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Recurrent Subconjunctival Hemorrhage in a Child With an Orbital Mass

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

A well-appearing 14-month-old girl was referred for the evaluation of recurrent left subconjunctival hemorrhage with increasing frequency since 7 months of age. Multiple prior office visits with referring ophthalmologists during which eyes were dilated showed normal results. Clinical examination revealed 180° of left interior subconjunctival hemorrhage with overlying conjunctival elevation and mild fullness of the left lower eyelid (Figure 1A). Fixation was central, steady, and maintained bilaterally, with full ocular motility and absence of appreciable proptosis. Penlight anterior segment examination demonstrated normally formed anterior segment structures without inflammation or hyphema in either eye, although the left eye exhibited a small intraocular lightly pigmented lesion at the 5-o'clock limbus. Examination under anesthesia (EUA) was deemed appropriate and further demonstrated normal intraocular pressures bilaterally. Handheld slitlamp examination localized the left eye 2-mm limbal lesion on the posterior corneal surface. The surrounding corneal and iris structures were normal appearing without evidence of vascularization. Ultrasound biomicroscopy was used to further evaluate both the intraocular lesion and better characterize the left lower eyelid fullness. This demonstrated no iris, angle, or ciliary body involvement by the intraocular lesion. A 10-MHz contact B-scan examination showed a large inferior orbital lesion measuring more than 17 mm in the anterior-to-posterior dimension (Figure 1B). Doppler ultrasonography did not demonstrate significant flow to suggest a vascular cause.

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Metastatic neuroblastoma

C. Examine the abdomen during the EUA

The differential diagnosis of an orbital mass in a toddler includes dermoid cyst, infantile hemangioma, subperiosteal hematoma, orbital abscess, rhabdomyosarcoma, leukemia (chloroma), neuroblastoma, venous-lymphatic malformation, histiocytosis, and infantile fibromatosis.1 Aspiration of the lesion (choice A) would be useful in an abscess or a cystic lesion but not in a solid tumor. Ordering orbital magnetic resonance imaging would be a better choice than computed tomography (choice B) because of radiation concerns in a child. Referring the patient for an orbital biopsy (choice D) would be appropriate but could delay treatment in a potentially life-threatening lesion. An abdominal examination (choice C) detected a mass and immediately directed subsequent diagnostic testing and treatment because of the high probability of a metastatic neuroblastoma.

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Article Information

Corresponding Author: Roger P. Harrie, MD, John A. Moran Eye Center, The University of Utah, 65 Mario Capecchi Dr, Salt Lake City, UT 84132 (roger.harrie@hsc.utah.edu).

Published Online: November 5, 2020. doi:10.1001/jamaophthalmol.2020.4087

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s family for granting permission to publish this information.

References
1.
Shields  JA , Shields  CL .  Eyelid, Conjunctival, and Orbital Tumors: An Atlas and Textbook. Lippincott Williams & Wilkins; 2008.
2.
Bernstein  ML , Leclerc  JM , Bunin  G ,  et al.  A population-based study of neuroblastoma incidence, survival, and mortality in North America.   J Clin Oncol. 1992;10(2):323-329. doi:10.1200/JCO.1992.10.2.323 PubMedGoogle ScholarCrossref
3.
Smith  SJ , Diehl  NN , Smith  BD , Mohney  BG .  Incidence, ocular manifestations, and survival in children with neuroblastoma: a population-based study.   Am J Ophthalmol. 2010;149(4):677-682.e2, E2. doi:10.1016/j.ajo.2009.11.027PubMedGoogle ScholarCrossref
4.
Whittle  SB , Smith  V , Doherty  E , Zhao  S , McCarty  S , Zage  PE .  Overview and recent advances in the treatment of neuroblastoma.   Expert Rev Anticancer Ther. 2017;17(4):369-386. doi:10.1080/14737140.2017.1285230 PubMedGoogle ScholarCrossref
5.
Pugh  TJ , Morozova  O , Attiyeh  EF ,  et al.  The genetic landscape of high-risk neuroblastoma.   Nat Genet. 2013;45(3):279-284. doi:10.1038/ng.2529 PubMedGoogle ScholarCrossref
6.
Sait  S , Modak  S .  Anti-GD2 immunotherapy for neuroblastoma.   Expert Rev Anticancer Ther. 2017;17(10):889-904. doi:10.1080/14737140.2017.1364995PubMedGoogle ScholarCrossref
7.
Yu  AL , Gilman  AL , Ozkaynak  MF ,  et al; Children’s Oncology Group.  Anti-GD2 antibody with GM-CSF, interleukin-2, and isotretinoin for neuroblastoma.   N Engl J Med. 2010;363(14):1324-1334. doi:10.1056/NEJMoa0911123 PubMedGoogle ScholarCrossref
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 CME points in the American Board of Surgery’s (ABS) Continuing 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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