BACKGROUND: Alport syndrome is an inherited Type IV collagenopathy characterised 
by renal failure, hearing loss and ophthalmic manifestations such as lenticonus 
and dot-and-fleck retinopathy. New signs have been described which can be useful 
both for diagnosis and for prognosticating the risk of complications. This study 
examines and describes a triad comprising the unusual 'stair-case' foveal sign, 
together with choroidal thinning and late-stage peripheral schisis in a patient 
with Alport syndrome.
CASE PRESENTATION: This is a case report of a 49-year-old Caucasian male with a 
background of X-linked Alport syndrome presenting with gradual and progressive 
diminution of vision in the left eye with a central blur. He had already 
undergone three renal allografts, was deaf and suffered from hypertension by the 
time of his first presentation to ophthalmology. On examination, corrected 
visual acuity was 6/9.5 in the right eye and 6/30 in the left eye. Optical 
coherence tomography imaging showed an unusual 'stair-case' sign of the fovea in 
both eyes, together with choroidal thinning. We postulate that an abnormal 
vitreomacular interface followed by vitreomacular traction and eventually 
separation, removing layers of the inner retina with the vitreous, led to this 
unusual appearance. Subsequently, this patient also developed schitic changes 
more peripherally in the retina which progressed over the following 5 years.
CONCLUSION: The stair-case foveal sign, choroidal thinning and mid-peripheral 
schisis are three signs that clinicians might expect to encounter on optical 
coherence tomography imaging of patients with Alport syndrome. These findings 
can be attributed to unique mutations of collagen IV which lead to a variety of 
clinical phenotypes affecting basement membrane structures. Identification of 
these features may not only be useful diagnostically and in forecasting 
complications such as macular holes, but also predict mode of inheritance and 
likelihood of early-onset renal failure.