Congenital cataract 82 eyes No associated findings 56 Microcornea 12 Persistent fetal vasculature (PFV) 3 PFV and microphthalmia 1 PFV, microphthalmia, and posterior 1 synechiae Lentiglobus and lenticonus
2 Flat anterior chamber, protruding 1 iris, and posterior synechiae Abnormal greyish anterior sclera 2 Complex anterior segment dysgenesis 2 Aniridia (PAX6 mutation) 2 Juvenile cataract 12 eyes Marden-Walker syndrome 2 Muscular dystrophy 2 Post laser coagulation for ROP 3 Juvenile rheumatic arthritis associated O with chronic anterior uveitis 2 Posttraumatic cataract 2 Posttraumatic cataract and anterior 1 lens capsule perforation 1 Table 2: Ocular anomalies and systemic diagnosis of the affected patients (20/63) with cataract are listed.
Cataract exhibits a variety of morphologies that comprises anterior polar, pyramidal, anterior lenticonus
, cortical lamellar, fetal nuclear, posterior polar, posterior lentiglobus, posterior subcapsular, persistent fetal vasculature (PFV) and traumatic disruption of lens.
AS has been associated with renal, auditory, and ocular disease owing to the presence of alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina resulting in spectrum of hereditary nephritis , corneal opacities, anterior lenticonus
, fleck retinopathy, temporal retinal thinning , and sensorineural deafness .
Also, patients with any local ocular disease such as glaucoma, lenticonus
, or retinitis or anyone with demyelinating conditions such as multiple sclerosis were excluded.
Wright KW19 et al study reviews the results were good in patients with persistent hyperplastic primary vitreous, posterior lenticonus
, and bilateral cataracts.
The topics include highlights on statistics and clinical research, new operative tools to improve the structure, wavefront aberrations from A to Z, corneal aberrometry changes following intra-corneal ring segment implantation, wavefront analysis and Scheimpflug imagery in the diagnosis of anterior lenticonus
, and wavefront basic images.
This loss of collagen IV organization is likely to contribute to the phenotype of these lenses as mutations in the COL4A1 gene cause anterior segment defects [58, 59], while mutations in the COL4A3 or COL4A4 genes result in Alport Syndrome in humans, which is associated with anterior and posterior lenticonus
, capsular ruptures, and cataracts [59-64].
Past medical history includes adulthood onset, autosomal recessive type AS, due to a missense mutation in the COL4A3 gene, with development of severe renal insufficiency, hypertension, anterior lenticonus
and mild sensorineural deafness for 4 years.
(1-3) Patients with Alport syndrome often, although not always, exhibit systemic manifestations, the most common of which are hearing impairments (1,4,5) and ocular defects, most typically anterior lenticonus
Pre Existing Conditions: Mature/hypermature cataract, pre-operative zonular weakness, previous trauma, posterior polar cataract, subluxated lens, pseudoexfoliation, posterior lenticonus
, small pupil, post complicated posterior segment surgeries and poor visibility due to corneal scarring/dense arcus.
Slit lamp examination of eyes revealed anterior lenticonus
in right eye and pseudophakia in left eye after cataract surgery.