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Phacomorphic Glaucoma from an Age-related Cataractous Lens in a 97-year-old Male

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Title: Phacomorphic glaucoma from an age-related cataractous lens in a 97-year-old male
Author (s): Kyle Vinson; Sravanthi Vegunta, MD; Brock Alonzo, MD.
Photographer: James Gilman, CRA, FOPS
Date: 07/4/2019
Ultrasonographer: Roger P. Harrie, MD
Keywords/Main Subjects: lens-induced glaucoma, phacomorphic, intumescent, age-related, cataract, lens, secondary angle-closure
Diagnosis: Phacomorphic glaucoma of left eye; Pseudoexfoliation syndrome of both eyes.

Brief Description: A 97-year-old male presented to the eye clinic complaining of gradual, painless loss of vision in his left eye. He reported being able only to see shadows with his left eye over the past 5 years. He denied perceiving flashes, floaters, or haloes, pain, irritation, or redness in his left eye. He also denied headache, nausea or vomiting. His right eye was asymptomatic.

Exam:

Right eye Left eye
VA distance CC 20/30 -3 Hand Motion
BCVA 20/30 + 1 No improvement
Pupils 3mm, round, reactive 5mm, irregular, minimally reactive, + afferent pupillary defect
VF Full Constricted
EOM Poor reliability Poor reliability
IOP (tonopen) 15 34

 

Slit Lamp Exam:

Right eye Left eye
Lids/Lashes 2+ dermatochalasis upper lid 2+ dermatochalasis upper lid
Conjunctiva/sclera Decreased tear film, white and quiet Decreased tear film, white and quiet
Cornea Clear Clear
Anterior chamber Narrow by Van Herick Extremely narrow by Van Herick
Iris Round, reactive Irregular
Lens 1+ Nuclear sclerosis, 2+ Cortical cataract, 2+ Pseudoexfoliation material 3+ Nuclear sclerosis, 2+ Cortical cataract, 4+ Posterior subcapsular cataract
Vitreous Normal No view
Disc Normal No view
Macula Normal No view
Vessels Normal No view

 


Figure 1. Slit lamp photograph of an intumescent cataract from a different individual, as our patient did not have photos taken.

Figure 2. Ultrasound biomicroscopy showing large cataractous lens with crowding of the angle.


Figure 3. B-scan ultrasonography showing large cataractous lens and posterior vitreous detachment without retinal detachment or masses noted.

 

Management: The plan was extracapsular cataract extraction with phacoemulsification and intraocular lens placement of the left eye.

Course: Cataract extraction was complicated by dropped nucleus, vitreous hemorrhage, and hyphema secondary to complete zonular disruption. B-scan ultrasonography at a subsequent postoperative visit confirmed these findings. In addition to his postoperative drops, which included topical moxifloxacin, ketorolac, and prednisolone; topical brimonidine tartrate and oral prednisone 60 mg daily were initiated to treat his postoperative inflammation. He subsequently underwent pars plana vitrectomy and lensectomy with plans for future secondary IOL placement.

Figure 4. Postoperative B-scan showing intraocular lens nucleus in the posterior chamber and vitreous cells, corresponding clinically with vitreous hemorrhage.

 

Summary of the Case: Lens-induced glaucoma can be broadly categorized as secondary closed-angle glaucoma or secondary open-angle glaucoma. The former is termed phacomorphic glaucoma, which can result from two distinct etiologies, either an anteriorly displaced lens, or an anteriorly or posteriorly dislocated lens. The most common cause is from a slowly developing, age-related cataractous lens with increased anterior-posterior diameter (as in this case). A rarer cause, which may be seen in younger patients, is a rapidly developing, intumescent or traumatic cataract, in which case a hyperosmotic cortical cataract liquefies and expands in size.

An anteriorly displaced lens may cause phacomorphic glaucoma by one of two mechanisms. The lens can push the iris forward, thereby blocking aqueous humor outflow and creating a pressure gradient that apposes the iris to the iridocorneal interface (iris bombé). In this mechanism, which is known as pupillary block, the anterior chamber depth is shallowed peripherally. 1,2 Alternatively, an anteriorly displaced lens can cause phacomorphic glaucoma through forward displacement of the lens-iris diaphragm, in which case the anterior chamber is shallowed centrally and peripherally.1 Phacomorphic glaucoma secondary to an anteriorly displaced lens is more common in patients with a preexisting shallow anterior chamber and in those with weakened zonules secondary to trauma, age, or pseudoexfoliation.2

The other etiology of phacomorphic glaucoma is an anteriorly or posteriorly dislocated lens. An anteriorly dislocated lens may directly result in phacomorphic glaucoma through a pupillary block mechanism. A posteriorly dislocated lens, on the other hand, may indirectly result in phacomorphic glaucoma by way of prolapsing vitreous into the anterior chamber that causes pupillary block.1–3

Diagnosis: The diagnosis of phacomorphic glaucoma is suspected by the presence of an asymmetric dense cataract or lens dislocation, narrowed or closed angles, elevated intraocular pressure, and the typical signs and symptoms of angle-closure glaucoma in the case of an acute precipitant.4 Gonioscopy is the gold-standard for confirming angle closure. Ultrasound biomicroscopy (UMB) should be performed, and typically reveals a large cataractous or intumescent lens and shallow anterior chamber. Disproportion between lens thickness and eye length, especially if asymmetry exists between the fellow eye, is characteristic.1 Optical coherence tomography (OCT) of the anterior segment is also useful to evaluate the angle anatomy.

The differential diagnosis is broad, and includes primary acute angle-closure glaucoma, phacolytic glaucoma, plateau iris glaucoma, and uveitic glaucoma. Primary angle-closure glaucoma is differentiated from phacomorphic glaucoma by the absence of an asymmetric dense cataractous, intumescent, or dislocated lens. Phacolytic glaucoma is distinguished from phacomorphic glaucoma by the presence of an open angle on gonioscopy, hypermature cataract, cell and flare in the anterior chamber, and white, cortical material in the anterior chamber.1 Plateau iris glaucoma is the more likely diagnosis when an anteriorly displaced or dislocated lens is absent and gonioscopy or anterior segment OCT reveals iridotrabecular contact. Uveitic glaucoma is the favored diagnosis when signs and symptoms of uveitis are present (e.g., cell and flare in the anterior chamber, ciliary flush), open angles are observed on gonioscopy, and an anteriorly displaced or dislocated lens are absent.1

Management: Phacomorphic glaucoma secondary to an anteriorly displaced lens is typically resistant to medical therapy alone. The definitive treatment is removal of the lens, which is usually achieved by phacoemulsification or small incision cataract surgery (SICS).1,4,5 However, laser peripheral iridotomy is usually necessary prior to lens extraction if pupillary block is present, and occasionally argon laser peripheral iridoplasty is required after iridotomy to further widen the iridocorneal angle.1,4

References:

  1. Teekhasaenee C, Dorairaj S, Ritch R. Secondary Angle-Closure Glaucoma. In: M. Shaawary T, B. Sherwood M, A. Hitchings R, G. Crowston J, eds. Glaucoma. 2nd ed. Elsevier Saunders; 2015:401-409.
  2. M. Liebmann J, Ritch R. Glaucoma secondary to lens intumescence and dislocation. In: Ritch R, Shields MB, Krupin T, eds. The Glaucomas. 1st ed. St. Louis: Mosby; 1989:1027-1045.
  3. Yanoff M, Sassani J. Lens. In: Yanoff M, Sassani J, eds. Ocular Pathology. 7th ed. Elsevier Saunders; 2015:329-343.
  4. B. Kaplowitz K, G. Kapoor K. An Evidence-Based Approach to Phacomorphic Glaucoma. J Clin Exp Ophthalmol. 2013;04(02):1-6. doi:10.4172/2155-9570.s1-006
  5. Moraru A, Pînzaru G, Moţoc A, Costin D, T Popa G, N Oblu P. Functional results of cataract surgery in the treatment of phacomorphic glaucoma. Rom J Ophthalmol. 2017;61(3):202-206. doi:10.22336/rjo.2017.37

Faculty Approval by: Griffin Jardine, MD

Identifier: Moran_CORE_26882

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