Some morphological types of cataracts have a better prognosis than others. Anterior polar (Fig. 36.1), lamellar (Fig. 36.2), sutural (Fig. 36.3), and posterior lentiglobus (Fig. 36.4) cataracts are associated with the best visual prognoses, whereas dense, central, and posterior cataracts have poorer visual prognoses.⁴ Acquired cataracts generally have a more favorable visual prognosis than congenital cataracts.

Fig. 36.1 Anterior polar cataract. A small anterior polar cataract generally results in minimal visual deprivation, but is commonly associated with anisometropic amblyopia.

Fig. 36.2 Lamellar cataract.

Fig. 36.3 Sutural cataract.

Fig. 36.4 Bilateral posterior lentiglobus. This 8-month-old child presented with lentiglobus in both eyes. (A) There is a dense cataract overlying the lentiglobus in the left eye. (B) The lens cortex is still clear over the lentiglobus in the right eye.

Certain types of cataracts are frequently associated with other ocular abnormalities. For instance, nuclear cataracts are often associated with microphthalmos (Fig. 36.5) while autosomal dominant anterior polar cataracts (Fig. 36.6) are more commonly associated with corneal guttata or astigmatism. Anterior subcapsular and anterior capsular cataracts are often associated with severe skin diseases (syndermatotic cataract). Partially reabsorbed cataracts in an infant boy are suggestive of Lowe’s syndrome or Hallermann-Streiff syndrome. Wedge-shaped or sectional cataracts may occur with Stickler’s syndrome and Conradi’s syndrome and may be due to lyonization.

Fig. 36.5 Nuclear cataract. This child underwent a lensectomy and anterior vitrectomy in the right eye when 4 weeks of age. The lens was normal in the left eye. Both eyes had corneal diameters of 10 mm, but the axial length was only 15.74 mm in the right eye compared to 17.32 mm in the left eye.

Fig. 36.6 Autosomal dominant anterior polar cataracts. The child’s mother, sister, and brother also had bilateral anterior polar cataracts.

Persistent fetal vasculature

The term persistent fetal vasculature (PFV) describes a wide spectrum of congenital anomalies which commonly consist of a retrolental plaque (Fig. 36.7) in a microphthalmic eye with prominent blood vessels on the iris, a shallow anterior chamber, elongated ciliary processes, and occasionally intralenticular hemorrhages.⁵ Less commonly, these patients may present with a vascularized pupillary membrane, microcoria, and an anterior capsular cataract (Fig. 36.8).⁶ PFV is unilateral in 90% of patients. Although the lens may be clear initially, over time they usually become cataractous. The lens may undergo spontaneous absorption. In others, it becomes swollen, with loss of the anterior chamber and glaucoma. The fibrovascular plaque may bleed if cut. The retrolental plaque may contract with traction on the vitreous base and peripheral retina. Usually the posterior pole is normal, but fibrous tissue from the hyaloid remnants may cause peripapillary tractional retinal detachment. Conditions that can mimic PFV include retinoblastoma, retinopathy of prematurity, retinal dysplasia, and posterior uveitis. The presence of microphthalmos, a shallow anterior chamber, elongated ciliary processes, a cataract, and a retrolental opacity with a persistent hyaloid artery are all helpful in distinguishing PFV from these conditions.