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Orbital Swelling in a Child With Retinoblastoma Following Intra-arterial Chemotherapy

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

A 9-month-old girl presented to the emergency department with significant left periocular edema and erythema 3 days after receiving intra-arterial chemotherapy (IAC) with 0.4 mg/kg of melphalan, 50 mg of carboplatin, and 2 mg of topotecan (with routine intravenous dexamethasone therapy, 0.5 mg/kg) for treatment of unilateral International Classification of Retinoblastoma group C retinoblastoma in the left eye (Figure, A). At the time of IAC, she had an upper respiratory tract infection (coughing, congestion, rhinorrhea, fever [temperature of 38.9 °C]). On postoperative day 1, her temperature reached 39.4 °C, and she was diagnosed as having bilateral acute otitis media and discharged with amoxicillin therapy. On postoperative day 3, she returned to the emergency department with left eye swelling, continued fever, and poor oral intake. On physical examination, her temperature was 40.2 °C, and periorbital swelling and erythema were present without drainage. Dilated fundus examination at the bedside showed the known large retinoblastoma tumor. A large exudative retinal detachment was noted, which was not present prior to treatment. Blood bacterial culture testing showed no growth, respiratory viral testing revealed rhinovirus, and results of comprehensive metabolic testing were unremarkable. Complete blood cell count showed mild leukopenia (2700/μL [to convert to ×109 per liter, multiply by 0.001]) and anemia (hemoglobin, 9.2 g/dL [to convert to grams per liter, multiply by 10]) but were otherwise within reference ranges. Computed tomography scan of the orbits, recommended by the on-call general ophthalmologist, demonstrated preseptal and periorbital soft tissue swelling and edema with subtle retrobulbar stranding, without substantial proptosis, along with retinal detachment (Figure, B). She was admitted for intravenous fluids for her noted dehydration.

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A 9-month-old girl presented to the emergency department with significant left periocular edema and erythema 3 days after receiving intra-arterial chemotherapy (IAC) with 0.4 mg/kg of melphalan, 50 mg of carboplatin, and 2 mg of topotecan (with routine intravenous dexamethasone therapy, 0.5 mg/kg) for treatment of unilateral International Classification of Retinoblastoma group C retinoblastoma in the left eye (Figure, A). At the time of IAC, she had an upper respiratory tract infection (coughing, congestion, rhinorrhea, fever [temperature of 38.9 °C]). On postoperative day 1, her temperature reached 39.4 °C, and she was diagnosed as having bilateral acute otitis media and discharged with amoxicillin therapy. On postoperative day 3, she returned to the emergency department with left eye swelling, continued fever, and poor oral intake. On physical examination, her temperature was 40.2 °C, and periorbital swelling and erythema were present without drainage. Dilated fundus examination at the bedside showed the known large retinoblastoma tumor. A large exudative retinal detachment was noted, which was not present prior to treatment. Blood bacterial culture testing showed no growth, respiratory viral testing revealed rhinovirus, and results of comprehensive metabolic testing were unremarkable. Complete blood cell count showed mild leukopenia (2700/μL [to convert to ×109 per liter, multiply by 0.001]) and anemia (hemoglobin, 9.2 g/dL [to convert to grams per liter, multiply by 10]) but were otherwise within reference ranges. Computed tomography scan of the orbits, recommended by the on-call general ophthalmologist, demonstrated preseptal and periorbital soft tissue swelling and edema with subtle retrobulbar stranding, without substantial proptosis, along with retinal detachment (Figure, B). She was admitted for intravenous fluids for her noted dehydration.

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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

Article Information

Corresponding Author: Anthony B. Daniels, MD, MSc, Division of Ocular Oncology and Pathology, Department of Ophthalmology and Visual Sciences, Vanderbilt Eye Institute, Vanderbilt University Medical Center, 2311 Pierce Ave, Ste 2252, Nashville, TN 37205 (anthony.b.daniels@vumc.org).

Published Online: January 21, 2021. doi:10.1001/jamaophthalmol.2020.4675

Conflict of Interest Disclosures: Dr Froehler has received grants from the National Institutes of Health, Genentech, Medtronic, Stryker, Microvention, and Penumbra and personal fees from Genentech, Medtronic, Stryker, Balt, Cerenovus, Viz.ai, and Corindus. Dr Daniels has received grants from the National Eye Institute and Research to Prevent Blindness Foundation during the conduct of the study and grants from Spectrum Pharmaceuticals and Alcon Research Institute outside the submitted work; and has a patent with Vanderbilt University Medical Center. No other disclosures were reported.

Funding/Support: This work was supported by grant K08 EY 027464 from the National Eye Institute (Dr Daniels), by a Research to Prevent Blindness Career Development Award (Dr Daniels), and by an unrestricted grant from Research to Prevent Blindness to the Vanderbilt Department of Ophthalmology and Visual Sciences.

Role of the Funder/Sponsor: The funders 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 the mother of the patient for granting permission to publish this information.

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 credit toward the CME of the American Board of Surgery’s Continuous Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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