Red Cataract In Propionic Acidaemia

To report a unique case of a ‘red cataract’ and surgical extraction seen in a patient with propionic acidaemia.

 

Department of Ophthalmology, NHS Tayside, Dundee, Scotlant, United Kingdom

We present a case of a 26 year old male with the rare autosomal recessive disorder - propionic acidaemia. He presented with a gradual reduction vision in both eyes. We present our examination findings of bilateral red lenticular opacification. We also showcase a video recordings of the cataract extraction surgeries and discuss our learning points from them.

 

Although preoperative visual acuities were variable and unreliable due to learning difficulties, cataract extraction was indicated due to difficulties in activities of daily living. Slit lamp photos illustrated bilateral lenticular opacities with a prominent red hue. Cataract surgery was performed under sub-tenons local anaesthetic. Vision blue was used to help visualise the capsule. The red cataract was soft and minimal phaco-energey was required. We found it resembled a paediatric cataract extraction. There were no intraoperative complications. Post operatively, the patient and mother reported a significant improvement to the patient’s quality of life and visual alertness.

We were able to perform routine cataract surgery in a patient with propionic acidaemia with red lenticular opacification. There was a subjective improvement in the patients quality of life following cataract extraction.