual of Ophthalmology_Jackson_2007

Vitreous Haemorrhage

Background Vitreous haemorrhage is a sign, not a diagnosis, so it is important to determine the cause.

History Ask about floaters, photopsia, duration of reduced acuity, diabetes, sickle cell disease, cardiovascular risk factors (risk of vein occlusion), anticoagulants, eye or head trauma, headache, and previous eye disease.

Examination Note VA, rubeosis, anterior chamber red blood cells, IOP, whether phakic, pseudophakic, or aphakic, posterior vitreous detachment (PVD), haemorrhage location and density. Fundoscopy may be easier using a 28 D lens (or equivalent). Dilate and examine the fellow eye, as this may assist diagnosis, e.g. diabetic retinopathy, age-related macular degeneration (AMD).

Differential diagnosis Consider PVD, retinal tears, neovascular AMD with break-through bleeding, neovascularization from central or branch retinal vein occlusion, sickle cell retinopathy, and trauma. Intracranial haemorrhage may be associated with retinal or vitreous haemorrhage (Terson’s syndrome), particularly in patients with raised intracranial pressure and coma. Vitreous haemorrhage alone seldom produces an RAPD or NPL.

Investigations Request B-scan ultrasound if the fundal view is poor. Look specifically for retinal breaks, retinal or vitreous detachment, and macular elevation (disciform lesions).

Management For PVD see page 521, retinal tears page 526, nonclearing diabetic vitreous haemorrhage page 534, vein

occlusion page 471, AMD page 454, sickle cell retinopathy page 487, and trauma page 551.

Follow-up In those not undergoing vitrectomy to clear the haemorrhage, repeat the ultrasound examination at 2 weeks then at intervals until the fundal view improves. The interval depends on the cause – PVD related haemorrhage carries a high risk of retinal tears and requires frequent scans (≈2 weekly), whereas diabetic vitreous haemorrhage is less likely to be associated with rhegmatogenous retinal detachment. Watch for raised IOP and ghost cell glaucoma. Haemorrhage tends to clear more quickly in those with anterior chamber red blood cells, aphakia, and previous vitrectomy. Haemorrhage from AMD and central retinal vein occlusion carry a poor prognosis.

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Uveal effusion syndrome

Uveal Effusion Syndrome

Background Idiopathic choroidal effusions secondary to reduced trans-scleral outflow, typically in middle-aged men. Hypermetropic or nanophthalmic eyes are predisposed.

Clinical features Spontaneous onset of choroidal effusions (Fig. 11.10) with shifting subretinal fluid, often with a chronic relapsing–remitting course. IOP is normal and there is no vitritis. Macular involvement may reduce VA.

Differential diagnosis

■Rhegmatogenous retinal detachment : look for retinal breaks and an absence of shifting fluid.

■Serous retinal detachment : shifting subretinal fluid may occur in uveal effusion syndrome but look for signs suggesting other causes, e.g. scleritis, retinal vasculitis, vitritis, or disc swelling.

■Ocular hypotony : choroidal effusions settle as the IOP normalizes.

■Choroidal mass : visible on examination or with ultrasound.

Investigation Look for scleritis and choroidal mass using B-scan ultrasound. Measure axial length and refraction. Fluorescein angiography may rule out other diseases and has

been reported to show choroidal leakage that is also seen with indocyanine green.

Treatment There are reports of improvement with NSAIDs but this may be coincidental. Subscleral sclerectomy is often effective.

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Hereditary vitreoretinal degenerations

Hereditary Vitreoretinal

Degenerations

If a hereditary degeneration is suspected, ask to examine the relatives. Genetic testing is available for the conditions listed below.

Stickler’s syndrome

An autosomal dominant vitreoretinal degeneration, also known as hereditary progressive arthro-ophthalmopathy. Caused by a mutation of the gene coding type II or XI collagen, and classified as type 1 or 2, respectively. Associated with abnormalities of the ears (sensorineural and conductive deafness), face (cleft palate, flat midface), joints (premature arthropathy, laxity), and bones (spondyloepiphyseal dysplasia). Ocular involvement includes congenital high myopia (average −5.00 D), angle anomalies and glaucoma, presenile wedge-shaped cataracts, and congenital vitreous abnormalities (type 1 membranous, or type 2 beaded condensations). Patients are at very high risk of retinal detachment, irrespective of whether they exhibit typical paravascular pigmented lattice (Fig. 11.11). Some, but not all clinicians arrange regular review so that prophylactic retinopexy can be applied as required.

Familial exudative vitreoretinopathy

Familial exudative vitreoretinopathy (FEVR) is usually autosomal dominant (chromosome 11), but occasionally X-linked recessive and sporadic. It produces a fundus appearance similar to retinopathy of prematurity (Fig. 11.12). Three stages are based on the severity of the retinal features: (1) peripheral nonperfusion, especially temporally; (2) localized tractional retinal detachment, exudation, and neovascularization, and (3) more extensive tractional, exudative and rhegmatogenous detachments. Fluorescein angiography shows areas of nonperfusion and leakage. Most cases are mild but often progressive, so observation is required. Some require retinal photocoagulation to reduce the ischaemic stimulus, or retinal detachment surgery.

Hereditary vitreoretinal degenerations

Fig. 11.13: Juvenile X-linked retinoschisis.

Juvenile X-linked retinoschisis

An X-linked retinal dystrophy with secondary vitreous changes and variable phenotypic expression. Caused by a defect of the gene coding a protein (retinoschisin) thought to influence cellular adhesion. Presents in childhood with reduced VA and cystic foveal cavities that coalesce to produce schisis of the nerve fibre layer (Fig. 11.13). Peripheral retinoschisis may also occur, as may neovascularization, vitreous haemorrhage and retinal detachment. ERG shows reduced B wave amplitude, and OCT shows schitic separation of the retinal layers, sometimes in more than one plane. Treatment may include amblyopia therapy, panretinal photocoagulation for new vessels, and retinal detachment surgery.2

Marfan’s Syndrome

Background An autosomal dominant connective tissue disorder.

Systemic features These include tall, thin stature, long limbs and fingers (arachnodactyly), joint laxity, scoliosis, pectus excavatum, high arched palate, aortic dilatation, mitral valve prolapse, and hernias.

Ocular features Look for myopia, corneal abnormalities (including keratoconus), angle anomalies, ectopia lentis (Fig. 11.14), and retinal detachment.

Differential diagnosis of ectopia lentis:

■Trauma.

■Homocystinuria : an inborn error of metabolism with marfanoid appearance, fair complexion, osteoporosis, mental deficiency, circulatory insufficiency and thromboembolic events (sometimes precipitated by general anaesthesia).

Heterozygotes have hyperhomocysteinaemia with an increased risk of premature (age <50 years) retinal vein, and retinal artery occlusion.

■Weil-Marchesani syndrome : short stature, reduced joint mobility, myopia, microsperophakia, and pupil block from displaced lenses.

■Rare causes : sulphite oxidase deficiency, hyperlysinaemia, Ehlers-Danlos syndrome, pseudoexfoliation syndrome, high myopia, congenital glaucoma, retinitis pigmentosa, aniridia, hypermature cataract, and intraocular tumour.

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Marfan’s syndrome

Investigations Genetic testing of the affected fibrillin gene is available.

Management Arrange genetic counselling with an experienced clinician and cardiology review. Lens instability may lead to prolapse into the anterior chamber with pupil block, elevated IOP, and corneal decompensation requiring emergency lens removal. Zonular deficiency makes routine cataract surgery difficult, so consider vitreolensectomy with an anterior chamber or sutured posterior chamber IOL, or aphakia and contact lens correction.

Posterior Segment Trauma

Blunt or penetrating ocular trauma can produce various injuries due to ocular penetration, tissue compression, shearing, and tensile strain. If the injury results from an alleged assault or workplace injury, make detailed notes documenting the timing and circumstances of injury: measure or preferably photograph all injuries, and consider an orbital X-ray. Label injuries correctly: cut (clean edges); laceration (ragged wound); penetration (fullthickness defect); perforation (entry and exit wound). Consider tetanus prophylaxis and extraocular injury. For eyelid injury see page 50; cornea, page 205; angle recession, page 313; uveitis, page 353 and optic neuropathy, page 661. Exclude the following posterior segment injuries.

Commotio retinae

Photoreceptor damage produces a white retinal sheen immediately after injury (Fig. 11.15). VA is unaffected unless the macula is involved. No treatment is required. Provide a retinal detachment warning (p. 522) and review in 1–2 weeks.

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Posterior segment trauma

Retinal breaks

More common in the inferotemporal and superonasal quadrants. Look for Shafer’s sign (p. 521). Some traumatic breaks do not require retinopexy as the healing response produces a chorioretinal adhesion. Provide a retinal detachment warning and review in 1 week. Head injury without direct ocular trauma seldom produces retinal breaks.

Retinal dialysis

These very peripheral retinal tears may progress to retinal detachment and are easily missed. Perform indented fundoscopy or three-mirror examination unless penetrating injury is suspected. Retinal detachment surgery is usually required (cryobuckle) if retinal detachment is present, so keep nil by mouth and arrange same-day vitreoretinal review (Fig. 11.16).