Posterior Lenticonus and Lentiglobus

Posterior lenticonus (lentiglobus) is characterized by a conical (spherical in lentiglobus) deformity of the posterior surface of the lens (Fig 9-4). This condition usually occurs as a sporadic unilateral anomaly and is associated with congenital cataract. Other, rare ocular associations include microphthalmia, microcornea, persistent fetal vasculature, and uveal colobomas. Posterior lenticonus may also be a manifestation of Alport syndrome (see previous section) or oculocerebrorenal syndrome (Lowe syndrome), an X-linked disorder characterized by glaucoma, cognitive impairment, and infantile renal tubulopathy (Fanconi type) with resultant aminoaciduria, metabolic acidosis, proteinuria, rickets, and hypotonia. Histologically, the cataracts display focal, internally directed excrescences of the lens capsule. Oculocerebrorenal syndrome is due to mutations in the OCRL gene.

Figure 9-4 Macroscopic image showing posterior lenticonus.

(Courtesy of Hans E. Grossniklaus, MD.)

Excerpted from BCSC 2020-2021 series: Section 4 - Ophthalmic Pathology and Intraocular Tumors. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.