Comment on: Progressive posterior lenticonus with total lenticular opacity.

Sir,

We read the unique case report of bilateral lenticonus titled “Posterior lenticonus with persistent fetal vasculature” (published in Indian J Ophthalmol 2018; 66:1335-6)[1] with interest and would like to make some contributions.

A 5-year-old girl presented to us with a whitish opacity in the left eye noticed by the parents for 2 months’ duration. Ocular examination showed normal corneal diameters with unilateral total cataract and exotropia in the left eye [Fig. 1a]. Ultrasound B-scan showed a large bulge in the posterior lens capsule extending up to 10 mm axially which confirmed the diagnosis of posterior lenticonus [Fig. 1b]. There was no persistent fetal vasculature. Bimanual lens aspiration was performed after a curvilinear capsulorhexis with gentle hydrodissection and low vacuum settings. Intraoperatively, the posterior capsule appeared thinned out centrally and stretched posteriorly as the intraocular pressure increased and bulged forward as the pressure decreased. Ganesh et al. described this as a “jellyfish sign.”[2] In the bag fixation of hydrophobic acrylic foldable intraocular lens (IOL) resulted in posterior tilting, even in the presence of an intact posterior capsule and relative thickening of the capsule around the axial cone. Hence, a three-piece IOL was placed in the sulcus after primary posterior capsulotomy (PPC) and limited anterior vitrectomy [Fig. 1c]. Postoperatively, her fundus was normal and vision improved to 20/400. She is on follow-up with amblyopia therapy.

Figure 1

(a) Clinical photograph of a 5-year-old child presenting with total cataract and exotropia in the left eye. (b) Preoperative ultrasound B-scan demonstrating a large bulge in the posterior capsular area. (c) Postoperative slit-lamp image showing the placement of a three-piece IOL in the sulcus (precluding in the bag fixation)

Posterior lenticonus is a progressive circumscribed bulge in the posterior capsule and cortex. In most cases, it occurs unilaterally and sporadic; bilateral presentation suggests an autosomal dominant inheritance. The pathogenesis is intriguing and has attracted multiple theories apart from hyaloid artery traction like overgrowth of posterior lens fibers due to aberrant hyperplasia of subcapsular epithelium or an inherited weakness of the posterior lens capsule.[34] The posterior bulge increases with age, and as the cataract advances it tends to obscure the lenticonus. Preoperative ultrasound B-scan or ultrasound biomicroscopy can aid the surgeon in diagnosing such cases and avoiding intraoperative surprises. Cataract surgery is challenging due to the fragile and bulging posterior capsule. Hydrodissection is contraindicated. Fluctuations in the intraocular pressure and anterior chamber turbulence can threaten the capsular bag stability. A low flow rate and low bottle height are dedicated to avoiding a posterior capsule rupture. It is required to perform a PPC if the bulge is more than 5 mm, in young children as posterior capsule opacification is rapid and virtually inevitable with a floppy, outstretched capsule or to convert a preexisting posterior capsule rupture to a desired size.[5]

Lee et al. found that the final visual outcome was better in patients with total lenticular opacity when compared with a posterior polar opacity, probably due to an earlier age at presentation and cataract extraction.[6] The dense amblyopia accompanying this condition is due to high index myopia and astigmatism, stimulus deprivation, and anisometropia. Earlier surgery may be indicated to prevent visual impairment in children with posterior lenticonus.

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Conflicts of interest

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