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A Child With Acute Eyelid Edema and Proptosis

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

A 2-year-old girl presented to the emergency department with 2 days of left upper eyelid swelling that started 1 day after an episode of vomiting due to a viral illness. She had no associated fever, chills, upper respiratory tract symptoms, lethargy, or changes in behavior. She had an unremarkable ocular and medical history. On examination, visual acuity was fix-and-follow in each eye. Both eyes were soft to palpation. Pupils were round, symmetric, and reactive to light without an afferent pupillary defect. Ocular movements were full, and the patient had grossly full visual fields to confrontation. External examination revealed mild left-sided ptosis, axial proptosis, and upper eyelid fullness with no mass or tenderness to palpation (Figure, A). Slitlamp and fundus examination results were unremarkable, and there was no left optic nerve edema or pallor. Magnetic resonance imaging (MRI) of the orbits revealed a left intraconal mass that was isointense relative to rectus muscles on T1-weighted images and hypointense with a hyperintense rim on T2-weighted images (Figure, B).

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Orbital venolymphatic malformation with acute intralesional hemorrhage

B. Obtain computed tomographic scan of the orbits

The differential diagnosis for orbital masses in children differs from that in adults and includes dermoid cyst, vascular malformation, optic nerve glioma, rhabdomyosarcoma, and orbital metastasis. Neuroimaging modalities, such as MRI or computed tomography (CT), can be very helpful in differentiating among these lesion types. This patient’s MRI, however, did not show distinguishing features to assist in narrowing the differential diagnosis. It would be inappropriate to observe a new and undifferentiated orbital mass that could potentially be life threatening, vision threatening, or both (choice A). As recommended by the neuroradiology service, CT scanning was performed to further characterize the mass and make an accurate diagnosis (choice B). Although biopsy is the criterion standard for diagnosis, multimodal imaging can sometimes accurately identify a benign mass and make an invasive biopsy unnecessary (choice C). This minimizes the risk of complications such as hemorrhage, which is especially true in vascular lesions. Aggressive treatment with chemotherapy and radiotherapy should not be initiated until after a diagnosis is established because these treatments are appropriate only for certain malignant lesions, such as rhabdomyosarcoma, optic nerve glioblastoma, and orbital metastasis (choice D).

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Article Information

Corresponding Author: Kara M. Cavuoto, MD, Bascom Palmer Eye Institute, Attn: Mr. Jose Aponte, 900 NW 17th St, Miami, FL 33136 (kcavuoto@med.miami.edu).

Published Online: November 25, 2020. doi:10.1001/jamaophthalmol.2020.4067

Conflict of Interest Disclosures: None reported.

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

References
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Shields  JA , Shields  CL , Scartozzi  R .  Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1.   Ophthalmology. 2004;111(5):997-1008. doi:10.1016/j.ophtha.2003.01.002 PubMedGoogle ScholarCrossref
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Saha  K , Leatherbarrow  B .  Orbital lymphangiomas: a review of management strategies.   Curr Opin Ophthalmol. 2012;23(5):433-438. doi:10.1097/ICU.0b013e3283560a99 PubMedGoogle ScholarCrossref
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Khan  SN , Sepahdari  AR .  Orbital masses: CT and MRI of common vascular lesions, benign tumors, and malignancies.   Saudi J Ophthalmol. 2012;26(4):373-383. doi:10.1016/j.sjopt.2012.08.001 PubMedGoogle ScholarCrossref
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Gündüz  K , Kurt  RA , Erden  E .  Well-circumscribed orbital venous-lymphatic malformations with atypical features in children.   Br J Ophthalmol. 2009;93(5):656-659. doi:10.1136/bjo.2008.147488PubMedGoogle ScholarCrossref
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Rootman  J , Heran  MKS , Graeb  DA .  Vascular malformations of the orbit: classification and the role of imaging in diagnosis and treatment strategies.   Ophthalmic Plast Reconstr Surg. 2014;30(2):91-104. doi:10.1097/IOP.0000000000000122PubMedGoogle ScholarCrossref
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Nassiri  N , Rootman  J , Rootman  DB , Goldberg  RA .  Orbital lymphaticovenous malformations: current and future treatments.   Surv Ophthalmol. 2015;60(5):383-405. doi:10.1016/j.survophthal.2015.03.001 PubMedGoogle ScholarCrossref
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Barnacle  AM , Theodorou  M , Maling  SJ , Abou-Rayyah  Y .  Sclerotherapy treatment of orbital lymphatic malformations: a large single-centre experience.   Br J Ophthalmol. 2016;100(2):204-208. doi:10.1136/bjophthalmol-2015-306657 PubMedGoogle ScholarCrossref
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Hill  RH  III , Shiels  WE  II , Foster  JA ,  et al.  Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations.   Ophthalmic Plast Reconstr Surg. 2012;28(2):119-125. doi:10.1097/IOP.0b013e318242ab0f PubMedGoogle ScholarCrossref
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Sires  BS , Goins  CR , Anderson  RL , Holds  JB .  Systemic corticosteroid use in orbital lymphangioma.   Ophthalmic Plast Reconstr Surg. 2001;17(2):85-90. doi:10.1097/00002341-200103000-00002 PubMedGoogle ScholarCrossref
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Gandhi  NG , Lin  LK , O’Hara  M .  Sildenafil for pediatric orbital lymphangioma.   JAMA Ophthalmol. 2013;131(9):1228-1230. doi:10.1001/jamaophthalmol.2013.4201 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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