From the King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Arif O. Khan, MD, King Khaled Eye Specialist Hospital, P. O. Box 7197, Riyadh 11462, Saudi Arabia.
Anterior polar cataracts are usually static congenital lens opacities that are visually insignificant but may be associated with amblyogenic refractive error or occlusion of the visual axis.1,2 This report describes a child with a unilateral, congenital, visually insignificant anterior polar cataract that progressed to a complete white cataract in the setting of focal anterior capsular disruption in the affected eye.
Institutional review board approval was granted for this retrospective case report. A 10-month-old female infant, the third child of a marriage between first cousins, was evaluated because of a white spot in the left eye that had been noted since birth. The patient’s birth and developmental history were normal, and there was no known childhood eye or medical disease in the family. During examination, the child became agitated when the right eye was covered. There was no strabismus, and versions appeared full.
Slit-lamp examination of the left eye showed a round, white anterior subcapsular lens opacity of approximately 3 mm. The overlying anterior capsule was intact and flat. Cycloplegic refraction (cyclopentolate 0.5%) was +2.75 −0.50 × 060 in the right eye and +8.75 −1.00 × 040 in the left eye. Findings on fundus examination with a 20-diopter lens were unremarkable in both eyes. The patient was prescribed atropine 1% ophthalmic ointment in the left eye every other day for chronic pupillary dilation, a contact lens for the left eye to correspond to +10.25 −1.00 × 040 in the spectacle plane (overplus to compensate for the loss of accommodation from atropine), and part-time occlusion of the right eye (half of all waking hours).
The child did not return for follow-up examination until 6 years later, and she had not been using any ocular treatment since the initial ophthalmic examination. Uncorrected visual acuity was 20/25 in the right eye and hand motions at 3 feet in the left eye. There was no strabismus, and versions were full. The pupils were normal. Slit-lamp examination of the left eye showed a total white cataract with central focal anterior capsular disruption associated with protruding lens material (Figure). The cornea was clear without scarring, there was no uveitis, and intraocular pressure measured by Goldmann applanation tonometry was 15 mm Hg in each eye. There was no history of ocular trauma. Gonioscopy could not be performed because of the child’s sensitivity to a lens on the cornea. B-scan ultrasound of the left eye showed a normal retinal configuration and no vitreous opacities. After discussion with the patient’s family, the decision was made not to perform cataract surgery because of the poor visual prognosis.
Figure. (A) When the Patient Returned for Follow-Up at Almost 7 Years, a Complete White Cataract Was Present in the Affected Left Eye. The Anterior Capsule Overlying the Area of Previously Diagnosed Anterior Polar Cataract Was Disrupted, with Lens Tissue Protruding Anteriorly. There Was No Uveitis, and the Presence of Total White Cataract Was Confirmed After Full Pupillary Dilation. (B) The Anterior Extent of the Lens Tissue Can Be Appreciated with Slit-Beam Examination.
In the current patient, progressive growth of a congenital anterior polar lens opacity presumably led to focal disruption of the overlying associated anterior capsule and subsequent loss of lens clarity. Spontaneous rupture of the anterior capsule has been previously documented in children with anterior lenticonus (Alport syndrome)3,4 and adults with hypermature cataract.5,6 There is one case report of an adult with spontaneous anterior capsule rupture in the setting of anterior polar cataract7; in that patient, rupture of the anterior capsule was associated with uveitis, but not with significant lenticular opacification.7
Anterior polar cataract is the most common form of congenital anterior lens opacity.1 Congenital anterior lens opacities are usually static and visually insignificant; however, they can be associated with amblyopia.1,2 In a series of 63 children with congenital anterior lens opacities, there was a high incidence of amblyopia (approximately one-third of cases) associated with anisometropia or partial occlusion of the visual axis.1 However, only one patient in that series had cataract progression that necessitated surgery. Amblyopia was present in 17 of 59 patients (28.8%) in another study of congenital anterior lens opacity.2 Again, anisometropia was the most significant risk factor for amblyopia, and in that series, no patient had significant progression in the size of the lens opacity.
In contrast, a smaller series8 described five children with progressive bilateral congenital anterior lens opacity, all of whom needed cataract surgery because of progression. Two of the patients needed surgery for anterior pyramidal cataracts. Anterior pyramidal cataracts may be a more aggressive form of congenital anterior lens opacity, one that is more prone to growth and occlusive amblyopia.9 Both anterior polar cataract and anterior pyramidal cataract likely have the same etiology: imperfect separation of the lens from the surface ectoderm during the fifth week of gestation.9 The current patient had a clearly documented anterior polar cataract on initial examination and may have had features of anterior pyramidal cataract during the growth process.
In an English language PubMed search, the authors did not find previous documentation of anterior capsule rupture and total cataract formation for any form of congenital anterior lens opacity; this outcome was reported only in patients with Alport syndrome (anterior lenticonus)3,4 and adult hypermature cataract.5,6 There have been reports of both anterior polar cataract10 and anterior pyramidal cataract11,12 breaking off and moving into the anterior chamber. In the current case, no free-floating anterior capsular segment was visible in the anterior chamber, although gonioscopy was not performed.
Congenital anterior lens opacities are generally static, visually insignificant lesions. However, affected children must be assessed and observed for associated amblyogenic refractive errors and for the infrequent occurrence of progressive cataract formation.
- Jaafar MS, Robb RM. Congenital anterior polar cataract: a review of 63 cases. Ophthalmology. 1984;91:249–254.
- Ceyhan D, Schnall BM, Breckenridge A, Fontanarosa J, Lehman SS, Calhoun JC. Risk factors for amblyopia in congenital anterior lens opacities. J AAPOS. 2005;9:537–541. doi:10.1016/j.jaapos.2005.09.001 [CrossRef]
- Olitsky SE, Waz WR, Wilson ME. Rupture of the anterior lens capsule in Alport syndrome. J AAPOS. 1999;3:381–382. doi:10.1016/S1091-8531(99)70051-0 [CrossRef]
- Wilson ME Jr, Trivedi RH, Biber JM, Golub R. Anterior capsule rupture and subsequent cataract formation in Alport syndrome. J AAPOS. 2006;10:182–183. doi:10.1016/j.jaapos.2005.09.008 [CrossRef]
- Scott JG. Spontaneous rupture of the lens capsule. Br J Ophthalmol. 1953;37:58–60. doi:10.1136/bjo.37.1.58 [CrossRef]
- Sukhija J, Ram J, Brar GS, Bandhyopadhyaya S. Spontaneous rupture of the anterior lens capsule. Indian J Ophthalmol. 2006;54:216–217. doi:10.4103/0301-4738.27089 [CrossRef]
- Gaviria JG, Johnson DA, Scribbick FW III, Gallardo MJ. Spontaneous anterior capsular rupture associated with anterior polar cataract. Arch Ophthalmol. 2006;124:134–135. doi:10.1001/archopht.124.1.134 [CrossRef]
- Nelson LB, Calhoun JH, Simon JH, Harley RD. Progression of congenital anterior polar cataracts in childhood. Arch Ophthalmol. 1985;103:1842–1843.
- Wheeler DT, Mullaney PB, Awad A, Zwaan J. Pyramidal anterior polar cataracts. Ophthalmology. 1999;106:2362–2637. doi:10.1016/S0161-6420(99)90540-7 [CrossRef]
- Shah UN, Garg P, Vemuganti GK. Corneal edema with dislocated anterior polar cataract. Am J Ophthalmol. 2002;133:399–401. doi:10.1016/S0002-9394(01)01324-1 [CrossRef]
- Brown N, Ellis P. Anterior polar pyramidal cataract. Presenting as an anterior chamber foreign body. Br J Ophthalmol. 1972;56:57–59. doi:10.1136/bjo.56.1.57 [CrossRef]
- Thomas R, Gopal KS, George JA. Anterior dislocation of the pyramidal part of a congenital cataract. Indian J Ophthalmol. 1985;33:51–52.