Proptosis due to intraorbital space-occupying lesions in children

Graefe's Archive for Clinical and Experimental Ophthalmology volume 258, pages2541–2550(2020)Cite this article

Abstract

Purpose

To report the 10-year experience of two tertiary medical centers with children presenting with proptosis due to an intraorbital space-occupying lesion.

Methods

Patients were identified by file review. Data were collected on demographics, findings on ophthalmologic and imaging evaluations, etiology, treatment, and outcome.

Results

Nineteen children (7 male) were included. Eleven patients had optic nerve glioma, including 9 with substantially decreased visual acuity. Treatment consisted of chemotherapy alone or with radiation, resection or anti-VEGF agents, MEK inhibitor, or observation only (n = 1). Visual and cosmetic outcomes were poor in all cases. Outcome for arteriovenous malformations was good following corticosteroid treatment (n = 1), but catheterization led to persistent proptosis and fluctuating visual acuity (n = 1). Compound capillary hemangioma (n = 1) was treated with laser and systemic beta blockers with satisfactory results. Rhabdomyosarcoma had a good prognosis in one patient treated with resection and radiation but was fatal in another even after chemotherapy. Juvenile xanthogranuloma, frontal bone osteoma, and localized hypertrophic neuropathy of the supraorbital nerve (n = 1 each) were treated by resection with good visual and cosmetic outcomes.

Conclusions

Proptosis accompanied by visual loss is an uncommon presentation in children and suggests an orbital tumor. We found that visual outcome was better when the nerve was not involved by tumor. Optic nerve glioma was the most common cause and failed to respond to various treatments. Catheterization for arteriovenous malformation did not prevent proptosis, and final visual acuity fluctuated. Surgery for rhabdomyosarcoma and xanthogranuloma led to remission with preservation of vision in 2 of 3 cases.

This is a preview of subscription content, access via your institution.

Access options

Buy single article

Instant access to the full article PDF.

US$ 39.95

Tax calculation will be finalised during checkout.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

References

  1. 1.

    Khan SN, Sepahdari AR (2012) Orbital masses: CT and MRI of common vascular lesions, benign tumors, and malignancies. Saudi J Ophthalmol 26:373–383. https://doi.org/10.1016/j.sjopt.2012.08.001

    Article  PubMed  PubMed Central  Google Scholar 

  2. 2.

    Miller NR (2004) Primary tumours of the optic nerve and its sheath. Eye (Lond) 18:1026–1037. https://doi.org/10.1038/sj.eye.6701592

    Article  CAS  Google Scholar 

  3. 3.

    Glass LRD, Canoll P, Lignelli A et al (2014) Optic nerve glioma: case series with review of clinical, radiologic, molecular, and histopathologic characteristics. Ophthal Plast Reconstr Surg 30:372–376. https://doi.org/10.1097/IOP.0000000000000106

    Article  PubMed  Google Scholar 

  4. 4.

    Tow SL, Chandela S, Miller NR, Avellino AM (2003) Long-term outcome in children with gliomas of the anterior visual pathway. Pediatr Neurol 28:262–270

    Article  Google Scholar 

  5. 5.

    Spicer GJ, Kazim M, Glass LRD et al (2013) Accuracy of MRI in defining tumor-free margin in optic nerve glioma surgery. Ophthal Plast Reconstr Surg 29:277–280. https://doi.org/10.1097/IOP.0b013e318291658e

    Article  PubMed  Google Scholar 

  6. 6.

    Varan A, Batu A, Cila A et al (2013) Optic glioma in children: a retrospective analysis of 101 cases. Am J Clin Oncol 36:287–292. https://doi.org/10.1097/COC.0b013e3182467efa

    Article  PubMed  Google Scholar 

  7. 7.

    Dutton JJ (1994) Gliomas of the anterior visual pathway. Surv Ophthalmol 38:427–452

    Article  CAS  Google Scholar 

  8. 8.

    Nair AG, Pathak RS, Iyer VR, Gandhi RA (2014) Optic nerve glioma: an update. Int Ophthalmol 34:999–1005. https://doi.org/10.1007/s10792-014-9942-8

    Article  PubMed  Google Scholar 

  9. 9.

    Bessero A-C, Fraser C, Acheson J, Davagnanam I (2013) Management options for visual pathway compression from optic gliomas. Postgrad Med J 89:47–51. https://doi.org/10.1136/postgradmedj-2011-130414

    Article  PubMed  Google Scholar 

  10. 10.

    Shofty B, Ben-Sira L, Freedman S et al (2011) Visual outcome following chemotherapy for progressive optic pathway gliomas. Pediatr Blood Cancer 57:481–485. https://doi.org/10.1002/pbc.22967

    Article  PubMed  Google Scholar 

  11. 11.

    Warrier S, Prabhakaran VC, Valenzuela A et al (2008) Orbital arteriovenous malformations. Arch Ophthalmol 126:1669. https://doi.org/10.1001/archophthalmol.2008.501

    Article  PubMed  Google Scholar 

  12. 12.

    Gil-Salú JL, González-Darder JM, Vera-Román JM (2004) Intraorbital arteriovenous malformation: case report. Skull Base 14:31–36. https://doi.org/10.1055/s-2004-821358

    Article  PubMed  PubMed Central  Google Scholar 

  13. 13.

    Krema H (2015) Primary surgical excision for pediatric orbital capillary hemangioma. Semin Ophthalmol 30:214–217. https://doi.org/10.3109/08820538.2013.835841

    Article  PubMed  Google Scholar 

  14. 14.

    Tronina SA, Bobrova NF, Khrinenko VP (2008) Combined surgical method of orbital and periorbital hemangioma treatment in infants. Orbit 27:249–257. https://doi.org/10.1080/01676830802250101

    Article  PubMed  Google Scholar 

  15. 15.

    Hill RH, Shiels WE, Foster JA et al (2012) Percutaneous drainage and ablation as first line therapy for macrocystic and microcystic orbital lymphatic malformations. Ophthal Plast Reconstr Surg 28:119–125. https://doi.org/10.1097/IOP.0b013e318242ab0f

    Article  PubMed  Google Scholar 

  16. 16.

    Karcioglu ZA, Hadjistilianou D, Rozans M, DeFrancesco S (2004) Orbital rhabdomyosarcoma. Cancer Control 11:328–333. https://doi.org/10.1177/107327480401100507

    Article  PubMed  Google Scholar 

  17. 17.

    Eade E, Tumuluri K, Do H et al (2017) Visual outcomes and late complications in paediatric orbital rhabdomyosarcoma. Clin Exp Ophthalmol 45:168–173. https://doi.org/10.1111/ceo.12809

    Article  PubMed  Google Scholar 

  18. 18.

    Dagher R, Helman L (1999) Rhabdomyosarcoma: an overview. Oncologist 4:34–44. https://doi.org/10.1634/theoncologist.4-1-34

    Article  PubMed  CAS  Google Scholar 

  19. 19.

    Sultan I, Qaddoumi I, Yaser S et al (2009) Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol 27:3391–3397. https://doi.org/10.1200/JCO.2008.19.7483

    Article  PubMed  Google Scholar 

  20. 20.

    Ginat DT, Vargas SO, Silvera VM et al (2016) Imaging features of juvenile xanthogranuloma of the pediatric head and neck. AJNR Am J Neuroradiol 37:910–916. https://doi.org/10.3174/ajnr.A4644

    Article  PubMed  CAS  Google Scholar 

  21. 21.

    Newell GB, Stone OJ, Mullins JF (1973) Juvenile xanthogranuloma and neurofibromatosis. Arch Dermatol 107:262. https://doi.org/10.1001/archderm.1973.01620170070019

    Article  PubMed  CAS  Google Scholar 

  22. 22.

    Wei LA, Ramey NA, Durairaj VD et al (2014) Orbital osteoma: clinical features and management options. Ophthal Plast Reconstr Surg 30:168–174. https://doi.org/10.1097/IOP.0000000000000039

    Article  PubMed  CAS  Google Scholar 

  23. 23.

    Selva D, White VA, O’Connell JX, Rootman J (2004) Primary bone tumors of the orbit. Surv Ophthalmol 49:328–342. https://doi.org/10.1016/j.survophthal.2004.02.011

    Article  PubMed  Google Scholar 

  24. 24.

    Simmons Z, Mahadeen ZI, Kothari MJ et al (1999) Localized hypertrophic neuropathy: magnetic resonance imaging findings and long-term follow-up. Muscle Nerve 22:28–36

    Article  CAS  Google Scholar 

  25. 25.

    Laviv Y, Toledano H, Michowiz S et al (2012) BRAF, GNAQ, and GNA11 mutations and copy number in pediatric low-grade glioma. FEBS Open Bio 2:129–134. https://doi.org/10.1016/j.fob.2012.05.004

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  26. 26.

    Farazdaghi MK, Katowitz WR, Avery RA (2019) Current treatment of optic nerve gliomas. Curr Opin Ophthalmol 30:356–363. https://doi.org/10.1097/ICU.0000000000000587

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Funding

This study was partially supported by the Zanvyl and Isabelle Krieger Fund, Baltimore, MD, USA. The funding organization had no role in the design or conduct of this research.

Author information

Affiliations

  1. Sackler Faculty of Medicine, Tel Aviv University, 6997801, Tel Aviv, Israel

    Alon Zahavi, Geulah S. Ben-David & Helen Toledano

  2. Department of Ophthalmology, Rabin Medical Center, Beilinson Hospital, 4941492, Petah Tikva, Israel

    Alon Zahavi

  3. Krieger Eye Research Laboratory, Felsenstein Medical Research Center, 4941492, Petah Tikva, Israel

    Alon Zahavi & Nitza Goldenberg-Cohen

  4. Department of Radiology, Rabin Medical Center, Beilinson Hospital, 4941492, Petah Tikva, Israel

    Judith Luckman

  5. Department of Oncology, Schneider Children’s Medical Center of Israel, 49420235, Petah Tikva, Israel

    Helen Toledano

  6. Department of Neurosurgery, Hadassah Medical Center, 91120, Jerusalem, Israel

    Shalom Michowiz

  7. Department of Ophthalmology, Bnai-Zion Medical Center, 3339419, Haifa, Israel

    Yoav Vardizer & Nitza Goldenberg-Cohen

  8. Technion - Israel Institute of Technology, 3200003, Haifa, Israel

    Nitza Goldenberg-Cohen

Authors
  1. Alon Zahavi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Judith Luckman
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Geulah S. Ben-David
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Helen Toledano
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Shalom Michowiz
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Yoav Vardizer
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Nitza Goldenberg-Cohen
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors participated in all areas of study design and manuscript approval.

Corresponding author

Correspondence to Nitza Goldenberg-Cohen.

Ethics declarations

Conflict of interest

No conflicts of interest.

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the Rabin Medical Center and Bnai Zion Medical Center Institutional Ethics Committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Due to the retrospective nature of the study, informed consent was waived by the Rabin and Bnai Zion Medical Center Institutional Ethics Committee.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Zahavi, A., Luckman, J., Ben-David, G.S. et al. Proptosis due to intraorbital space-occupying lesions in children. Graefes Arch Clin Exp Ophthalmol 258, 2541–2550 (2020). https://doi.org/10.1007/s00417-020-04840-3

Download citation

Keywords

  • Tumor
  • Optic nerve
  • Optic nerve glioma
  • Chemotherapy
  • Surgery