Uveitis-Glaucoma-Hyphema Syndrome

From EyeWiki



Disease Entity

  • Glaucoma secondary to other eye disorders H40.51X (right eye), H40.52X (left eye) H40.53X (bilateral)
  • Mechanical complication of IOL implant T85.22X (displaced) or T85.23X (other)
  • Lens-induced iridocyclitis H20.21 (right eye), H20.22 (left eye), H20.23 (bilateral)

Disease

Uveitis-Glaucoma-Hyphema (UGH) Syndrome is a complication of intraocular chafing from intraocular lens (IOL) implants leading to a spectrum of iris transillumination defects and pigmentary dispersion to microhyphemas and hyphemas with elevated intraocular pressure (IOP). It is most commonly caused by chaffing from anterior chamber intraocular lenses, but can occur from any type of pseudophakic lens. It is characterized by chronic inflammation, cystoid macular edema (CME), secondary iris neovascularization, recurrent hyphemas, and glaucomatous optic neuropathy leading to a loss of vision. Surgical intervention is often required as definitive treatment.[1]

History

The term UGH Syndrome was first coined by Ellington in 1978. He noticed that certain styles of anterior chamber intraocular lenses had warped footplates leading to a rocking motion of the lens and mechanical irritation of adjacent anterior chamber angle structures. The incidence of UGH has sharply declined due to refinement of lens design, IOL fabrication, IOL material, surgical techniques, and increased use of posterior chamber IOLs. As these factors changed, studies showed incidence of UGH syndrome decrease from a mean of 2.2 to 3% to 0.4 to 1.2% depending on the IOL type over a one year period.[2]

Etiology

The disease is the result of mechanical irritation of anterior segment structures from an intraocular lens. This can also occur from cosmetic iris implants.[3]

Epidemiology

This entity can occur in any age group with an intraocular lens. While most commonly in elderly adults, it has been reported in the pediatric population.[4] Evidence of UGH occurring within 6 months of intraocular lens implant is consistently higher in anterior chamber lenses than in iris plane lenses than in posterior chamber lenses.[2] Single-piece acrylic IOLs placed within the sulcus tend to have a high UGH complication rate.[5]

General Pathology

Anterior chamber bleeding can be due to by peripupillary contact of iris with lens optic posts/haptics, and warpage of foot plates or edge imperfections leading to mechanical irritation and erosion of uveal structures including the iridocorneal angle, iris, and ciliary body. This chaffing can lead to a breakdown of the blood-aqueous barrier and subsquent release of pigment, red blood cells, protein, and white blood cells into the anterior chamber.[2] Release of red blood cells cause a microhyphema or hyphema. The release of protein and white blood cells. With pigment, red blood cells, and white blood cells in the anterior chamber the trabecular meshwork can become blocked causing an increase in intraocular pressure. Additionally contact with angle structures by the IOL can cause destruction of outflow structures and increased IOP. Electron microscopy of explanted IOLs often reveals melanosomes on the IOL, likely from damaged iris pigment epithelial cells.[6]

Prevention

Prevention of UGH syndrome is the main reason for the decreased incidence of the disease. During routine cataract surgery a 1 piece lens and haptics should be placed within the lens capsule. If this is not possible due to intraoperative complications, then there are other lens types and techniques that should be undertaken to prevent uveal disruption from the intraocular lens. A 1 piece lens should never be placed in the sulcus.[5] If there is enough support a 3-piece lens should be placed within the sulcus. Additionally, reverse optic capture of the lens can be attempted by placing the edges under the anterior capsulorhexis.[5] It is important to place the 3 piece lens in the correct orientation within the sulcus as the lens is vaulted. If there is inadequate capsular support then a 3 piece sulcus lens can be fixated via scleral fixation. If an ACIOL is required then the correct size of lens should be chosen. The general size for ACIOL placement is horizontal corneal white-to-white distance plus 1mm.[5] An incorrectly sized ACIOL can result in UGH syndrome via tilting and haptic chaffing on anterior segment structures. The ACIOL is also vaulted and must be placed in the correct orientation. There is increased risk of corneal decompensation with an ACIOL which can create further complications.[7]

Diagnosis

Dislocated IOL with haptic out of the capsular bag and in the anterior chamber. No obvious hyphema or hypopyon is seen.

The diagnosis of UGH Syndrome is clinical, based on history, physical exam, and supplemented by imaging.

Symptoms

Patients often present with intermittent decreased or blurred vision, intermittent white-out of vision, photophobia, redness, and ocular pain in the involved eye. The patient’s ocular discomfort may be out of proportion to ocular findings.

Signs

Same dislocated IOL with haptic causing transillumination defect in the iris from mechanical chaffing.

Examination can reveal a variety of physical exam findings. The ophthalmologist should look for the following signs on slit lamp microscopy: raised IOP, microhyphema or hyphema, anterior chamber cell and flare or hypopyon, iris neovascularization, iris-lens contact, iris transillumination defects, dislocated or malpositioned IOL, misplaced haptic, vitreous hemorrhage if the posterior capsule is not intact, and or CME. Examples of these can be seen in the figures to the right. Additionally, gonioscopy should be performed to look for blood within the angle, increased pigmentation of the trabecular meshwork, signs of mechanical erosion. Gonioscopy can also be used to aid in haptic location that may be the cause of anterior chamber structure erosion.

Diagnostic procedures

Ultrasound biomicroscopy (UBM) is often used in the diagnosis of UGH syndrome to aid in the visualization of malpositioned IOLs and their contact with uveal tissue. The UBM probe should be used to confirm the position of haptics and optics and their relationships to surrounding ocular structures. This can help the clinician in reconfirming the clinical suspicion of an UGH syndrome and aid in treatment decision-making.[6] Additionally, ocular coherence tomography (OCT) can aid in the guidance of diagnosing CME; however, it is not needed to diagnose UGH itself, only associated ifnlammatory response.

Management

Treatment

IOL repositioning, explantation and/or exchange successfully resolves recurrent episodes of hyphema or uveitis from UGH syndrome.[6] However, medical treatment of underlying signs/symptoms should be undertaken until definitive surgery can be undertaken:

  • Uveitis: topical corticosteroids for control of anterior inflammation.[8]
  • Intraocular Hypertension: IOP lowering topical and systemic medications such as prostaglandin analogues, beta adrenergic antagonists, alpha adrenergic agonists, and carbonic anhydrase inhibitors.[8] Parasympathomimetic agents, such as Pilocarpine, should be avoided due to its miotic effects and possible increase in mechanical chaffing to the iris.[6]
  • Hyphema: limited activity, head elevation, cycloplegics for ciliary spasm or photophobia, and topical corticosteroids for associated inflammation.[9]

Additional Resources

References

  1. Foroozan R. Tabas JG, Moster ML. Recurrent microhyphema despite intracapsular fixation of a posterior chamber intraocular lens. J Cataract Refract Surg 2003;29:1632-5.
  2. 2.0 2.1 2.2 Apple DJ, Mamlis N, Loftfield K, Googe JM, Novak LC, Kavka-van Norman D, Brady SE, Olson RJ. Complications of Intraocular Lenses. A Historical and Histopathological Review. Surv Ophthalmol 1984;29:1-54
  3. Arthur SN, Wright MM, Krammarevsky N, Kaufman SC, Grajewski AL. Uveitis-Glucoma-Hyphema Syndrome and Corneal Decompensation in Association with Cosmetic Iris Implants. Am J Ophthalmology 2009;148:790-3.
  4. Lin CJ, Tan CY, Lin SY, Jou JR. Uveitis-glaucoma-hyphema syndrome caused by posterior chamber intraocular lens—a rare complication in pediatric cataract surgery. Ann Ophthalmology 2008;40(3-4):183-4.
  5. 5.0 5.1 5.2 5.3 Chang DF, Masket S, Miller KM, Braga-Mele R, Little BC, Mamalis N, Oetting TA, Packer M. Complications of sulcus placement of single-piece acrylic intraocular lenses. Recommendations for backup IOL implantation following Posterior Capsule Rupture. J Cataract Refract Surg 2009;35:1445-58.
  6. 6.0 6.1 6.2 6.3 Piette S, Canlas O, Tran HV, Ishikawa H, Liebmann JM, Ritch R. Ultrasound Biomicroscopy in Uveitis-glaucoma-hyphema Syndrome. Am J Ophthalmology 2002;133:839-41.
  7. Snellingen T, Shrestha JK, Huq F, Husain R, Koirala S, Rao GN, Pokhrel RP, Kolstad A, Upadhyay MP, Apple DJ, Arnesen E, Cheng H, Olsen EG, Vogel M. South Asian Cataract Management Study: complications, vision outcomes, and corneal endothelial cell loss in a randomized, multi-center clinical trial comparing intracapsular cataract extraction with and without anterior chamber intraocular lens implantation. Ophthalmology 2000;107:231–240
  8. 8.0 8.1 Eliassi-Rad B. Uveitic Glaucoma. EyeWiki. http://eyewiki.aao.org/Cataract. Accessed December 6, 2015.
  9. Oldham GW. Hyphema. EyeWiki. http://eyewiki.aao.org/Cataract. Accessed December 6, 2015.