Reply: Posterior lenticonus with persistent fetal vasculature.

Sir,

We have reported for the first time a rare case of bilateral posterior lenticonus with bilateral persistent fetal vasculature (PFV).[1] Both eyes of the patient have subtle findings suggestive of PFV. If not looked at carefully and specifically, PFV may be missed out. This case adds evidence to existing hypothesis that PFV may have a role in pathogenesis of posterior lenticonus.[12]

The case mentioned in the letter[3](“Progressive posterior lenticonus with total lenticular opacity”) is of a child with of posterior lenticonus with total cataract. There is no association of PFV noted. The case is well documented, and diagnosed and managed properly. However, we would like to add a few things.

It is difficult to distinguish posterior lenticonus from posterior capsular defect with ultrasonography alone. Performing ultrasound biomicroscopy (UBM) with 35 MHz probe adds valuable information in surgical planning as lens curvature and continuity of posterior capsule can be clearly seen.[45]

During surgery, since posterior capsule is thinned out, precautions need to be taken to avoid inadvertent capsule rupture. Gentle hydrodissection, low flow settings and bimanual surgery are recommended[6]“Jellyfish sign” mentioned in the letter is due to anterior and posterior bulging of posterior capsule with change in IOP and flow rate.[7] It may also be seen in cases of posterior capsular defect where anterior hyaloid face is intact. Posterior capsulorhexis can be performed depending on the age and IOL can be implanted in the bag or sulcus based on size of posterior capsulorhexis and area of thinning. With due precautions, complications during surgery are rare and anatomical outcomes are good.[8]

Visual outcomes may be affected by presence of amblyopia in long standing cases. Hence, in addition to cataract extraction, emphasis has to be laid on amblyopia therapy post-operatively for visual rehabilitation.

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