We describe a unique bilateral presentation of primary nuclear cataracts in the anterior vitreous with concurrent posterior capsule defects in a healthy male infant. Although the exact pathophysiology is unknown, we review distinguishing features on our evaluation that aid in diagnosis and explore possible etiologies.
An 8-week-old male infant was referred for bilateral dull red reflex, initially detected 1 week prior on a pediatrician’s examination. He was born at 38 weeks’ gestation and received standard prenatal and postnatal care. His birth and medical history were negative for forceps use, infection, trauma, or systemic disease. No other abnormalities were found on routine pediatric examination. The family history was significant for an older sister with bilateral infantile cataracts, surgical and clinical details of which were not known.
On clinical examination, he exhibited conjugate roving eye movements and blinked to light with each eye. Extraocular movements were full and pupils were round and reactive to light. Bilateral nuclear lens opacities were evident and obscured a detailed funduscopic view. Blood work for TORCH titers, serum glucose, phosphate and calcium, and urine studies were ordered and were negative.
During assessment under general anesthesia, intraocular pressure with pneumotonometer was 19 mm Hg in the right eye and 14 mm Hg in the left eye. Corneal diameter was 10.5 mm along the horizontal and vertical meridians in both eyes and axial length was 17.20 mm in the right eye and 17.10 mm in the left eye. Anterior segment examination was notable for bilateral, diffuse anterior and posterior subcapsular opacities. In addition, the depth between the anterior and posterior capsules was markedly shallowed. A sizeable homogenous, white opacity was detected in the anterior-middle vitreous in both eyes (Figure 1). As the globe was gently rotated with forceps, the noted opacity moved freely (Video). B-scan echography highlighted a well-circumscribed opacity in the anterior vitreous bilaterally and a small posterior stalk emerging from the optic nerve, not attached to the vitreous opacity, was visible in the left eye (Figure 2). Given these findings, we diagnosed our patient with bilateral cataracts displaced in the anterior vitreous associated with a primary posterior capsule defect (PCD).
Intraoperative photograph of the (A) right and (B) left eye highlighting diffuse cortical changes. A more dense posterior opacity is noted inferonasally in each eye, obscuring the view of the fundus.
B-scan echography of the (A) right and (B) left eye. A circular, well-circumscribed opacity is visible anteriorly in each eye and a small stalk coming off of the optic nerve is evident in the left eye.
The patient underwent a 25-gauge bimanual lensectomy and anterior vitrectomy. An anterior capsulotomy was completed using the vitrector. After initial aspiration of cortical material, a preexisting PCD was identified. The white opacity noted preoperatively in the anterior vitreous was identified by clinical appearance as the lens nucleus. The nuclear and remaining cortical material were removed with the vitrector without incident and anterior vitrectomy was completed (Video). One week following the initial surgery, a 25-gauge lensectomy of the left eye was performed and an identical preexisting PCD was observed with lens nucleus in the anterior vitreous. Ten months postoperatively, our patient remained aphakic, fitted with bilateral contact lenses. He was fixing and following reliably with each eye and the pattern of nystagmus showed a decrease in both amplitude and frequency.
We describe a distinct presentation of bilateral congenital cataracts in a healthy 8-week-old male infant. A unique finding in our case is the significant amount of lens material found in the anterior vitreous, secondary to a preexisting PCD. Preexisting PCDs have been reported in adult patients with posterior polar cataracts and are a well-known sequela of trauma.1,2 In the latter, a detailed initial history and meticulous clinical examination can forewarn the diagnosis. In traumatic events, the capsule tear often occurs centrally in the anatomically thinnest and most vulnerable location.2
In comparison to these two entities, the presence of a congenital cataract with a preexisting PCD is rare and was first reported in 1992 by Vajpayee and Sandramouli.3 More recently, in a study of congenital cataracts, posterior capsule tears were identified preoperatively in 6.75% of 400 cases.4 To facilitate early recognition, these authors describe unique anterior segment findings that were consistently present in all 27 cases. Specifically, the edges of the preexisting PCD were thickened, likely due to chronic migration of epithelial cells. In addition, they found that if the globe was moved back and forth, small white opacities in the degenerated anterior vitreous moved freely, analogous to a fishtail.4 The fishtail sign has also been described in children with posterior polar cataracts with secondary “moth-eaten” destruction of the posterior capsule and resulting vitreous debris.5
The exact etiology of a congenital cataract and preexisting PCD is not known. Some propose the capsular tear may arise secondary to early embryonic insult, such as infection.3 Others suggest posterior lentiglobus as the cause. Vasavada et al. described more patients with a unilateral presentation, consistent with lentiglobus, and speculate accelerated posterior cortical thinning may have produced the premature, full-thickness defect.4,6
In contrast, our patient had the unique finding of bilateral lenticular material in the vitreous cavity, in the absence of trauma or infection. In addition, all previously published reports describe the lens nucleus in its native location, anterior to the preexisting PCD with small amounts of vitreous debris.3,4,6 However, our patient harbored a single, homogenous opacity in the anterior vitreous of significant size, which we believe to be lens nucleus given its intraoperative and ultrasound appearance. In addition, the edges of the capsule defect were not fibrosed, possibly reflecting an acute time course. A similar fishtail sign was observed in our patient, indicating the preoperative vitreous location of lens material (Video).
We propose that posterior capsule traction from the embryologic hyaloid artery during gestation could create a full-thickness capsular disruption and subsequent cataract formation in the anterior vitreous. Other clinical reports have also speculated that traction from the hyaloid artery system could produce focal posterior capsule weakness; indeed, some authors suspect this mechanism in posterior lentiglobus.7 Although our patient did not have features of microphthalmos or microcornea, his B-scan demonstrated a unilateral stalk emerging from the left optic nerve not attached to the lens; a similar stalk was not evident on echography of the right eye, but literature suggests that after causing posterior capsule disturbances, the hyaloid vasculature may still fully reabsorb during development.7
Persistent fetal vasculature occurs bilaterally in 10% of cases and can produce many ophthalmic findings.8,9 Histopathologic study of eyes with persistent fetal vasculature shows various lenticular disturbances, and definite dehiscence of the posterior capsule was evident in one report in 50% of eyes with persistent fetal vasculature.10 Following rupture of the posterior capsule, retrolental spread of mesenchymal tissue can induce lenticular edema and further enhance rapid cataractous changes.10 We speculate that in our patient the hyaloid vasculature produced posterior capsule disturbances centrally, early in gestation, and generated a full-thickness defect triggering accelerated nuclear cataract formation in the anterior vitreous.
Once a preexisting PCD is found, the surgeon must proceed cautiously with lensectomy; it is recommended to avoid hydrodissection to prevent capsular bag instability and further enlargement of the posterior tear.4 In our patient, complete cataract extraction was achieved through an anterior, bimanual approach as lens material easily migrated forward, omitting the need for pars plana approach and posterior vitrectomy. The preexisting PCD can be converted to a primary posterior capsulotomy, allowing for successful secondary intraocular lens placement in the future.
Our case report does have some limitations. An intraoperative biopsy of lens material may have aided in clarifying the diagnosis and etiology. In addition, given the family history, a genetics evaluation could have provided greater insight into our patient’s condition; this consultation was ordered but not performed. However, both parents were examined and no lens opacity or cataract changes were seen. Also, there was no evidence of a similar condition in the patient’s sibling as indicated by the patient’s family.
We describe the unique finding of bilateral congenital cataracts in the anterior vitreous of a healthy male infant. The pathogenesis for this condition is poorly understood and we propose persistent fetal vasculature as a possible mechanism. This condition can be successfully managed with detailed preoperative ophthalmic examination and meticulous surgical technique.
- Kymionis GD, Diakonis VF, Liakopoulos DA, Tsoulnaras KI, Klados NE, Pallikaris IG. Anterior segment optical coherence tomography for demonstrating posterior capsular rent in posterior polar cataract. Clin Ophthalmol. 2014;8:215–217.
- Nanavaty MA, Mehta PA, Raj SM, Vasavada AR. Diagnosis of preexisting posterior capsule defect in traumatic white mature cataract with intact anterior capsule. Eye (Lond). 2006;20:949–951. doi:10.1038/sj.eye.6702061 [CrossRef]
- Vajpayee RB, Sandramouli S. Bilateral congenital posterior-capsular defects: a case report. Ophthalmic Surg. 1992;23:295–296.
- Vasavada AR, Praveen MR, Nath V, Dave K. Diagnosis and management of congenital cataract with preexisting posterior capsule defect. J Cataract Refract Surg. 2004;30:403–408. doi:10.1016/S0886-3350(03)00502-9 [CrossRef]
- Mistr SK, Trivedi RH, Wilson ME. Preoperative considerations and outcomes of primary intraocular lens implantation in children with posterior polar and posterior lentiglobus cataract. J AAPOS. 2008;12:58–61. doi:10.1016/j.jaapos.2007.08.003 [CrossRef]
- Vasavada AR, Praveen MR, Dholakia SA, Trivedi RH. Preexisting posterior capsule defect progressing to white mature cataract. J AAPOS. 2007;11:192–194. doi:10.1016/j.jaapos.2006.11.115 [CrossRef]
- Kilty LA, Hiles DA. Unilateral posterior lenticonus with persistent hyaloid artery remnant. Am J Ophthalmol. 1993;116:104–106. doi:10.1016/S0002-9394(14)71756-8 [CrossRef]
- Lim Z, Rubab S, Chan YH, Levin AV. Pediatric cataract: the Toronto experience-etiology. Am J Ophthalmol. 2010;149:887–892. doi:10.1016/j.ajo.2010.01.012 [CrossRef]
- Rahi JS, Dezateux C. Congenital and infantile cataract in the United Kingdom: underlying or associated factors. British Congenital Cataract Interest Group. Invest Ophthalmol Vis Sci. 2000;41:2108–2114.
- Haddad R, Font RL, Reeser F. Persistent hyperplastic primary vitreous: a clinicopathologic study of 62 cases and review of the literature. Surv Ophthalmol. 1978;23:123–134. doi:10.1016/0039-6257(78)90091-7 [CrossRef]