To our knowledge this is the first report in the literature of a patient with a background of tumoral calcinosis presenting with a sudden deterioration in vision due to choroidal neovascularisation (CNV), secondary to an angioid streak. It is interesting that the ocular features only became evident 14 years after diagnosis, though soft-tissue and vascular calcifications were observed at a much earlier stage. Similarly, a previous case in the literature reported that the ocular involvement only became apparent in a patient 29 years after diagnosis.14
Perhaps the ocular features manifesting at a later stage during the course of the disease are a feature of this syndrome.
Angioid streaks are small breaks in Bruch's membrane, an elastic membrane of the retina. They can lead to CNV with subsequent deterioration in VA. Previous treatments such as photodynamic therapy have yielded poor results, with most eyes showing poor visual outcomes in the long term.15
More recently, the anti-VEGF therapies using ranibizumab and bevacizumab have been used to treat CNV. Case reports in the literature have reported favourable results with these treatments, with most eyes showing a maintenance or improvement in VA with treatment, as in this case.16 17
A recent case report in the literature looking at the use of intravitreal ranibizumab for CNV secondary to angioid streaks found an improvement in VA after four injections, with VA remaining stable for 1 year after treatment.18
Ranibizumab appears to be an effective and promising treatment for CNV secondary to angioid streaks.
The fact that patients with tumoral calcinosis can develop angioid streaks and CNV indicates that they should be strongly advised to present urgently to an eye department, should any deterioration in vision occur. In our case, the presence of flame-shaped haemorrhages in the fellow eye indicates a need for close, long-term monitoring to determine whether similar complications develop in this eye.
Further study is required to determine the full spectrum of ocular involvement in this condition. Further investigation is also necessary to more accurately determine the immediate and long-term benefits of anti-VEGF therapies in the management of CNV secondary to angioid streaks, using larger numbers of patients.
Finally, though tumoral calcinosis is a rare condition, it is important for ophthalmologists to be aware of it as part of a differential diagnosis and to be aware of the potential complications that can arise from ocular involvement.
- Tumoral calcinosis is a rare disorder of phosphate metabolism, typically presenting with ectopic deposition of calcium in soft-tissue para-articular regions, dental pulp, skin and the vasculature. Ocular involvement includes calcific-lid lesions of the eyelids, conjunctival calcific deposits, band keratopathy and angioid streaks.
- The anti-VEGF therapies ranibizumab and bevacizumab appear to be an effective and promising treatment for CNV secondary to angioid streaks, including for patients with tumoral calcinosis.
- Patients with tumoral calcinosis should be routinely followed up by an ophthalmologist to help prevent visual deterioration and ocular complications from this condition.