Posterior keratoconus

Abstract

Posterior keratoconus (PKC) is a rare, typically non-inflammatory condition that is characterised by an abnormal posterior corneal curvature, which may be accompanied by overlying stromal opacification. It is usually congenital and can be associated with other ocular and systemic abnormalities. PKC remains a clinical diagnosis, although imaging techniques including ultrasound biomicroscopy and anterior segment optical coherence tomography may be useful tools for confirmation and classification. Genetic studies should be considered, although no specific genetic defects have been identified thus far. As a potential cause of amblyopia, early diagnosis and management are crucial in maximising visual potential. Occasionally, management considerations may also include surgical intervention, such as corneal transplantation. Intraocular lens power calculation at the time of cataract surgery requires special consideration. Here, we review the present literature on PKC and consider future directions in the management of this rare entity.

Posterior keratoconus in a patient with familial exudative vitreoretinopathy

“…[ 2 ] There is constellation of posterior segment features quoted in the literature that can occur along with posterior keratoconus with undetermined genetic linkage. [ 3 4 5 6 7 8 ] Our patient had a unique association of bilateral sporadic posterior keratoconus with FEVR, which to our knowledge never reported earlier. Atypical presentation of FEVR with associated retinal detachment and subtle posterior keratoconus makes our case a diagnostic challenge.…”

Salzmann nodular degeneration in posterior keratoconus: a case report

“…The PKC is usually congenital and can be associated with other ocular and systemic abnormalities, such as microcornea, synechiae, corectopia, anterior polar cataract, and so on [ 6 ]. The trauma has been sporadically proposed as an important cause of acquired PKC, as are an oblique penetrating injury leading to posterior lamellar splitting, destruction of the posterior surface, and keratohematoma [ 7 ].…”

“…Previous studies suggested that the histologic features of SND include attenuated epithelium, disruption of the Bowman layer, fibroblast overgrowth, and deposition of the extracellular matrix [ 3 – 5 ]. Posterior keratoconus (PKC) is a rare corneal developmental anomaly characterized by an entire or localized abnormality of the posterior corneal surface [ 6 ]. Also, cases acquired after trauma have been reported [ 7 ].…”

Bilateral posterior central corneal steepening with decreased visual acuity

“…Bilateral corneal opacities, increased localized central posterior corneal curvature, amblyopia, and a history of strabismus surgery before age 5 in this 40-year-old woman point to a clinical diagnosis of congenital posterior keratoconus (keratoconus posticus circumscriptus with central–paracentral involvement). 1 Complete ocular and systemic examination is a must to rule out coexisting abnormalities. Normal irides and gonioscopy imply lack of iridocorneal adhesions and angle abnormalities.…”

“…The most likely diagnosis of this patient is posterior keratoconus. 1–3 The Pentacam images show posterior steepening and the anterior segment OCT shows posterior corneal curvature abnormality. This condition is considered an anterior segment dysgenesis disorder and may be associated with systemic findings such as genitourinary abnormalities as in this case.…”