West Coast Retina

Case of the Month

Case of the Month Listing

Edited by

Robert N. Johnson, MD


March, 2012

A 53-year-old man with a two-day history of floaters in his left eye.

Presented by Paul Stewart, MD

Figures 1: Color photo montage of the right and left eye.  Note the areas of pigment clumping and atrophy in the periphery of each eye along retinal veins.

Case History

A 53-year-old Caucasian man presented with a two-day history of floaters and decreased vision in his left eye.  He noted difficulty in dark adaptation for the past five years but denied loss of peripheral vision.

His past medical history was noteworthy for a right-side cerebrovascular accident 2 years ago resulting in left lower leg paresis. Additionally, he suffered right 6th nerve palsy one-year ago that resolved within 3 weeks. He also had a history of kidney stones. Medications included simvastatin and Plavix.  His family history was unremarkable.

Examination revealed a best-corrected visual acuity of 20/20 in the right eye and 20/25 in the left eye.  His intraocular pressures were normal.  Extraocular motility and confrontational visual fields were full in both eyes.  Anterior segment examination of both eyes was remarkable only for mild nuclear sclerotic cataracts.

Fundus examination of the right eye showed clear ocular media and a normal optic disc without pallor.  There was no vascular attenuation (Figure 1 and 2).  The macula was normal (Figure 2 and 3)  In the periphery there were multiple areas of atrophy along with bone spicules following the course of retinal veins (Figure 1 and 4).

Fundus examination of the left eye (Figure 1) showed clear ocular media with a posterior vitreous detachment.  The optic disc was pink with sharp disc margins.  There was no vascular attenuation.  The macula was normal (Figures 2 and 3).  In the periphery there were multiple areas of atrophy along with bone spicule formation following the course of retinal veins (Figure 5 and 6) in a similar pattern to that seen in the right eye.  In the periphery there was vitreous traction associated with the pigment changes (Figure 6) without retinal tear or subretinal fluid.

Figure 2: Color photos of the right and left macula showing a normal appearing macula, nerve and retinal vessels.  A focal area of atrophy and pigment clumping is present along the inferotemporal vein in the right eye.

Figure 3: Horizontal SD-OCT scan through the right and the left macula are normal.

Figure 4: Color fundus photos of the temporal periphery of the right eye. Note the areas of bone-spicule pigment clumping and atrophy along multiple retinal veins.

Figure 5: Color fundus photo of the superior periphery of the left eye. Note areas of bone-spicule pigmentation and atrophy along retinal veins.

Figure 6: Color fundus photo of the superonasal retina of the left eye. Note the similar areas of bone-spicule pigmentation and atrophy along retinal veins.

What is your Diagnosis?

Differential Diagnosis

This 53-year-old man presented with an incidental symptomatic posterior vitreous detachment and was found to have asymptomatic retinal pigment changes in both eyes.

The differential diagnosis can be considered in several categories of disorders including degenerative, hereditary, infectious and inflammatory diseases. These disorders would include: pigmented paravenous chorioretinal atrophy, retinitis pigmentosa, gyrate atrophy, serpiginious choroidopathy, angioid streaks, sarcoidosis, syphilis, acute retinal necrosis, cytomegalovirus retinitis, tuberculous, choroiditis, and toxoplasmosis.



This case represents an example of pigmented paravenous chorioretinal atrophy (PPCA).  PPCA was first described in 1937 in a 47 year old man with alopecia areata whose fundus was incidentally noted to have the increased pigmentation along peripheral veins associated with chorioretinal atrophy.1  The appearance was termed retinochoroiditis radiata.  The patient had a positive reaction to tuberculin and it was postulated that the fundus appearance was a consequence of tuberculous periphlebitis.

In 1948, syphilis was postulated as a cause.2 The current term for this entity was first applied in 1962 by Franceschetti.3 There have been approximately one-hundred cases reported with a preponderance of male involvement (90 percent), and less than 10 percent involving the macula.4  The disease has variably been described as a congenital process, a primary degeneration, or a consequence of prior inflammation.  There are several reports with familial involvement and a recent report of a family with dominant inheritance and a mutation in the crumbs 1 homologue (CRB1) gene.5 CRB1 mutations have also been associated with certain forms of retinitis pigmentosa and Leber congenital amaurosis.  The majority of patients are asymptomatic though some have focal visual field defects corresponding to the areas of involvement and some have abnormal ERG findings.6  The heterogeneity of described cases and variable associations may indicate that the typical fundus picture of PPCA is a final common pathway of several disorders.

In our case, the patient’s nerves were pink and sharp without waxy pallor, his vessels were of normal caliber, and his 30-2 Humphrey visual fields were normal.  These findings made a diagnosis of retinitis pigmentosa unlikely.  He had no vitreous cell and no evidence of active inflammation or vasculitis, making an infectious process such as ARN or TB less likely.  The absence of inflammation also decreased the probability that an inflammatory process like sarcoidosis was the cause.

Our patient presented because of decreased vision and floaters due to an acute posterior vitreous detachment.  The finding of pigmented paravenous chorioretinal atrophy was incidental.  The traction seen along the vessels with pigmentary changes had not been previously reported to be a feature of PPCA and we will continue to monitor for further evolution of the natural history of this entity.

Take Home Points

  • Pigmented paravenous chorioretinal atrophy is usually discovered incidentally and in most cases is asymptomatic.
  • The pathogenesis is unknown and the fundus findings may represent a final common pathway of several different entities

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  1. Hewitson-Brown T.  Retinochoroiditis radiate. Br J Ophthalmol. 1937. 21: 645.
  2. Chi Hsin-Hsing. Retinal choroiditis radiate. Am J Ophthalmol. 1948. 31: 1485.
  3. Franceschetti A. A curious affection of the fundus oculi. Helicoid peripapillary chorioretinal degeneration. It’s relation to pigmented paravenous chorioretinal degeneration. Doc Ophthalmol. 1962. 16: 81.
  4. Murray AT, Kirkby GR. Pigmented paravenous chorioretinal atrophy: a literature review supported by a unique case and insight. Eye. 2000. 14: 711.
  5. McKay GJ, Clarke S, Davis JA, Simpson DAC, Silvestri G. Pigmented paravenous chorioretinal atrophy is associated with a mutation within the crumbs homolog 1 (CRB1) gene. IOVS. 2005.  46: 322.
  6. Kukner AS, Yilmaz T, Celebi S, Aydemir O, Ulas F. Pigmented paravenous retinochoroidal atrophy: a literature review supported by seven cases. Ophthalmologica. 2003. 217: 436.