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Uveitis Glaucoma Hyphema Syndrome
Introduction
Uveitis glaucoma hyphema (UGH) syndrome or Ellingson syndrome is a complication of intraocular (IOL) implantation. Chafing from the implants leads to a spectrum of iris transillumination defects and hyphema with raised intraocular pressure (IOP) and pigment dispersion.[1] This condition involves 3 components that define the syndrome that include uveitis, glaucoma, and hyphema.[2] Uveitis is an uvea inflammation comprising the iris, ciliary body, and choroids.[3] Numerous conditions, such as infections, autoimmune illnesses, and trauma, can cause it.[4] Secondary problems include hyphema and uveitis that might result from elevated IOP.[5] Glaucoma is usually caused by increased IOP, leading to functional visual field loss, optic neuropathy, and retinal nerve fiber layer thinning.[6] Uveitis complications can result in secondary glaucoma, which can cause the UGH Syndrome.[7] Hyphema is an accumulation of blood in the eye's anterior chamber that may develop as a side effect of a traumatic event or as a consequence of glaucoma or uveitis.[8]
The pathophysiology involving uveitis, glaucoma, and hyphema are part of the pathogenesis of UGH syndrome.[9] The inflammation linked to uveitis may impact the drainage routes in the eye, which could result in a greater barrier to aqueous fluid outflow. Consequently, this leads to an increase in IOP. Hyphema worsens IOP since blood in the anterior chamber might restrict outflow channels and worsen inflammation.[10] UGH syndrome can also occur with the posterior chamber IOLs placed in the sulcus, causing posterior iris chafing by the loop or the optic. The syndrome occurs more commonly with planar loop design than with angulated loops.[11]
The syndrome can develop immediately or over the years, causing decreased vision in the affected eye, and may present with transient vision loss or the gradual diminution of vision. The eye may appear congested with an increase in IOP. Blood or clotted blood may be present in the inferior anterior chamber angle and anterior chamber reaction. If untreated, it may lead to glaucomatous optic neuropathy.[1][12]
A careful history, examination, and appropriate investigations can confirm the diagnosis. Ultrasound biomicroscopy is the most reliable procedure to diagnose UGH syndrome.[13] Other evaluation methods include slit-lamp examination, gonioscopy for the visualization of the angles, IOP measurement,[14] and ultrasound brightness (B) scan [15] to visualize the posterior segment in case of vitreous hemorrhage, and optical coherence tomography[16] to visualize both anterior and posterior segments.[17][18] Treatment options include IOL explantation or exchange, topical corticosteroids, and cycloplegics.[19]
Etiology
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Etiology
A multifaceted etiology underlies UGH syndrome.[1] The syndrome's symptoms include bleeding in the anterior chamber of the eye (hyphema), uveitis (inflammation of the uvea), and glaucoma due to elevated IOP. The causes of UGH syndrome are frequently associated with postoperative problems from ophthalmic surgery, specifically cataract extraction.[20] Surgery-related microtrauma to the iris and ciliary body can cause inflammation, compromising the blood-aqueous barrier and resulting in uveitis. Hyphema can also result from mechanical stress caused by the IOL, particularly if it is positioned incorrectly or makes contact with the iris. Combining these variables may raise IOP, which may lead to glaucoma.[21] Variations in the anterior portion of the eye and specific anatomical predispositions may also be linked to the disease. Management of UGH syndrome necessitates a comprehensive strategy addressing pressure and inflammation-related components. Anti-inflammatory drugs, glaucoma medications, and surgical treatments are utilized to restore ocular health.
The UGH syndrome is most commonly caused by rubbing of the iris from the anterior chamber IOL but can occur from any pseudophakic lens.[9] The literature describes warped edges of the footplate, which caused injury to the iris root by a rocking motion. This results in recurrent ocular inflammation, high IOP, and bleeding in the anterior chamber. Removal of the implant usually results in the resolution of the condition.[22] Azar 91z model ACIOL (flexible closed-loop ACIOL) implantation was associated with complications because of design and manufacturing problems, causing sharp edges, vaulting, excess movements, and irritation of ocular tissue.[23]
Iris-supported IOLs with metal loops are reported to cause UGH syndrome. The Kelman multiflex ACIOL (flexible open-loop ACIOL) has an anterior vault to avoid rubbing the iris in the correct configuration. When Kelman multiflex ACIOL is implanted incorrectly (upside-down lens syndrome), corneal edema, increased IOP, cystoid macular edema, and UGH syndrome may occur.[24][25] MA50 IOLs placed in the sulcus have been associated with complications like UGH syndrome.[26] Other iris-supported IOLs, like iris clamp lenses, are causative of UGH syndrome.[27]
Lenses with imperfect construction, imperfectly positioned lenses, or improperly sized lenses may cause chafing.[28] A few cases have been seen with the scleral fixated IOLs.[29] Among the scleral fixated lenses, the ab externo approach of hydrophobic acrylic lenses with suture burial technique and the glued scleral fixated lenses cause UGH syndrome as a complication.[30][31]
The technique of scleral fixation with friction knots using single-piece lenses showed a risk of UGH syndrome.[32] Other surgical devices, like iris implants, capsular tension rings, and glaucoma filtration devices, have been implicated as a source of mechanical irritation.[33] Among the IOLs, the condition occurs with scleral fixated lenses, single-piece acrylic IOLs implanted in the sulcus, or sulcus fixated three-piece lenses, with the implantation of a single-piece acrylic lens in the bag.[34][35]
Iris transillumination defects may be noted in the area of iris chafing. Single-piece IOL in the capsular bag may be associated with UGH syndrome in pseudoexfoliation syndrome (pseudophacodonesis due to zonular laxity resulting in chafing of the posterior iris surface and focal capsular fibrosis around the haptic causing iris touch), plateau iris syndrome (with anterior rotation of ciliary processes), and intensive facedown position in yoga with pseudophacodonesis secondary to pseudoexfoliation.[36][37]
Almost all intraocular implants may be associated with UGH syndrome, including retropupillary iris-claw IOL, iris-sutured IOL, placement of single-piece PCIOL (posterior chamber IOL), or multipiece PCIOL in the anterior chamber, express mini glaucoma shunt, and cosmetic iris implants.[38] Systemic anticoagulation may predispose to UGH syndrome.[39] An extensive series comprised of 71 patients with UGH syndrome noted that pseudophacodonesis was a risk factor for UGH syndrome. Their analysis showed that blood thinners did not increase the risk of UGH syndrome.[40]
Reports of late-onset UGH syndrome associated with the Soemmering ring cataract cause an anterior shift of sulcus-fixated, leading to iris-haptic touch.[41] Sulcus implantation of single-piece foldable acrylic PCIOL may be associated with UGH syndrome.[42][43] However, Taskapili and colleagues showed that implanting foldable acrylic PCIOL in the sulcus might have positive visual outcomes, an acceptable complication profile, and good centration.[42][44]
Epidemiology
The incidence of UGH syndrome has reduced since strict control of the quality of IOLs, refined material for manufacture, and improvement of the manufacturing techniques and lens design.[45] The iris-ciliary touch by the footplates or haptics of IOLs is believed to play a pivotal role in the syndrome's pathogenesis, including the release of inflammatory mediators. The UGH syndrome is most commonly seen in adults and children after cataract surgery.[46] The incidence of UGH syndrome has declined over the years from 2.2% to 1.2% over 1 year.[47] Using first-generation anterior chamber IOLs is commonly linked to the development of UGH syndrome, though single-piece IOL in the capsular bag can cause the syndrome. Lin and colleagues reported a child with anterior migration of the optic of the PCIOL causing UGH syndrome, which subsided after removal of the PCIOL.[48]
Pathophysiology
The UGH syndrome appears to arise from the repetitive mechanical iris or ciliary body trauma by a malpositioned or subluxated lens.[35] The exact pathology of UGH syndrome is unknown, but some hypotheses have been constructed over time. It is believed that innate immunity, cytokines, and eicosanoid synthesis are activated by mechanical trauma to the iris by the optics or haptics complement or fibrin activation by the IOL. Adherence of bacteria (avirulent bacteria- similar to that found in postoperative endophthalmitis) and leukocytes are common to the IOL surface.[49]
The surface of IOLs, especially PMMA (polymethyl methacrylate), activates the plasma-derived enzymes. The contaminants may cause toxicity on the IOL surface during manufacturing. Poorly manufactured edges, iris-claw IOLs, or rigid closed-looped lenses were more prone to cause iris chafing. Still, newer lenses have reduced risk due to better designs, fabrications, and improved surgical techniques.[50] Unauthorized, poorly manufactured copies of Choyce Mark lenses were the usual culprit rather than the properly manufactured and sized original Choyce Mark lenses.[47]
Poor finish of the edges (serrated, sharpened, or uneven), warped footplates, imperfect polishing, and finishing of injection-molded IOLs lead to mechanical excoriation of the iris, increasing the risk of UGH Syndrome.[21] The recurrent hyphema is thought to occur from recurrent mechanical trauma to the angle of the anterior chamber, the iris, or the ciliary body—disruption of the blood-aqueous barrier results in uveitis. The increased IOP may be caused by various factors, including hyphema, uveitis, pigment dispersion, direct damage to the angle of the anterior chamber, and steroid response from the steroid drops used to treat the syndrome.
The UGH syndrome was originally reported with ACIOLs. Material, implant edges, manufacturing process, and quality control are important factors influencing the occurrence of the UGH syndrome. The ACIOL should be properly sized (ACIOL diameter equals horizontal white to white diameter in mm + 1 mm).[51] Smaller ACIOLs are unstable and may rotate or tilt, causing damage to the anterior segment structures. A larger ACIOL causes damage to the angle, and the vault may touch and decompensate the corneal endothelium.
PCIOLs placed in the sulcus may irritate the posterior iris surface and cause UGH syndrome. Specifically, single-piece acrylic PCIOLs with square optic edges and thick square haptics or small uniplanar haptics may rub the posterior pigmented surface of the iris. Multi-piece PCIOL with posterior angulation (causing a posterior shift of the optic), smooth optic surface, and round edges are preferred for placement at the ciliary sulcus.[42] The position and size of the PCIOL are also crucial for placing a sulcus IOL.
Histopathology
The optic of the IOL may be covered with a thick membrane composed of inflammatory cells, fibrosis, and red blood corpuscles (RBCs). The optic may get discolored and have a brownish color due to the deposition of hemoglobin from RBCs. The inflammatory deposits are usually more severe at the margin of the optic, and the inflammatory debris may cover the whole IOL optic.[47] The debris may get deposited on the haptic or footplate as well. The inflammatory debris and RBCs may cover the whole IOL optic totally or partially, forming a cocoon membrane.[52] The junction of the optic and haptic in multipiece IOL forms a nidus for inflammatory deposits (see Image. Uveitis Glaucoma Hyphema Syndrome or Ellingson Syndrome).
History and Physical
The UGH syndrome is typically a complication of cataract surgery. The UGH syndrome may occur after uneventful ocular surgery or after an ocular surgery with intraoperative complications resulting in malposition of the implant. Patients usually present with episodes of blurred vision weeks to months after surgery. The blurry vision may be accompanied by pain, photophobia, erythropsia (apparent reddish hue of the visible objects), and eye redness. The vision is blurred, but complete vision loss is usually not seen unless accompanied by advanced glaucoma. The patient often presents with intermittent white-out of vision or intermittent decreased vision.[12] Usually, multiple acute episodes of blurred vision or pain occur in cases with UGH syndrome.
A few variations of UGH syndrome are recognized, including UGH plus and IPUGH (incomplete posterior UGH) syndrome. UGH plus includes vitreous hemorrhage, with UGH seen in anterior chamber lenses with iris support posterior chamber lenses placed in the sulcus or the capsular bag.[35] Communication between the vitreous cavity and the anterior chamber through the anterior hyaloid face, whose integrity is disrupted after surgery, occurs spontaneously or due to degenerative changes; this causes the passage of blood and gives rise to the simultaneous bleed in both the anterior chamber and the vitreous cavity. IPUGH syndrome is bleeding into the posterior chamber (usually between the IOL and the posterior capsule, similar to endocapsular hematoma or 'in-the-bag hyphema') without uveitis. Some cases of IPUGH syndrome may have glaucoma.[53]
Slit-lamp examination shows conjunctival congestion, anterior chamber cells, and the presence of microscopic hyphema. Sometimes macroscopic hyphema is visible without a slit lamp. Due to the endothelial touch by an IOL, corneal edema may be noted.[54] Keratic precipitates may be seen over the corneal endothelium. Transillumination defect in the iris may be seen in the area of repetitive uveal trauma by the implant. C- or J-shaped haptics typically create an arcuate transillumination defect. Iris transillumination defect and microhyphema syndrome have been described with the implantation of PCIOLs with elliptical polypropylene haptics containing a 10° anterior angulation' in the sulcus.[55]
Transillumination defects may also be seen in other disorders causing iris atrophy and may not be a specific sign of UGH syndrome.[40] About 5% to 15% of patients with PCIOL in the sulcus have iris-transillumination defects at the peripheral 1/2 or 2/3 of the iris due to friction from the IOL optic. Around 1% of the patients may have an IOL-induced hemorrhage.[55] Sometimes, iris neovascularization is seen. A poorly positioned optic or haptic of IOL or other implant causing visible mechanical trauma to the uveal tissue is usually observed. Other slit-lamp findings include pigments on the corneal endothelium, posterior synechia, inflammatory deposits over the optic or haptic of the IOL, and pseudophacodonesis. Blood in the angle or within the trabecular meshwork can be visualized with the help of gonioscopy. Gonioscopy can also aid in finding the haptic that may have caused the anterior chamber angle structure erosion and help visualize blood in the trabecular meshwork. Gonioscopy in dilated pupils may be used to locate the exact location of the haptics of an IOL and to identify areas of uveal touch by the implant.[56]
A slit-lamp examination may also help visualize and quantify anterior chamber cells, flare, vitreous cells, or any deposits over the IOL. PCIOL placed in the sulcus may cause reverse pupillary block characterized by posterior bowing of the iris, transillumination defects of the iris, pigment dispersion syndrome, increased pigmentation of the anterior chamber angle, increased IOP, and uveitis; this is seen in axial myopia and vitrectomized eyes.[57] In addition, fundus examination should be performed using a 90D lens. Indirect ophthalmoscopy using a 20D lens should be used to examine the peripheral retina. The optic nerve examination is paramount to identifying the retinal nerve fiber layer defects and evidence of glaucomatous damage.
Evaluation
Anterior segment photograph helps document the disease, monitor the response to therapy, and counsel the patient. Ultrasound biomicroscopy (UBM) helps diagnose UGH syndrome as it may be used to confirm the position of haptics and optics and their relationship with surrounding ocular structures.[13] UBM may also be used to visualize the malpositioned IOL and their proximity to uveal tissue. Anterior segment optical coherence tomography can be used to visualize the anterior chamber angle structures as it is non-invasive. If the structures are not seen with (AS-OCT), then UBM can be utilized.
Cosmetic iris implants may lead to uveitis hyphema glaucoma syndrome and corneal decompensation, so specular microscopy helps evaluate the corneal endothelial status by providing the endothelial counts and morphology of the cells.[33] Central corneal thickness also helps quantify corneal edema, often associated with the UGH syndrome. Ultrasound brightness scan is helpful in the diagnosis of atypical UGH syndrome cases that present with vitreous hemorrhage.[13]
Glaucoma evaluation can be performed by gonioscopy, central corneal thickness, perimetry, and optical coherence tomography of the optic nerve. All the other causes of uveitis need to be ruled out. An individualized approach should be used to investigate such cases and may include complete blood count, erythrocyte sedimentation rate, serology for human immunodeficiency virus, Mantoux test, autoimmune antibody assays, chest x-ray, x-ray of the spine, and lumbosacral joint in both anterior-posterior and lateral views.[58] Optical coherence tomography of the macula can detect cystoid macular edema in UGH cases. Fundus fluorescein angiography helps to rule out the other causes of vitreous hemorrhage or iris new vessels.[59] A coagulation profile and a history of anticoagulant medications should be sought in cases with recurrent hyphema in UGH syndrome.
Treatment / Management
In patients with UGH Syndrome, IOP should be reduced using topical and systemic medications. Antiglaucoma medications and corticosteroids lower IOP and control anterior segment inflammation.[60] Inflammation needs to be controlled with topical steroids. The dosage and the type of steroid preparation are selected based on the diffusion across the cornea and the preparation's potency.[61] Adjuvant therapy in the form of non-steroidal anti-inflammatory drugs and cycloplegics is also helpful in relieving ciliary spasms and inflammation. These medications bring symptomatic relief to the patient. Cycloplegics need to be added to alleviate ciliary spasms and iris sphincter spasms.
Miotics (including pilocarpine) should be avoided as they increase the mechanical chaffing of the iris. In sulcus-placed PCIOLs causing reverse pupillary block leading to UGH syndrome, laser peripheral iridotomy may help.[57] Depending upon the cause of the UGH syndrome, the treatment modality changes from patient to patient.[62](B2)
Specific treatment may be offered in patients where the cause is localized. In patients with express shunts, localized iris chaffing might be managed with laser iridoplasty. Rarely, if an iris blood vessel is found to be the source of bleeding, an argon or neodymium-doped yttrium aluminum garnet laser can be used to stop the bleeding. Special concern is necessary for myopic, vitrectomised patients presenting with the signs and symptoms of UGH syndrome. Those with raised IOP due to the concavity of the iris or reverse pupillary block may be treated with peripheral laser iridotomy only.[63][64] IOL exchange, explant, repositioning, or amputation of IOL haptic should be performed if the inflammation is not controlled medically and the vision is reduced (see Image. Uveitis Hyphema Glaucoma Syndrome Indicating Iridectomy). IOL exchange is the definitive treatment of UGH syndrome, where no other pathology is responsible for the patient's signs and symptoms. An explanation of the IOL may not be needed in cases with IPUGH syndrome.[53] The UGH syndrome is one of the most common indications (around 12%) of IOL exchange.[65](B2)
Suturing the haptics of in-the-bag IOL to the iris in an eye with pseudophacodonesis may successfully resolve the associated UGH syndrome.[66] Intracameral injection of an anti-vascular endothelial growth factor agent, bevacizumab, reduces the frequency of acute attacks of UGH syndrome. Intracameral bevacizumab may also cause regression of iris neovascularization and lessen the inflammatory macular edema.
Differential Diagnosis
The differential diagnoses include:
- Trauma: The UGH syndrome could be mistaken for blunt trauma to the eye in patients with hyphema and anterior segment inflammation.[67] Also, traumatic cases have other signs of ocular trauma.
- Inflammatory glaucoma: Rubeosis iridis may be present. A history of surgery may or may not exist.[19] Other signs of uveitis are present.
- Hyphematous conditions: Vascular abnormalities, iris tufts, varices causing bleed may be confused with UGH syndrome. Herpes zoster and herpes simplex uveitis may be associated with hyphema.
- Retinal vascular occlusions (especially central retinal venous occlusion) should be ruled out in cases with iris new vessels and neovascular glaucoma.
- Coagulation disorders and sickle cell disease should be excluded in cases with UGH syndrome.[68]
- Uveitis: Uveitis can usually be controlled well using corticosteroids and cycloplegics. In inflammation due to UGH syndrome, patients' signs and symptoms persist after using topical corticosteroids and cycloplegics.[69][70]
- Retinoblastoma: A few cases may present as hyphema in the late stages.
- Chronic postoperative endophthalmitis: This is differentiated as the patient presents with similar complaints. Initially, topical or systemic corticosteroids relieve the symptoms.[71][72] The presence of hyphema and uveal touch by the implant with transillumination defects in the iris favors the diagnosis of UGH syndrome.
Prognosis
Though UGH syndrome may be managed conservatively, surgery with explantation of the implants is usually curative. Surgery, however, may not guarantee the resolution of UGH syndrome. Polymethyl methacrylate, acrylic, and silicone lenses with better designs and quality have reduced the complications. In a large series of 71 patients with UGH syndrome, the patients treated surgically achieved better final visual acuity and lower IOP. In contrast, the conservatively treated patients did not experience improved visual acuity or reduced IOP.[40]
Complications
Some complications of UGH can include:
Corneal staining: Long-standing hyphema causes corneal staining, which usually occurs with hyphema and raised IOP. This starts from the periphery of the cornea and grows centripetally.[24]
Chronic inflammation: May be noted and lead to posterior synechia or peripheral anterior synechia.
Pseudophakic bullous keratopathy: Endothelial damage due to constantly raised IOP, damage by the implant itself, and inflammation can result in endothelial pump failure, leading to pseudophakic bullous keratopathy that might ultimately need penetrating or endothelial keratoplasty.
Vitreous hemorrhage: May be noted in a few cases of UGH syndrome when communication occurs between the anterior and posterior segments (posterior capsular rent or defect with rupture of the anterior hyaloid face).[9]
Glaucomatous nerve damage: Raised IOP may result in optic nerve damage and blindness.
CME: May occur in a few cases.[73]
Deterrence and Patient Education
Patients with UGH syndrome should be educated on their conditions, including their ocular manifestations, treatment, and complications. Complicated cataract surgery and complications cause significant impairments in terms of vision and mental well-being. The patient should be well motivated and educated about the nature of the condition and the benefits of timely intervention. Careful counseling is needed for such persons because the management depends upon the cause of the prevailing condition. The management options include explantation of the IOL, implant, or other procedures to reduce the damage. All the conditions should be assessed beforehand, like the patients' ability to lay supine and any other coexisting medical conditions that may further add to the complications.
Pearls and Other Issues
The single-piece foldable acrylic PCIOL or other PCIOLs should be implanted within the capsular bag to prevent UGH syndrome. When this is impossible due to posterior capsular rent or extension of anterior capsulorhexis, multipiece PCIOL should be implanted in the sulcus in the correct orientation (as it has a vault) and preferably with reverse optic capture (haptics in the sulcus and optic behind the anterior capsulorhexis margin).[42] When capsular support is inadequate, available surgical options include scleral-fixated IOL (glued or without glue, sutured or without suture), iris-fixated IOL (retropupillary iris-claw lens or iris-claw lens in the anterior chamber), iris-sutured PCIOL, and ACIOL. ACIOL should be appropriately sized, and the total ACIOL diameter should be close to the horizontal white-to-white diameter (in mm) plus 1 mm.[51] The ACIOLs have a vault, and incorrect orientation causes upside-down lens syndrome associated with complications, including corneal decompensation, pupillary capture, chronic anterior uveitis, iris adhesions, and CME.
Enhancing Healthcare Team Outcomes
The UGH syndrome is a late complication of cataract surgery. The key to optimal management involves patient compliance and patient education regarding prognosis. The optometrist plays a vital role in refraction and documenting the patient's visual acuity. The opthalmologist should discuss all the management options, and the patient should have realistic expectations. If neovascularization of the iris, vitreous hemorrhage, or CME is present, a retina specialist should be consulted. The pharmacist should explain the dosage of the eye drops. Ophthalmic technicians play an important role in glaucoma or retinal investigations. Clinicians or specialists must be consulted in patients with systemic disorders. Excellent interprofessional coordination may ensure the best outcomes for patients with UGH syndrome. Care coordination is pivotal in ensuring seamless and efficient patient care. This coordination minimizes errors, reduces delays, and enhances patient safety, ultimately leading to improved outcomes and patient-centered care that prioritizes the well-being and satisfaction of those affected by UGH.
Media
(Click Image to Enlarge)
Uveitis Hyphema Glaucoma Syndrome Indicating Iridectomy. Displacement of intraocular lens within anterior chamber indicating the need for iridectomy.
Contributed by K Tripathy, MD
(Click Image to Enlarge)
Uveitis Glaucoma Hyphema Syndrome or Ellingson Syndrome. Uveitis glaucoma hyphema syndrome or Ellingson syndrome presents with features of inflammation, high eye pressure, and bleeding.
Contributed by K Tripathy, MD
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