Case Report: Management of Pediatric Ectopia Lentis and Refractive Errors

Abstract

This case report describes the management of a 7-year-old female with bilateral ectopia lentis, developmental delays, and high anisometropic hyperopic astigmatism. 

In this case report, we will discuss the diagnostic findings and surgical interventions, including Capsular Tension Segment (CTS), fixation of the intraocular lens (IOL)-bag complex, and postoperative outcomes, highlighting a successful multifaceted approach to managing a unique case of pediatric ectopia lentis. Introduction

Ectopia lentis, a condition where the crystalline lens inside the eye gets dislocated, poses a significant challenge for pediatric ophthalmologists.  The crystalline lens, normally transparent and focused, acts like a natural camera lens, helping us see clearly. In ectopia lentis, this lens gets displaced from its usual position, affecting how light focuses on the retina, leading to blurry vision.

This condition is particularly challenging in children because their eyes are still developing. Unlike adults, children often can't explain their vision problems clearly, making diagnosis more difficult. 

In our case, the patient's condition was further complicated by poor compliance with spectacle use and short axial lengths of her eyes, which required the use of a single-piece IOL to achieve post-operative emmetropia.

Patient Presentation

Adopted by her maternal aunt, our 7-year-old female patient, diagnosed with autism spectrum disorder and developmental delay, had a history of not wearing her spectacles and poor visual acuity despite correction (as mentioned earlier). Her medical history included developmental delays, sensory disturbances, hypotonia, and refractive amblyopia. 

The young patient underwent a comprehensive ophthalmic examination that included:

• Visual Acuity (without correction): OD: 20/125, OS: 20/80
• Cycloplegic Refraction: Right Eye (OD): +5.75 +0.25 x 5, Left Eye (OS): -0.50 +7.50 x 87

Diagnosis and Clinical Management

The primary diagnosis of ectopia lentis was confirmed through clinical examination and cycloplegic refraction, highlighting significant refractive errors and lens subluxation. We observed that ectopia lentis was more noticeable in the left eye (OS > OD).

Surgical Intervention

Due to the patient's spectacle non-compliance, lens subluxation, and its detrimental impact on visual function, surgical intervention was indicated:

Preoperative Preparation:

General anesthesia was administered. Preoperative ultrasonography confirmed superior lens dislocation in both eyes.

Surgical Technique:

• Anterior Capsulorhexis: A 5 mm capsulorhexis was created to access the lens material in each eye.
• Lens Material Removal: Iris hooks and a vitrector were used to aspirate lens material and polish the lens capsule, preventing posterior capsule opacification.
• Capsular Tension Segments (CTS): Two 9.5 mm capsular tension segments were introduced into the capsular bag and fixed to the sclera using 5-0 prolene sutures with a double-flanged polypropylene suture technique.
• Intraocular Lens (IOL): A single-piece acrylic lens was injected into the capsular bag.
• Posterior Capsulotomy and Anterior Vitrectomy: A 4 mm posterior capsulotomy and vitrectomy were performed to address posterior capsule opacification and ensure long-term visual clarity.

Postoperative Care:

Discharge instructions included eye protection, prescribed eye drops, and regular follow-up visits to monitor recovery and visual acuity.

Postoperative Follow-Up

Results and Observations

After seven months (post-surgery), the patient exhibited significant improvement in visual acuity and stabilization of lens position.

• Visual Acuity (corrected): OD: 20/70, OS: 20/70
• Cycloplegic Refraction: Right Eye (OD): -2.00 +1.00 x 90, Left Eye (OS): -0.50 +1.50 x 90

The surgical outcomes were positive, demonstrating effective lens stabilization and enhanced visual function. Continuous monitoring and adjustments to the patient's prescription were needed to correct any remaining refractive errors or changes in lens position.

Discussion

Challenges and Considerations

Managing ectopia lentis in pediatric patients, especially those with additional developmental delays, requires an innovative approach:

• Surgical Decision-making: CTS fixation and double-flanged polypropylene sutures stabilized a high-powered single-piece IOL in the bag, aiming for emmetropia in a patient who doesn't wear glasses. This technique is beneficial in cases where three-piece IOLs with appropriate refractive power are unavailable.
• Postoperative Management: Continuous follow-up and prescription adjustments are critical to maintaining visual acuity and preventing amblyopia.

Conclusion

This case highlights the complexity and success of managing pediatric ectopia lentis through surgical intervention and tailored postoperative care. Innovative techniques, such as capsular tension segment fixation with double-flanged sutures, were crucial in stabilizing the lens and enhancing visual function. This approach underscores the importance of a multidisciplinary strategy in addressing complex ophthalmic conditions in pediatric patients, integrating surgical, optical, and developmental considerations for optimal outcomes.

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