In my practice, many patients present for cataract surgery with early visual changes and a desire for premium IOLs. However, I recently cared for a patient with scleroderma who presented with an advanced cataract. Her surgical case proved both challenging and rewarding.
INITIAL CONSULTATION
A 59-year-old woman presented with hand motion vision in her right eye, which was correctable to 20/300 with a manifest refraction of -1.50 -4.50 x 85. She reported a loss of vision over the past 6 months, which was likely expedited by previous systemic and nasal steroid use.
On slit-lamp examination, she was noted to have a white cataract in her right eye. I observed advanced anterior subcapsular and cortical changes with moderate nuclear density, which was suspicious for an intumescent cataract. There was no view of the posterior pole. In her left eye, the patient had a moderate cataract with mixed astigmatism, and visual acuity was correctable to 20/25. Her corneal topography showed significant against-the-rule astigmatism in both eyes, which correlated to her refractions.
As commonly seen with scleroderma, the patient’s eyes were enophthalmic secondary to significant orbital fat atrophy. Her eyelids were stiff and contracted, and she had an incomplete blink, resulting in significant exposure keratopathy and superficial punctate keratitis. There was shrinkage of the conjunctiva and shortening of the inferior fornix in both eyes. Her anterior chamber depth and axial length were noted to be short.
PREOPERATIVE PLANNING
This case presented several challenges. Removal of a white intumescent cataract is complex, especially in the setting of a tight orbit and a short eye. Scleroderma is associated with increased risk of zonular weakness and capsular contraction, but, in this case, given the severity of corneal astigmatism, a toric IOL would be beneficial.
The patient worked in front of a computer, and she was interested in receiving a presbyopia-correcting IOL. She had previously seen several surgeons, all of whom tried to deter her from this option. As stated above, there was no view of the posterior pole in her right eye, and the status of her optic nerve and macula could not be assessed. Notably, patients with scleroderma may have choroidopathy and vascular occlusions, limiting vision potential. Posterior segment findings were normal in her left eye, which was somewhat reassuring.
After obtaining a B-scan, I planned to use an extended depth of focus toric IOL (Symfony Toric, Johnson & Johnson Vision) as my primary lens choice. While consenting the patient, I detailed the pros and cons of this premium IOL. She was informed that the IOL would not be placed in the setting of an irregular capsulorhexis, zonular issues, or other surgical concerns. She knew that the status of her retina could affect her vision. We also discussed the potential need for a future IOL exchange if she experienced photopsias or if the IOL was not stable. She was agreeable to the risks and motivated to proceed. We delayed surgery until her ocular surface was optimized with the use of artificial tears, omega-3 fatty acids supplements, punctal plugs, and steroid ointment.
SURGERY
Intravenous mannitol was administered to soften the vitreous and decrease the posterior pressure. In my experience, 12.5 to 25.0 g administered 30 to 60 minutes before surgery is effective, less than the commonly published dose of 1.0 to 2.0 g/kg.
The patient was placed in a slight reverse Trendelenburg position to reduce central venous pressure and thus lower posterior venous pressure. Under a dispersive OVD, Vision Blue (DORC International BV) was instilled in the anterior chamber and painted onto the central portion of the capsule.
With intumescent white cataracts, high intracapsular pressure is a concern and may lead to sudden radialization of the capsulorhexis. In these cases, my preferred technique is to puncture the anterior capsule with a 27-gauge needle and aspirate the liquefied cortex. Microforceps are then used to expand the capsulorhexis by spiraling outward to the desired diameter.
During hydrodissection, care was taken to mobilize the lens and minimize stress on the at-risk zonular fibers. Chopping techniques were employed for efficient nuclear disassembly. Capsular retractors and Cionni capsular tension rings were available in the event of significant zonular laxity. In this case, there was no noted zonular weakness, and the Symfony Toric IOL was successfully implanted. I opted to place a capsular tension ring to ensure stabilization of the lens and reduce the likelihood of postoperative IOL decentration.
Figure 1 | A happy postop patient with scleroderma.
CONCLUSION
Postoperatively, the patient had an excellent uncorrected visual outcome at distance and intermediate ranges (Figure 1). This case taught me not to shy away from implanting presbyopia-correcting IOLs in patients who may not be so-called perfect candidates. With appropriate consultation and financial considerations, these lenses may offer patients improved quality of life and may be considered more broadly.
