Secondary congenital aphakia

Area of Science:

• Ophthalmology
• Pediatric Medicine
• Genetics

Background:

• Congenital rubella syndrome (CRS) is a complex condition with multisystemic effects.
• Ocular manifestations of CRS include cataracts, microphthalmia, and glaucoma.
• Secondary congenital aphakia is a rare condition characterized by the absence of the lens.

Observation:

• A 2-year-old patient with diagnosed CRS presented for cataract surgery.
• Intraoperative findings revealed the absence of the lens cortex, leading to a diagnosis of secondary congenital aphakia.
• Associated CRS features included sensorineural deafness, congenital heart disease, intellectual disability, microcephaly, and microphthalmia.

Findings:

• The patient was diagnosed with secondary congenital aphakia during cataract surgery.
• Surgical management involved posterior capsulorhexis and anterior vitrectomy.
• Intraocular lens implantation was deferred due to the aphakic state.

Implications:

• This case highlights the importance of intraoperative vigilance for unexpected findings in CRS patients.
• Modified surgical techniques may be necessary for managing secondary congenital aphakia.
• Delayed intraocular lens implantation is a viable strategy in such complex cases.