Retinal Detachment

Retinal detachment is the separation of the neurosensory retina from the retinal pigmented epithelium and choroid. Rhegmatogenous retinal detachment, the most common type, stems from a break in the retina, allowing fluid to accumulate in the subretinal space. In the setting of an intact retina, detachment occurs when the vitreous pulls on the retina (traction) or when an underlying condition leads to increased leakage of fluid (exudative). Symptoms of photopsia, floaters, and visual defects can present over hours or gradually over weeks. Retinal detachment with visual loss is an emergency. Once macular detachment occurs, visual prognosis is poor. Symptomatic rhegmatogenous retinal detachment with intact central acuity warrants urgent surgery. For non-rhegmatogenous retinal detachments, treatment is directed toward the primary process.

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Overview

Definition

Retinal detachment is the separation of the retina from the underlying retinal pigment epithelium and choroid.

Anatomy

Retina:

  • Innermost layer, containing photoreceptors that convert light stimuli into nervous impulses
    • Rods: black-and-white vision (function in dim light)
    • Cones: color vision (function in bright light)
  • Optic retina
    • Neural or neurosensory layer 
      • Contains the photoreceptor cells 
      • Ends anteriorly along the ora serrata
    • Outer pigmented cell layer or retinal pigment epithelium (RPE) 
      • Attached to the choroid
      • Single layer of melanin-containing cells that absorb light and reduce the light scatter within the eye
  • Non-visual retina: anterior continuation of pigment cell layer over the ciliary body and posterior surface of the iris 
  • Macula 
    • Center of the retina 
    • Contains the fovea, which holds the highest density of cone cells
Eye anatomy

Anatomy of the human eye

Image by Lecturio.
Components uvea and general structure of the eye

Left: the components of the innermost, neural, and sensory layers of the eye
Right: general structure of the eye

Image by Lecturio.

Epidemiology

  • Incidence: 1 in 10,000
  • Men > women
  • Increased risk in Southeast Asians (related to higher risk of myopia)

Categories and Risk Factors

Rhegmatogenous retinal detachment

  • Most common
  • A tear, break, or hole occurs in the retina.
  • Risk factors:
    • Posterior vitreous detachment 
    • Lattice degeneration or abnormal thinning of the retina
    • Pathologic myopia
    • Enclosed ora bays
    • Meridional folds
    • Cataract surgery
    • Trauma
    • Previous retinal detachment of the other eye
    • Marfan’s syndrome
    • Cytomegalovirus (CMV) retinitis
    • Retinoschisis
    • Family history of retinal detachment

Non-rhegmatogenous retinal detachment

  • Does not include a break in the neurosensory retina
  • Types:
    • Traction retinal detachment: involves proliferative membranes on the retina or vitreous
    • Exudative (serous) retinal detachment: related to fluid entering the subretinal space
  • Risk factors for traction retinal detachment:
    • Proliferative vitreoretinopathy
    • Proliferative diabetic retinopathy (diabetic complications)
    • Retinopathy of prematurity
    • Penetrating eye injury
    • Sickling hemoglobinopathies (sickle cell anemia) 
    • Retinal vein occlusion
    • Trauma
  • Risk factors for exudative retinal detachment:
    • Tumors:
      • Primary ocular tumors
      • Ocular metastases
    • Ocular diseases:
      • Sympathetic ophthalmia
      • Central serous chorioretinopathy
      • Polypoidal choroidal vasculopathy
      • Optic nerve pit
      • Acute retinal necrosis
      • Coats’ disease
    • Systemic diseases or conditions:
      • Sarcoidosis
      • Syphilis
      • Toxoplasmosis
      • Tuberculosis
      • Corticosteroid therapy
      • Vogt-Koyanagi-Harada syndrome
      • Pre-eclampsia, eclampsia, uncontrolled hypertension
      • Organ transplantation

Pathophysiology

Precursors of retinal detachment

  • Posterior vitreous detachment
    • The vitreous slowly shrinks (vitreous degeneration) and pulls on fibrils connecting the vitreous to the retina.
    • More common in patients > 70 years
    • Also occurs in myopia, ocular trauma, and ocular inflammation
    • Leads to:
      • Rhegmatogenous detachment by causing a retinal tear or hole
      • Traction detachment when vitreous is strongly attached to the retina
  • Retinal breaks
    • Discontinuity in the neurosensory retina
    • Presents as a retinal hole (round) or tear (horseshoe-shaped)
    • Allows liquid vitreous fluid to pass through and accumulate in the subretinal space
    • Associated with rhegmatogenous retinal detachment
Retinal detachment

The image shows a retinal break (tear) where fluid can enter the subretinal space.

Image by Lecturio.

Rhegmatogenous retinal detachment

  • Retinal holes or tears allow fluid to enter the subretinal space and separate the neural retina from the RPE.
  • Detachment can occur over hours to months.

Non-rhegmatogenous retinal detachment

  • Tractional retinal detachment:
    • Proliferative membranes pull on the neurosensory retina, separating it from the retinal pigment epithelium.
    • Associated with diseases leading to neovascularization (e.g., diabetes mellitus, retinopathy of prematurity)
  • Exudative retinal detachment:
    • Impaired outflow of fluid from the vitreous to the choroid or change in the vascular permeability in the affected region → serous or hemorrhagic fluid accumulation
    • Caused by: 
      • Hydrostatic factors (e.g., severe acute hypertension)
      • Inflammation (e.g., sarcoid uveitis)
      • Large lesion or neoplasm causing effusions

Sequela of retinal detachment

  • Separation of the retina from the choroid → loss of blood supply → ischemia → rapid and progressive photoreceptor degeneration 
  • When central retina is involved → loss of vision

Clinical Presentation

Initial symptoms

  • Photopsia:
    • Flashes of light, sometimes associated with eye movement
    • Effect of mechanical depolarization of the axons in the nerve fiber layer of the retina, brought about by vitreoretinal traction
    • Not commonly seen in exudative retinal detachment
  • Floaters:
    • When the retina tears, RPE cells and blood go to the vitreous cavity → “floaters”
    • When vitreous detaches from the annular ring around the optic nerve → large circular floater or Weiss ring 
    • Retinal separation disrupts a blood vessel and leads to vitreous hemorrhage → “shower of black spots”
    • After the black spots appear, irregular clots form → “cobwebs”

Progression to retinal detachment

  • Onset:
    • Small retinal tears: progress slowly (weeks to months)
    • Large tears: progress over days
    • Non-rhegmatogenous detachment: takes weeks to months
  • Localized or peripheral detachment:
    • Usually starts in the periphery
    • Curtain-like or dim-shadow visual field defect 
  • Extensive detachment or macular detachment:
    • Sudden painless loss of vision (“descending curtain”): an emergency!
    • Detachment involving the macula: ↑ risk of permanent visual loss 
    • Relative afferent pupillary defect (RAPD); indicative of death of the photoreceptors
Table: Overview of symptoms and their causes
SymptomCause
PhotopsiaVitreoretinal traction
FloatersVitreous cells, blood
Visual field loss or defectPeripheral detachment
Blurred vision or loss of central visionMacular detachment

Diagnosis

Initial assessment

  • History
  • Visual acuity test
  • Confrontational visual field test, which helps localize the lesion
  • Pupillary reaction (check for RAPD)

Ophthalmologic examination

  • Assess both eyes: retinal detachment increases risk in the other eye
  • Dilated funduscopic examination and slit-lamp biomicroscopy 
    • Fundoscopy: retinal tears and/or obvious detachment seen as gray, elevated retina that undulate
    • Anterior segment examination: may show pigmented vitreous cells or “tobacco dust” (free-floating retinal pigment epithelial cells)
  • Ophthalmic B-scan ultrasound: 
    • Use in cases of non-diagnostic ophthalmologic exam
    • Detects detachment in dense vitreous hemorrhage (as this prevents a good fundoscopic view of the retina)

Management

Procedures

  • Laser (photocoagulation) retinopexy: use of laser to induce greater chorioretinal adhesion
  • Cryoretinopexy: cryotherapy applied to produce scarring and retinal adhesion 
  • Pneumatic retinopexy: in-office procedure utilizing cryoretinopexy and injection of intravitreal gas bubble to tamponade the affected area
  • Scleral buckle:
    • Pliable silicone element sutured to the sclera to produce buckling (inward scleral indentation)
    • Brings the detached retina in closer apposition to the eye wall, reduces internal vitreo-retinal traction, and closes the retinal breaks.
  • Vitrectomy: removal of vitreous and use of silicone oil or air bubble to replace the vitreous

Management considerations

  • Prevention:
    • Protective eyewear, especially in contact sports
    • Early diagnosis of posterior vitreous detachment and, when indicated, performance of vitrectomy
    • Treatment of retinal tears or holes to prevent retinal detachment: laser retinopexy or cryoretinopexy
  • Macular detachment: indicates poor visual prognosis 
  • Complications of retinal detachment repair:
    • Proliferative retinopathy: most common cause of repair failure
    • Cataract formation in vitrectomy

Treatment of rhegmatogenous retinal detachment

  • Symptomatic: 
    • For those with preserved central vision acuity: Perform treatment within 24 hours (to prevent progression to macular detachment).
    • Small detachment: laser retinopexy or cryoretinopexy
    • Options for large detachment:
      • Pneumatic retinopexy
      • Scleral buckle placement
      • Vitrectomy
  • Asymptomatic:
    • Small lesions: consider laser or cryoretinopexy
    • Options for large lesions:
      • Laser retinopexy or cryoretinopexy
      • Scleral buckling
      • Vitrectomy
Scleral buckle

The image illustrates scleral buckling, the placement of a pliable silicone sutured to the sclera. Scleral buckling pushes the sclera inward, thereby keeping the separated structures closer and allowing the retina to reattach.

Image by Lecturio.

Treatment of non-rhegmatogenous retinal detachment

  • Tractional:
    • Treat underlying disease (e.g., diabetic retinopathy)
    • Removal of tractional forces by vitrectomy (may be combined with scleral buckling)
  • Exudative:
    • Treat underlying disease process.
    • Malignancies may require enucleation.

Differential Diagnosis

  • Central retinal artery occlusion: occlusion of the central retinal artery, the main blood supply of the optic nerve, by an embolus or an atheroma. Patients usually present with sudden, painless, monocular visual loss. Funduscopic findings include retinal whitening with a “cherry-red” spot. Central retinal artery occlusion is a medical emergency and visual recovery is dependent on immediate evaluation and treatment.
  • Retinoschisis: splitting of the retina (usually in the outer plexiform layer) with development of 2 major layers. The congenital form (juvenile X-linked recessive type) affects men more than women, while in acquired or senile retinoschisis, both men and women aged > 50 years are affected. Reduced visual acuity is the usual presentation of congenital retinoschisis. Patients with the acquired condition are usually asymptomatic. Can lead to retinal detachment, which is surgically treated.
  • Choroidal effusion: abnormal fluid accumulation in the suprachoroidal space (between the choroid and sclera). Choroidal effusion can result from glaucoma surgery or from trauma, infections, and neoplasms. Patients can be asymptomatic when effusion is small; however, refractive changes, decreased vision, and pain occur in larger effusions. Ophthalmic ultrasonography helps differentiate retinal detachment from choroidal effusion. Depending on the underlying pathology, management ranges from observation to surgical drainage.
  • Choroidal mass: can result from a benign nevus to malignant lesions such as melanomas. The choroid has abundant vascular supply, making it a common site of metastasis of neoplasms. Presentation can range from asymptomatic cases to blurry vision. Ultrasonography helps in differentiating the various eye lesions. Treatment is directed toward the etiology of the mass.
  • Suprachoroidal hemorrhage: bleeding between the choroid and sclera. The condition is rare, but can result from intraocular surgery. Risk factors include diabetes, uncontrolled hypertension, and anticoagulation or antiplatelet medications. Suprachoroidal hemorrhage can occur intraoperatively or postoperatively. Symptoms include headache, nausea, eye pain, and decreased visual acuity. Ultrasonography detects the extent and location of the bleeding. Management is aimed at reducing intraocular pressure with control of the pain and bleeding.

References

  1. Arroyo, J., Gardiner, M. Givens, J. (2020) Retinal detachment. UpToDate. Retrieved 5 Oct 2020, from https://www.uptodate.com/contents/retinal-detachment?search=retinal%20detachment&source=search_result&selectedTitle=1~144&usage_type=default&display_rank=1#H11
  2. Blair, K., Czyz, C. (2020) Retinal detachment. StatPearls. https://www.ncbi.nlm.nih.gov/books/NBK551502/#_article-28444_s8_
  3. Gariano, R., Kim, C. (2004) Evaluation and Mmment. Medscape. Retrieved 7 Oct 2020, from https://emedicine.medscape.com/article/798501-overview

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