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LENS-INDUCED UVEITIS



        ROHIT.UDAYA.PRASAD

                      3224
Lens-Induced Uveitis-
• Phacoanaphylactic Uveitis -
• It is an immunological response to lens proteins in
  the sensitized eyes presenting as severe
  granulomatous anterior uveitis .

• Phacotoxic Uveitis –
• It is an ill understood entity. This term is used to
  describe mild iridocyclitis associated with the
  presence of lens matter in the anterior chamber
  either following trauma or extra-capsular extraction
  or leak from hyper-mature cataracts
• INTRODUCTION –
• Lens-induced uveitis occurs in the setting of a ruptured or
  degenerative lens capsule and is characterized by a
  granulomatous antigenic reaction to lens protein. Before the
  modern era of microsurgery, this disease was more
  common, and the diagnosis was often made histologically, as
  eyes with phacoanaphylaxis were often enucleated for
  intractable inflammation and secondary glaucoma.

• While lens fragments may be retained in the anterior or
  posterior chamber during seemingly uncomplicated cataract
  surgery, they also may be dislocated posteriorly into the
  vitreous cavity during phacoemulsification of the
  nucleus, usually after zonular dehiscence or posterior capsule
  rupture. Lens-induced uveitis may develop, and the degree of
  intraocular inflammation in these patients often is governed
  by the size of the retained lens fragment, the time since
  cataract surgery, the patient's individual inflammatory
  response, and the extent of other intraocular manipulations.
• Pathogenesis -
• The term phacoanaphylaxis is probably inappropriate because
  no evidence exists of a classic type I immunoglobulin E (IgE)
  mediated anaphylactic reaction. The immunopathogenesis of
  lens-induced uveitis is believed to be the result of
  autosensitization to lens proteins. After a break in the lens
  capsule and sensitization to lens proteins, an immune
  complex–mediated phenomenon develops, which can be
  transferred by hyperimmune serum. Type II, III, and IV
  hypersensitivity reactions may be involved in the
  pathogenesis.
• The disease most likely is induced by altered tolerance to lens
  protein and not as a result of a rejection phenomenon of
  sequestered foreign materials. The specific type of
  immunological reaction in lens-induced uveitis may vary from
  patient to patient, and it may depend on the type of surgery or
  injury, the amount of retained lens in the vitreous cavity, and
  the previous immunological status of both the patient and the
  eye.
• Age: lens-induced uveitis are more common in
  the elderly population, with a peak incidence in
  the sixth to seventh decades.
• Sex: No sexual predilection exists are more
  common in younger age groups.
• Causes:
• Lens-induced uveitis is usually the result of traumatic or
  surgical disruption of the lens capsule and liberation of
  lens proteins into the aqueous or into the vitreous cavity.
  Posterior capsular rupture during phacoemulsification is
  the most common cause of posterior displacement of
  lens fragments. This complication is more common in
  patients with pseudoexfoliation syndrome, zonular
  dehiscence, a small pupil, friable iris, and hard nuclei or
  hypermature cataracts.
• Penetrating injury of the globe may result in severe lens-
  induced uveitis. The uveitis may remain undiagnosed
  clinically because of hyphema, decreased corneal
  clarity, and inflammation related to the trauma. A small
  punctured perforation site may remain unnoticed
  initially, and severe inflammation and cataract will be
  present 1 week later.
• History-
• Lens-induced uveitis typically develops 1-14 days
  after traumatic or surgical perforation of the lens
  capsule. In rare instances, the inflammation may
  develop several months after the disruption of the
  lens capsule.
• Clinical symptoms may include severe light
  sensitivity, epiphora, pain, floaters, loss of
  vision, and redness of the eye.
• Decreased vision may be due to refractive error
  (myopic or hyperopic shift) associated with such
  factors as macular edema, hypotony, or change in
  lens position.
• Visual acuity in patients with phacoanaphylactic
  uveitis is quite variable, ranging from 20/20 to no
  light perception.
• Clinically:
• The inflammation can vary from a mild anterior uveitis to a
  fulminant endophthalmitis. Typically, the inflammation is
  unilateral and involves only the traumatized eye.
• The most important clinical signs of lens-induced uveitis are
  lid swelling, perilimbal or diffuse injection, corneal
  haze, keratic precipitates (granulomatous),cells and
  flare, fibrin in the anterior chamber (occasionally), peripheral
  anterior synechiae, posterior synechiae, pupillary
  membrane, and iris nodules.
• In the posterior segment, lens fragments, inflammatory
  cells, traction bands in the vitreous, retinal
  edema, inflammatory cuffing of blood vessels, cystoid macular
  edema, and epiretinal membrane formation can be observed.
• If untreated, lens-induced uveitis/phacoanaphylactic
  endophthalmitis may result in chronic cystoid macular
  edema, cyclitic membrane formation, tractional retinal
  detachment, and phthisis bulbi.
Phacoanaphylactic reaction to penetrating injury of lens. This patient was a
25-year-old woman whose eye was penetrated with a 27-gauge needle during
an attempt to anesthetize the eyelid for chalazion removal. One week
later, a marked uveitis was present. Notice posterior synechiae.
• Typical appearance of retained lens fragments in posterior vitreous
  cavity. Lens material is a whitish substance that obscures fundus
  details.
• Patient with persistently elevated intraocular pressure after cataract
  surgery was found to have retained lens material and low-grade
  inflammation. Retained lens material is visible in retroillumination
  on downgaze
Typical clinical picture of retained lens material following cataract
surgery. White cortical material is easily visible in the pupillary space
• Lab Studies:

• Aqueous paracentesis in subtle or early cases
  may reveal inflammatory cells and particulate
  lens proteins without bacteria. This procedure is
  performed more efficiently at the time of
  anterior chamber washout and vitrectomy to
  remove the inciting lenticular antigens
• Imaging Studies:
• If the media opacity prevents an appropriate fundus
  examination, echography with A-scan and B-scan may be helpful when
  evaluating the posterior pole.
   ▫ Suspicion for acute endophthalmitis, intraocular foreign
      body, dropped lens nucleus, thickening of the choroid, retinal
      detachment, and choroidal effusion are all indications for
      echography if the anterior segment changes hinder examination of
      the posterior segment.
   ▫ The shape, position, and thickness of the traumatized lens; the
      presence of focal echogenic areas; and, sometimes, even the entrance
      and exit wounds are recognizable by ultrasound. It is clinically
      important to diagnose the isolated rupture of the posterior capsule of
      the lens by echography. Such ruptures are characterized by the
      irregular extension of the highly reflective posterior capsule toward
      the vitreous with significantly increased thickness of the lens.
• Ultrasound biomicroscopy (UBM) may have an important role in
  the evaluation of lens-induced uveitis after extracapsular cataract
  extraction, revealing hidden lens particles in the posterior chamber
  causing inflammation as well as lens-particles creating secondary
  glaucoma
• Medical Care:
•   Treatment may be medical or surgical. Medical therapy of phacoanaphylactic
  uveitis includes topical corticosteroids and may include cycloplegics and
  medication for elevated intraocular pressure as needed. Treatment should be
  tailored to the individual patient and adjusted according to response. Patient
  age, immune status, and tolerance for adverse effects always must be taken into
  account.
• Cycloplegics: Topical cycloplegics break or prevent the formation of posterior
  synechiae, stabilize the blood-aqueous barrier leading to reduced leakage of
  plasma proteins, increase uveoscleral outflow, and provide mild relief of ciliary
  spasm pain. The stronger the inflammatory reaction, the more frequently
  applied or stronger the cycloplegic.
• Corticosteroids: Corticosteroids block the formation of arachidonic acid from
  cell membrane precursors by inhibiting the action of phospholipase-
  A2, cyclooxygenase, and lipoxygenase. Thus, arachidonic acid is the premier
  precursor of potent inflammatory mediators, such as
  prostaglandins, thromboxane, and leukotrienes. Corticosteroids frequently are
  used in uveitis therapy. Topical steroid drops are given in dosages ranging from
  once daily to hourly. They also can be given in an ointment form. Periocular
  corticosteroids generally are given as depot-steroid injections when a more
  prolonged effect is needed or when a patient is noncompliant or poorly
  responsive to topical administration.
• Intraocular pressure–lowering agents: When phacoanaphylaxis is
  associated with high intraocular pressure ,aqueous suppressants are indicated.
  Beta-blockers, alpha-agonists, and carbonic anhydrase inhibitors are used to
  lower the pressure.
• Surgical Care:
• If persistent or uncontrolled inflammation or elevated intraocular pressure
  is not responsive to medical therapy or if such a large amount of exposed
  lens material is present that medical therapy is likely to fail, then surgical
  removal of the exposed lens material is indicated . The most common
  situation leading to this is posterior capsular rupture with the loss of lens
  fragments into the vitreous cavity during phacoemulsification . Removal of
  retained lens fragments by pars plana vitrectomy generally restores good
  visual function and reverses many complications in these patients. Surgical
  removal of retained lens material may be necessary depending upon the
  degree of inflammation, the size of the retained lens particle, and the
  presence of increased intraocular pressure. Observation is indicated when
  the lens fragments are small and the inflammation can be controlled.

• Retained lens fragments that are larger than one third to one half of the
  total cataract usually (but not always) require surgical removal.
• Several studies demonstrate no advantage to early surgery; therefore, the
  cataract surgeon may treat patients with retained lens fragments
  conservatively, and then refer the patient to a vitrectomy surgeon after an
  appropriate period of observation and medical therapy, unless the patient
  develops retinal detachment, highly elevated intraocular pressure, or some
  other condition in which posterior segment surgery is indicated more
  urgently.
• Complications:
• Cystoid macular edema:
• Secondary glaucoma
  ▫ Trabecular meshwork obstruction may occur with the
    accumulation of white blood cells (macrophages and
    activated T lymphocytes) or their aggregations. These may
    cause peripheral anterior synechiae and subsequent closed-
    angle glaucoma.
  ▫ Obstruction may arise from inflammatory debris
    (eg, proteins, fibrin, high molecular weight proteins) and
    from lens particles. These proteins increase the aqueous
    viscosity, which may contribute to increased intraocular
    pressure.
  ▫ Leakage of lens proteins through the injured lens capsule
    with or without leakage of serum proteins from uveal blood
    vessels in lens-induced uveitis may block the trabecular
    outflow causing secondary glaucoma.
• Retinal detachment
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Lens Induced Uveitis

  • 1. LENS-INDUCED UVEITIS ROHIT.UDAYA.PRASAD 3224
  • 2. Lens-Induced Uveitis- • Phacoanaphylactic Uveitis - • It is an immunological response to lens proteins in the sensitized eyes presenting as severe granulomatous anterior uveitis . • Phacotoxic Uveitis – • It is an ill understood entity. This term is used to describe mild iridocyclitis associated with the presence of lens matter in the anterior chamber either following trauma or extra-capsular extraction or leak from hyper-mature cataracts
  • 3. • INTRODUCTION – • Lens-induced uveitis occurs in the setting of a ruptured or degenerative lens capsule and is characterized by a granulomatous antigenic reaction to lens protein. Before the modern era of microsurgery, this disease was more common, and the diagnosis was often made histologically, as eyes with phacoanaphylaxis were often enucleated for intractable inflammation and secondary glaucoma. • While lens fragments may be retained in the anterior or posterior chamber during seemingly uncomplicated cataract surgery, they also may be dislocated posteriorly into the vitreous cavity during phacoemulsification of the nucleus, usually after zonular dehiscence or posterior capsule rupture. Lens-induced uveitis may develop, and the degree of intraocular inflammation in these patients often is governed by the size of the retained lens fragment, the time since cataract surgery, the patient's individual inflammatory response, and the extent of other intraocular manipulations.
  • 4. • Pathogenesis - • The term phacoanaphylaxis is probably inappropriate because no evidence exists of a classic type I immunoglobulin E (IgE) mediated anaphylactic reaction. The immunopathogenesis of lens-induced uveitis is believed to be the result of autosensitization to lens proteins. After a break in the lens capsule and sensitization to lens proteins, an immune complex–mediated phenomenon develops, which can be transferred by hyperimmune serum. Type II, III, and IV hypersensitivity reactions may be involved in the pathogenesis. • The disease most likely is induced by altered tolerance to lens protein and not as a result of a rejection phenomenon of sequestered foreign materials. The specific type of immunological reaction in lens-induced uveitis may vary from patient to patient, and it may depend on the type of surgery or injury, the amount of retained lens in the vitreous cavity, and the previous immunological status of both the patient and the eye.
  • 5. • Age: lens-induced uveitis are more common in the elderly population, with a peak incidence in the sixth to seventh decades. • Sex: No sexual predilection exists are more common in younger age groups.
  • 6. • Causes: • Lens-induced uveitis is usually the result of traumatic or surgical disruption of the lens capsule and liberation of lens proteins into the aqueous or into the vitreous cavity. Posterior capsular rupture during phacoemulsification is the most common cause of posterior displacement of lens fragments. This complication is more common in patients with pseudoexfoliation syndrome, zonular dehiscence, a small pupil, friable iris, and hard nuclei or hypermature cataracts. • Penetrating injury of the globe may result in severe lens- induced uveitis. The uveitis may remain undiagnosed clinically because of hyphema, decreased corneal clarity, and inflammation related to the trauma. A small punctured perforation site may remain unnoticed initially, and severe inflammation and cataract will be present 1 week later.
  • 7. • History- • Lens-induced uveitis typically develops 1-14 days after traumatic or surgical perforation of the lens capsule. In rare instances, the inflammation may develop several months after the disruption of the lens capsule. • Clinical symptoms may include severe light sensitivity, epiphora, pain, floaters, loss of vision, and redness of the eye. • Decreased vision may be due to refractive error (myopic or hyperopic shift) associated with such factors as macular edema, hypotony, or change in lens position. • Visual acuity in patients with phacoanaphylactic uveitis is quite variable, ranging from 20/20 to no light perception.
  • 8. • Clinically: • The inflammation can vary from a mild anterior uveitis to a fulminant endophthalmitis. Typically, the inflammation is unilateral and involves only the traumatized eye. • The most important clinical signs of lens-induced uveitis are lid swelling, perilimbal or diffuse injection, corneal haze, keratic precipitates (granulomatous),cells and flare, fibrin in the anterior chamber (occasionally), peripheral anterior synechiae, posterior synechiae, pupillary membrane, and iris nodules. • In the posterior segment, lens fragments, inflammatory cells, traction bands in the vitreous, retinal edema, inflammatory cuffing of blood vessels, cystoid macular edema, and epiretinal membrane formation can be observed. • If untreated, lens-induced uveitis/phacoanaphylactic endophthalmitis may result in chronic cystoid macular edema, cyclitic membrane formation, tractional retinal detachment, and phthisis bulbi.
  • 9. Phacoanaphylactic reaction to penetrating injury of lens. This patient was a 25-year-old woman whose eye was penetrated with a 27-gauge needle during an attempt to anesthetize the eyelid for chalazion removal. One week later, a marked uveitis was present. Notice posterior synechiae.
  • 10. • Typical appearance of retained lens fragments in posterior vitreous cavity. Lens material is a whitish substance that obscures fundus details.
  • 11. • Patient with persistently elevated intraocular pressure after cataract surgery was found to have retained lens material and low-grade inflammation. Retained lens material is visible in retroillumination on downgaze
  • 12. Typical clinical picture of retained lens material following cataract surgery. White cortical material is easily visible in the pupillary space
  • 13. • Lab Studies: • Aqueous paracentesis in subtle or early cases may reveal inflammatory cells and particulate lens proteins without bacteria. This procedure is performed more efficiently at the time of anterior chamber washout and vitrectomy to remove the inciting lenticular antigens
  • 14. • Imaging Studies: • If the media opacity prevents an appropriate fundus examination, echography with A-scan and B-scan may be helpful when evaluating the posterior pole. ▫ Suspicion for acute endophthalmitis, intraocular foreign body, dropped lens nucleus, thickening of the choroid, retinal detachment, and choroidal effusion are all indications for echography if the anterior segment changes hinder examination of the posterior segment. ▫ The shape, position, and thickness of the traumatized lens; the presence of focal echogenic areas; and, sometimes, even the entrance and exit wounds are recognizable by ultrasound. It is clinically important to diagnose the isolated rupture of the posterior capsule of the lens by echography. Such ruptures are characterized by the irregular extension of the highly reflective posterior capsule toward the vitreous with significantly increased thickness of the lens. • Ultrasound biomicroscopy (UBM) may have an important role in the evaluation of lens-induced uveitis after extracapsular cataract extraction, revealing hidden lens particles in the posterior chamber causing inflammation as well as lens-particles creating secondary glaucoma
  • 15. • Medical Care: • Treatment may be medical or surgical. Medical therapy of phacoanaphylactic uveitis includes topical corticosteroids and may include cycloplegics and medication for elevated intraocular pressure as needed. Treatment should be tailored to the individual patient and adjusted according to response. Patient age, immune status, and tolerance for adverse effects always must be taken into account. • Cycloplegics: Topical cycloplegics break or prevent the formation of posterior synechiae, stabilize the blood-aqueous barrier leading to reduced leakage of plasma proteins, increase uveoscleral outflow, and provide mild relief of ciliary spasm pain. The stronger the inflammatory reaction, the more frequently applied or stronger the cycloplegic. • Corticosteroids: Corticosteroids block the formation of arachidonic acid from cell membrane precursors by inhibiting the action of phospholipase- A2, cyclooxygenase, and lipoxygenase. Thus, arachidonic acid is the premier precursor of potent inflammatory mediators, such as prostaglandins, thromboxane, and leukotrienes. Corticosteroids frequently are used in uveitis therapy. Topical steroid drops are given in dosages ranging from once daily to hourly. They also can be given in an ointment form. Periocular corticosteroids generally are given as depot-steroid injections when a more prolonged effect is needed or when a patient is noncompliant or poorly responsive to topical administration. • Intraocular pressure–lowering agents: When phacoanaphylaxis is associated with high intraocular pressure ,aqueous suppressants are indicated. Beta-blockers, alpha-agonists, and carbonic anhydrase inhibitors are used to lower the pressure.
  • 16. • Surgical Care: • If persistent or uncontrolled inflammation or elevated intraocular pressure is not responsive to medical therapy or if such a large amount of exposed lens material is present that medical therapy is likely to fail, then surgical removal of the exposed lens material is indicated . The most common situation leading to this is posterior capsular rupture with the loss of lens fragments into the vitreous cavity during phacoemulsification . Removal of retained lens fragments by pars plana vitrectomy generally restores good visual function and reverses many complications in these patients. Surgical removal of retained lens material may be necessary depending upon the degree of inflammation, the size of the retained lens particle, and the presence of increased intraocular pressure. Observation is indicated when the lens fragments are small and the inflammation can be controlled. • Retained lens fragments that are larger than one third to one half of the total cataract usually (but not always) require surgical removal. • Several studies demonstrate no advantage to early surgery; therefore, the cataract surgeon may treat patients with retained lens fragments conservatively, and then refer the patient to a vitrectomy surgeon after an appropriate period of observation and medical therapy, unless the patient develops retinal detachment, highly elevated intraocular pressure, or some other condition in which posterior segment surgery is indicated more urgently.
  • 17. • Complications: • Cystoid macular edema: • Secondary glaucoma ▫ Trabecular meshwork obstruction may occur with the accumulation of white blood cells (macrophages and activated T lymphocytes) or their aggregations. These may cause peripheral anterior synechiae and subsequent closed- angle glaucoma. ▫ Obstruction may arise from inflammatory debris (eg, proteins, fibrin, high molecular weight proteins) and from lens particles. These proteins increase the aqueous viscosity, which may contribute to increased intraocular pressure. ▫ Leakage of lens proteins through the injured lens capsule with or without leakage of serum proteins from uveal blood vessels in lens-induced uveitis may block the trabecular outflow causing secondary glaucoma. • Retinal detachment