SUMMARY

This case study focuses on the management of a 7-year-old female patient with bilateral ectopia lentis, developmental delays, and high anisometropic hyperopic astigmatism. The comprehensive management approach included diagnostic findings and surgical interventions such as 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 is a condition in which the crystalline lens inside the eye becomes dislocated, affecting how light focuses on the retina and leading to blurry vision. This condition poses a significant challenge for pediatric ophthalmologists due to the ongoing development of children’s eyes and their difficulty explaining vision problems.

PATIENT PROFILE

Age: 7 years

Diagnosis: Bilateral ectopia lentis, developmental delays, high anisometropic hyperopic astigmatism, autism spectrum disorder, and refractive amblyopia

Clinical Background: The patient presented with poor compliance with spectacle use, short axial lengths of the eyes, and a history of not wearing her spectacles despite poor visual acuity. She also exhibited developmental delays, sensory disturbances, and hypotonia.

SIGNS & SYMPTOMS

Individuals with ectopia lentis typically display:
Blurred vision
Refractive errors
Lens subluxation or dislocation
Visual acuity impairment
Potential amblyopia

INITIAL ASSESSMENT

A comprehensive ophthalmic examination 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
The primary diagnosis of ectopia lentis was confirmed through clinical examination and cycloplegic refraction, with lens subluxation more noticeable in the left eye.

TREATMENT MODALITIES

Surgical Intervention: Due to the patient’s spectacle non-compliance and significant lens subluxation, surgical intervention was indicated.

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

Surgical Technique:
1. Anterior Capsulorhexis: A 5 mm capsulorhexis was created in each eye.
2. Lens Material Removal: Iris hooks and a vitrector were used to aspirate lens material and polish the lens capsule.
3. Capsular Tension Segments (CTS): Two 9.5 mm CTS were introduced into the capsular bag and fixed to the sclera using 5-0 prolene sutures with a double-flanged polypropylene suture technique.
4. Intraocular Lens (IOL): A single-piece acrylic lens was injected into the capsular bag.
5. Posterior Capsulotomy and Anterior Vitrectomy: A 4 mm posterior capsulotomy and vitrectomy were performed.

Postoperative Care:
Discharge instructions included eye protection, prescribed eye drops, and regular follow-up visits.

CHALLENGES & 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.
Postoperative Management: Continuous follow-up and prescription adjustments are critical to maintaining visual acuity and preventing amblyopia.

PROGNOSIS AND FUTURE DIRECTIONS

Postoperative Follow-Up: Seven months post-surgery, the patient exhibited significant improvements, with corrected visual acuity reaching 20/70 in both eyes. Cycloplegic refraction revealed measurements of -2.00 +1.00 x 90 for the right eye and -0.50 +1.50 x 90 for the left eye. These results demonstrated effective lens stabilization and enhanced visual function, indicating a positive surgical outcome.

This case highlights the successful management of a complex 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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Authors: Dr. Kamran Ahmed (MD) & Dr. Aishah Shafiq