Phthisis Bulbi
Updated: Apr 14, 2021
Right eye loss of vision • Xray of the Week
What is the the name of the right globe abnormality?
Figure 1. Phthisis Bulbi on CT and MR Imaging
A. Axial CT scan of the orbits demonstrating the phthisis bulbi with dystrophic calcification (yellow arrow). The globe is small and deformed with enophthalmos.
B. Coronal CT scan of the orbits demonstrating the phthisis bulbi with dystrophic calcification (yellow arrow).
C and D. Axial MRI T1WI and Axial MRI T2WI of the orbits demonstrating the phthisis bulbi. Note the heterogeneous small, shrunken, calcified, deformed and irregularly shaped right globe, with enophthalmos (green arrow).
Figure 2. Phthisis bulbi clinical image in a different patient. Ophthalmic Atlas Image by EyeRounds.org, The University of Iowa is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.
Discussion:
Phthisis bulbi (PB) is a term given to an anatomically misshapen and atrophied ocular globe, secondary to severe injury or long-standing pathology. PB develops due to persistent inflammation and the eye becomes visibly disfigured and shrunken. Common etiologies of PB include penetrating trauma, radiation, infection, and tumor. After the inciting trauma or pathology, the affected eye undergoes profound inflammatory sequelae including proliferation of fibrosis and scarring. There are three stages leading to the progression of PB: stage I is atrophia bulbi without shrinkage, II is atrophia bulbi with shrinkage, III is atrophia bulbi with shrinkage and disorganization (phthisis bulbi) (1).
Patients can be asymptomatic in initial stages or present with a wide spectrum of pain, irritation and blindness. The most common features of PB include a small-sized asymmetrical affected eye with enophthalmos, corneoscleral scarring, hypotonia, and cataracts (Fig.2). End-stage PB can result in retinal detachment. Intraocular exam will demonstrate extremely low intraocular pressure, sometimes reaching 0 mmHg. (2)
On CT imaging the globe will be notably shrunken with increased attenuation in the thickened sclera due to diffuse scarring. Possible ossification and foci of dystrophic calcifications can often be seen and it is difficult to assess normal structures of the eye (Fig. 1). MRI will demonstrate various areas of increased signal depending on the degree of calcification in T1-weighted images. T2-weighted images of PB can show filling defects due to calcifications, and FLAIR sequence will exhibit increased signal in the damaged eye contrasting with the contralateral eye (Fig. 1). (3)
In the beginning stages of PB, patients can be given a topical steroid and cycloplegic agent to mild symptomatic relief but PB will continue to progress. Currently, the only permanent treatment for PB involves enucleation of the eye when pain becomes severe. Prosthetic rehabilitation of the affected eye has resulted in good cosmetic outcomes especially following enucleation. (1,2)
References:
1. Kaiser PK, Friedman NJ, Pineda R. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology. 4th ed. Saunders; 2014. https://bit.ly/2R7bbYG
2. Dohlman CH, D’Amico DJ. Can an Eye in Phthisis Be Rehabilitated? A Case of Improved Vision With 1-Year Follow-up. Archives of ophthalmology. 1999;117(1):123-124. doi:10.1001/archopht.117.1.123
3. Midyett FA, Mukherji SK. Phthisis bulbi. Orbital Imaging; 2015:29-31. doi:10.1016/B978-0-323-34037-3.00017-3
Nirali Dave is a medical student at Medical University of Lublin in Poland, currently doing clinical rotations in New York. Before that she completed her undergraduate education at Rutgers University, and worked as a medical scribe. Nirali was first exposed to basic radiologic imaging while scribing, and was very quickly taken by the field. Her passion for radiology comes from the bridging of anatomy, health technologies, and patient care. In the future, she hopes to complete a diagnostic radiology residency and stay committed to clinical research and patient education.
Follow Nirali Dave on Twitter @ndave08
Kevin M. Rice, MD is the president of Global Radiology CME
Dr. Rice is a radiologist with Renaissance Imaging Medical Associates and is currently the Vice Chief of Staff at Valley Presbyterian Hospital in Los Angeles, California. Dr. Rice has made several media appearances as part of his ongoing commitment to public education. Dr. Rice's passion for state of the art radiology and teaching includes acting as a guest lecturer at UCLA. In 2015, Dr. Rice and Natalie Rice founded Global Radiology CME to provide innovative radiology education at exciting international destinations, with the world's foremost authorities in their field. In 2016, Dr. Rice was nominated and became a semifinalist for a "Minnie" Award for the Most Effective Radiology Educator.
Follow Dr. Rice on Twitter @KevinRiceMD
