Lens Management in Microspherophakia: Case Reports and Clinical Approaches
Primary Recommendation
Lensectomy with scleral-fixated posterior chamber intraocular lens (SFIOL) implantation is the definitive surgical treatment for microspherophakia, particularly when complicated by lens subluxation or secondary angle-closure glaucoma, providing excellent visual outcomes and sustained intraocular pressure control. 1, 2
Clinical Context and Presentation
Microspherophakia presents with characteristic features that guide management decisions:
- Lenticular myopia with a spherical lens of decreased equatorial diameter 1, 3
- Shallow anterior chamber predisposing to angle-closure glaucoma 1, 3
- Progressive zonular laxity leading to lens subluxation or dislocation 1, 2
- Secondary angle-closure glaucoma as a serious complication requiring urgent intervention 1, 2, 3
Critical pitfall: Microspherophakia should be ruled out even in middle-aged patients presenting with angle-closure glaucoma, not just pediatric cases. 1
Surgical Management Algorithm
Immediate Surgery Indications
Proceed urgently with lensectomy and SFIOL when:
- Pupillary block with acute angle-closure glaucoma (IOP >50 mmHg) 1, 2
- Lens subluxation causing visual compromise 1, 2
- Uncontrolled IOP despite medical management 1
In one case series, eyes presenting with pupillary block had mean preoperative IOP of 54.53 ± 7.33 mmHg, which normalized to 11.67 ± 2.88 mmHg post-lensectomy with SFIOL. 2
Elective Surgery Indications
Consider planned lensectomy with SFIOL for:
- Lens subluxation without acute glaucoma but with progressive zonular weakness 2
- Significant refractive error affecting quality of life 2
- Risk stratification for future complications in younger patients 4
Surgical Technique: Scleral-Fixated IOL
The evidence strongly supports SFIOL as the preferred approach over other techniques in microspherophakia. 1, 2
Outcomes from Case Series
A 2014 case series of 8 eyes (4 patients, mean age 28 years) demonstrated: 2
- Visual acuity improvement: From 0.008 decimals preoperatively to 0.50 decimals in acute glaucoma cases
- IOP normalization: All eyes achieved IOP 11-13 mmHg at 18-month follow-up
- Excellent centration: All SFIOLs remained well-centered with no complications
- No perioperative or postoperative complications reported
Key Technical Considerations
- Complete lens removal is essential to eliminate pupillary block risk 1, 2
- Scleral fixation provides stable IOL positioning despite zonular absence 1, 2
- IOP correction for corneal thickness: Thickened corneas may give falsely elevated IOP readings requiring adjustment 1
Alternative Approach: Iris-Fixated Phakic IOL
In highly selected cases without lens subluxation or glaucoma, iris-fixated phakic IOLs (Verisyse/Artisan) may be considered for refractive correction alone. 5
Strict Selection Criteria
This approach requires: 5
- Adequate anterior chamber depth (not shallow)
- No history of lens dislocation or subluxation
- Stable zonules on examination
- Patient compliance with annual monitoring
- No active or history of angle-closure glaucoma
Long-term Monitoring Requirements
When iris-fixated pIOLs are used: 5
- Annual endothelial cell counts are mandatory
- Scheimpflug photography to monitor spacing between pIOL, crystalline lens, and corneal endothelium
- Biomicroscopy for adequate clearance assessment
- Vigilance for lens subluxation development over time
Important caveat: This approach is NOT recommended for most microspherophakia patients and should be reserved for exceptional cases with stable anatomy. 5
Medical Management Considerations
Preoperative IOP Control
For acute angle-closure glaucoma presentation: 1, 3
- Topical and systemic IOP-lowering agents as temporizing measures
- Cycloplegics to deepen anterior chamber by relaxing ciliary muscle
- Avoid miotics (pilocarpine) which can worsen pupillary block by increasing lens-iris contact
When Surgery Cannot Be Immediate
If trabeculectomy was performed previously (as in one reported case): 1
- Monitor zonular stability closely over time
- IOP may remain controlled in mid-teens without medication
- Future lens instability remains a concern requiring ongoing surveillance
Pediatric Considerations
In younger patients (such as the reported 3-year-old case): 3
- Early identification is critical to prevent irreversible glaucomatous damage
- Megalocornea may be an associated finding
- Multidisciplinary care with pediatric ophthalmology, glaucoma, and genetics specialists
- Lifelong follow-up is mandatory given the progressive nature 4
Systemic Associations to Evaluate
- Weill-Marchesani syndrome (short stature, brachydactyly, joint stiffness)
- Marfan syndrome (tall stature, arachnodactyly, cardiovascular abnormalities)
- Axenfeld-Rieger syndrome (iris abnormalities, dental/facial anomalies)
- Iridocorneal endothelial syndrome
Long-term Outcomes and Prognosis
Based on available case reports: 1, 2
- Visual rehabilitation is achievable with BCVA improving from counting fingers to 20/40 or better
- IOP control is sustained at 2-year follow-up without medications in successfully treated cases
- No reported IOL decentration or dislocation with proper scleral fixation technique
- Contralateral eye monitoring is essential as the condition is typically bilateral
Critical monitoring point: Even in eyes where the lens appears stable (held by posterior synechiae), future zonular stability cannot be guaranteed and requires ongoing surveillance. 1