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Retinal Whitening After Lung Transplant for Cystic Fibrosis

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his mid-20s with cystic fibrosis (CF) underwent bilateral lung transplant. Four weeks after the transplant, the patient developed acute vision loss of the left eye. Visual acuity was 20/20 OD and hand motion OS. Anterior segment was remarkable for 1+ hyperemia and 4+ anterior chamber (AC) cell in the left eye. Posterior segment was notable for retinal whitening and occlusive vasculitis in the right eye and more extensive retinal whitening associated with an exudative retinal detachment in the left eye. Microbial and broad-range polymerase chain reaction (PCR) analysis and culture results of the AC and vitreous were negative as well as numerous blood cultures and serum fungitell. Despite broad-spectrum intravenous (ie, vancomycin and piperacillin/tazobactam) and intravitreal antibiotics (vancomycin and ceftazidime), antifungals (amphotericin B and voriconazole), and antivirals (foscarnet), the patient’s visual acuity and intraocular inflammation worsened in both eyes to light perception. Over a week, the areas of retinal whitening became elevated owing to enlarging subretinal infiltrates (Figure, A) that were nearly kissing in the right eye (Figure, B). Owing to the diagnostic dilemma, a pars plana vitrectomy with subretinal biopsy was performed in the left eye, and again, microbiological analysis including culture and PCR analysis was inconclusive. Pain worsened, intraocular pressure elevated into the mid-40s mm Hg, visual acuity waned to no light perception, and intraocular inflammation and subretinal infiltrates continued to worsen, producing nearly closed funnel retinal detachments in both eyes, including developing orbital cellulitis in the right eye despite broad-spectrum antimicrobials. During this period, the patient remained afebrile; however, the patient’s white blood cell count was uptrending, raising concern for systemic spread of the infection.

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Multidrug-resistant Pseudomonas aeruginosa abscess of the subretinal space in both eyes

C. Perform a transscleral subretinal biopsy

Despite numerous blood cultures, multiple standard intraocular microbial tests, and PCR analysis being inconclusive, the differential diagnosis of bilateral subretinal infiltrates with overlying retinitis is an infectious etiology until proven otherwise, making choice A dangerous. Infectious causes of retinitis include herpes viruses, Candida, ocular manifestations of HIV, toxoplasmosis, and less commonly, syphilis, tuberculosis, and rare case reports of other agents and noninfectious mimickers.1,2 The rapid progression (during 2-3 weeks) was inconsistent with noninfectious causes, and a drug-resistant herpes virus seemed unlikely with negative PCR results and ongoing antiviral therapy. Continuing current therapy despite deterioration was unlikely to have a meaningful effect on the disease because it had failed to do so thus far, and continued administration may have led to retinal toxicity or intraocular complications, making choice B a poor choice. Owing to suspicion for a drug-resistant or atypical organism, a repeated subretinal biopsy was entertained. However, a vitrectomy with subretinal biopsy via a small-gauge subretinal cannula was deemed likely to fail owing to an inability to engage the organized, subretinal abscess and collect enough material for diagnosis.3 Furthermore, access to the posterior segment was limited owing to the subretinal abscess, obliterating the vitreous cavity in both eyes, making iatrogenic damage probable with a typical vitreoretinal approach. Therefore, choice D is not correct. Instead, a transscleral, ultrasonography-guided biopsy of the subretinal infiltrate by using a 25-gauge needle was performed (choice C), as had been described previously,4,5 and yellowish fluid teeming with multidrug-resistant Pseudomonas aeruginosa was isolated.

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Article Information

Corresponding Author: Thomas J. Wubben, MD, PhD, Kellogg Eye Center, University of Michigan, 1000 Wall St, Ann Arbor, MI 48105 (twubben@med.umich.edu).

Published Online: July 9, 2020. doi:10.1001/jamaophthalmol.2020.1306

Conflict of Interest Disclosures: Dr Demirci reported other support from Castle Bioscience and Immunocore outside the submitted work. No other disclosures were reported.

Funding/Support: Dr Conrady was supported in part by the Heed Ophthalmic Foundation.

Role of the Funder/Sponsor: The funding source had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Additional Contributions: We thank Eric Weinlander, MD, Kevin Gregg, MD, and Kevin Chan, MD, at the University of Michigan for their help with this difficult case. We thank the patient’s family for granting permission to publish this information.

References
1.
de Smet  MD .  Differential diagnosis of retinitis and choroiditis in patients with acquired immunodeficiency syndrome.   Am J Med. 1992;92(2A):17S-21S. doi:10.1016/0002-9343(92)90332-6PubMedGoogle ScholarCrossref
2.
Conrady  CD , Hanson  KE , Mehra  S , Carey  A , Larochelle  M , Shakoor  A .  The first case of trypanosoma cruzi-associated retinitis in an immunocompromised host diagnosed with pan-organism polymerase chain reaction.   Clin Infect Dis. 2018;67(1):141-143. doi:10.1093/cid/ciy058PubMedGoogle ScholarCrossref
3.
Grewal  DS , Cummings  TJ , Mruthyunjaya  P .  Outcomes of 27-gauge vitrectomy-assisted choroidal and subretinal biopsy.   Ophthalmic Surg Lasers Imaging Retina. 2017;48(5):406-415. doi:10.3928/23258160-20170428-07PubMedGoogle ScholarCrossref
4.
Motley  WW  III , Augsburger  JJ , Hutchins  RK , Schneider  S , Boat  TF .  Pseudomonas aeruginosa endogenous endophthalmitis with choroidal abscess in a patient with cystic fibrosis.   Retina. 2005;25(2):202-207. doi:10.1097/00006982-200502000-00014PubMedGoogle ScholarCrossref
5.
Andrew Machuk  RW , Bong  D , Baker  CF .  Case report: Choroidal abscess after double lung transplantation in a cystic fibrosis patient.   Can J Ophthalmol. 2016;51(1):e13-e16. doi:10.1016/j.jcjo.2015.09.013PubMedGoogle ScholarCrossref
6.
Rutter  WC , Burgess  DR , Burgess  DS .  Increasing incidence of multidrug resistance among cystic fibrosis respiratory bacterial isolates.   Microb Drug Resist. 2017;23(1):51-55. doi:10.1089/mdr.2016.0048PubMedGoogle ScholarCrossref
7.
Dua  S , Chalermskulrat  W , Miller  MB , Landers  M , Aris  RM .  Bilateral hematogenous Pseudomonas aeruginosa endophthalmitis after lung transplantation.   Am J Transplant. 2006;6(1):219-224. doi:10.1111/j.1600-6143.2005.01133.xPubMedGoogle ScholarCrossref
8.
Sheares  BJ , Prince  AS , Quittell  LM , Neu  NM , Bye  MR .  Pseudomonas aeruginosa endophthalmitis after lung transplantation for cystic fibrosis.   Pediatr Infect Dis J. 1997;16(8):820-821. doi:10.1097/00006454-199708000-00018PubMedGoogle ScholarCrossref
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