About MEAJO | Editorial board | Search | Ahead of print | Current Issue | Archives | Instructions to authors | Online submission | Subscribe | Advertise | Contact | Login 
Middle East African Journal of Ophthalmology Middle East African Journal of Ophthalmology
Users Online: 5436   Home Print this page Email this page Small font sizeDefault font sizeIncrease font size

  Table of Contents 
Year : 2018  |  Volume : 25  |  Issue : 2  |  Page : 108-110  

Hand-held optical coherence tomography monitoring of submillimeter retinoblastoma treated with indocyanine green-enhanced transpupillary therapy

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA 19107, USA

Date of Web Publication18-Jul-2018

Correspondence Address:
Dr. Carol L Shields
The Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 840 Walnut Street, 14th Floor, Philadelphia, PA 19107
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/meajo.MEAJO_280_17

Rights and Permissions

Over recent years, hand-held optical coherence tomography (HH-OCT) has become critical for retinoblastoma diagnosis and management. We report precise HH-OCT findings in a case of sub-millimeter retinoblastoma treated with foveal-sparing indocyanine green-enhanced transpupillary thermotherapy (ICG-TTT). A 2-month-old Caucasian female with bilateral Group B retinoblastoma showed two recurrent macular tumors in the right eye, demonstrating 88 μm and 37 μm of growth to 344 μm and 413 μm in thickness, respectively, on HH-OCT. Each was treated with additional intravenous chemotherapy and foveal-sparing ICG-TTT. Tumor regression to 154 μm and 224 μm was documented on HH-OCT and maintained on follow-up. HH-OCT is vital in confirming clinical findings and influencing management decisions in retinoblastoma. In this case, HH-OCT precisely documented submillimeter retinoblastoma recurrence and treatment response.

Keywords: Chemotherapy, indocyanine green, optical coherence tomography, retina, retinoblastoma, transpupillary thermotherapy, transpupillary thermotherapy

How to cite this article:
Spencer MA, Welch R J, Shields CL. Hand-held optical coherence tomography monitoring of submillimeter retinoblastoma treated with indocyanine green-enhanced transpupillary therapy. Middle East Afr J Ophthalmol 2018;25:108-10

How to cite this URL:
Spencer MA, Welch R J, Shields CL. Hand-held optical coherence tomography monitoring of submillimeter retinoblastoma treated with indocyanine green-enhanced transpupillary therapy. Middle East Afr J Ophthalmol [serial online] 2018 [cited 2021 Oct 27];25:108-10. Available from: http://www.meajo.org/text.asp?2018/25/2/108/237034

   Introduction Top

Retinoblastoma is a highly malignant intraocular tumor, typically found in young children. The mainstay of retinoblastoma diagnosis and management is clinical acumen; however, several diagnostic modalities, such as ultrasonography, fluorescein angiography, and optical coherence tomography (OCT) are employed.[1] Portable hand-held OCT (HH-OCT) has become important in day-to-day retinoblastoma management. HH-OCT has been found to identify clinically “invisible” retinoblastoma, document tumor recurrence, and monitor tumor response to various therapies.[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18] In addition, HH-OCT of the foveola has been helpful in estimating visual acuity in preverbal retinoblastoma patients.[18]

Herein, we report the HH-OCT characteristics of two submillimeter parafoveal retinoblastomas that demonstrated <100 um growth, detected early and treated rapidly with chemotherapy and indocyanine green-enhanced transpupillary thermotherapy (ICG-TTT), in order to successfully protect the infant's foveal anatomy and ultimate visual acuity.

   Case Report Top

A 2-month-old Caucasian female with known germline retinoblastoma mutation and fix and follow vision in both eyes (OU) was discovered to have bilateral Group B retinoblastoma using the International Classification of Retinoblastoma. There was no leukocoria or strabismus. She received 3 cycles of intravenous chemotherapy (IVC) using vincristine, etoposide, and carboplatin (VEC) and was referred for further management.

Anterior segment and intraocular pressure were normal OU. Fundus examination of the right eye (OD) revealed two noncalcified macular retinoblastomas measuring 256 μm and 376 μm in thickness by HH-OCT [Figure 1]a, [Figure 1]b, [Figure 1]c. There was a larger partially calcified retinoblastoma nasal to the optic disc OD measuring 4 mm × 4 mm × 3 mm. Fundus examination of the left eye (OS) disclosed a partially calcified white macular retinoblastoma measuring 3 mm × 3 mm × 2.5 mm. There was no subretinal fluid, subretinal seeding, or vitreous seeding OU. These findings were consistent with partially regressed Group B retinoblastoma OU.
Figure 1: Fundus photography and hand-held optical coherence tomography images of two sub-millimeter retinoblastomas in the macula of the right eye. The dotted lines on the images represent hand-held optical coherence tomography b-scan orientation through each of the tumors. (a) Fundus photography initially showed 2 barely visible sub-millimeter retinoblastomas in the macula superiorly and nasally. (b) Hand-held optical coherence tomography of the superior macular tumor demonstrated hyperreflective retinal mass measuring 256 μm in thickness. (c) Hand-held optical coherence tomography of the nasal macular tumor demonstrated a small retinoblastoma measuring 376 μm in thickness. (d) Fundus photography of the right eye with clinically imperceptible growth of 2 sub-millimeter retinoblastomas. (e) Hand-held optical coherence tomography demonstrated retinoblastoma growth of 88 μm in thickness to 344 μm. (f) Hand-held optical coherence tomography demonstrated minor growth of 37 μm in tumor thickness to 413 μm. (g) Fundus photography of the right eye with chorioretinal scarring status post 3 sessions of foveal-sparing indocyanine green-enhanced transpupillary thermotherapy. (h) Hand-held optical coherence tomography demonstrated tumor regression to thickness of 154 μm with retinal and choroidal atrophy. (i) Hand-held optical coherence tomography with regressed retinoblastoma measuring 224 μm in thickness with retinal and choroidal atrophy

Click here to view

Treatment with 3 additional cycles of VEC was given and the extra-macular tumors OU were consolidated with ICG-TTT. ICG was employed for enhanced diode laser uptake in this blonde fundus. Two months following completion of IVC, retinoblastoma recurrence was noted in OU. In the OS, there was recurrent macular tumor and two new peripheral tumors, so intra-arterial chemotherapy using melphalan 5 mg was prescribed, with rapid tumor control but with choroidal ischemia.

In the OD, the two macular tumors demonstrated recurrence, near the foveola and threatening vision [Figure 1]d. By HH-OCT, there was 88 μm increase in thickness to 344 μm in the superior macular tumor [Figure 1]e and 37 μm increase in thickness to 413 μm in the nasal macular tumor [Figure 1]f, so further IVC (3 cycles) plus foveal-sparing ICG-TTT was provided. After treatment [Figure 1]g, HH-OCT confirmed regression of the superior macular tumor with thickness decreasing from 344 μm to 154 μm [Figure 1]h. The nasal macular tumor showed a reduction from 413 μm to 224 μm in thickness [Figure 1]i. At the last follow-up, 12 months following initial presentation, all tumors remain regressed. Fortunately, the foveal contour on HH-OCT was preserved, and the remainder of the retina was intact OD.

   Discussion Top

In retinoblastoma care, HH-OCT has proven useful in confirming clinical findings and influencing management decisions.[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18] In a recent report by Soliman et al.[6] that summarizes the use of OCT in 44 children with retinoblastoma, HH-OCT was found to influence the management of retinoblastoma by either confirming clinical findings (83% of OCT sessions) or changing the treatment course (17% of OCT sessions). In the case presented here, HH-OCT verified the clinical suspicion of submillimeter retinoblastoma growth OD and influenced the decision to treat with ICG-TTT. In addition, HH-OCT helped monitor response to treatment and titrated the number of ICG-TTT sessions based on the appearance of retinoblastoma by HH-OCT.

ICG-TTT is used to treat retinoblastoma that shows suboptimal response to conventional treatment or in eyes where the fundus is minimally pigmented.[19],[20],[21] Hasanreisoglu et al.[19] reported 42 retinoblastomas in 30 eyes of 21 patients that received ICG-TTT treatment and 79% of tumors showed complete regression after a median of 2 treatment sessions. Francis et al.[20] and Al-Haddad et al.[21] have also reported on ICG-TTT in retinoblastoma. In our patient, the minimally pigmented background fundus and previous demonstration of chemoresistance of the macular tumors OD confirmed on HH-OCT, influenced the decision to employ ICG-TTT.

HH-OCT is vital in retinoblastoma care. Herein, HH-OCT was used to confirm retinoblastoma recurrence influencing the decision to provide further treatment, and it also corroborated tumor response to therapy. HH-OCT serves an important role for retinoblastoma management, particularly in tracking submillimeter tumors.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This study was support by Eye Tumor Research Foundation, Philadelphia, PA (CLS)

Conflicts of interest

There are no conflicts of interest

   References Top

Shields CL, Lally SE, Leahey AM, Jabbour PM, Caywood EH, Schwendeman R, et al. Targeted retinoblastoma management: When to use intravenous, intra-arterial, periocular, and intravitreal chemotherapy. Curr Opin Ophthalmol 2014;25:374-85.  Back to cited text no. 1
Shields CL, Manalac J, Das C, Saktanasate J, Shields JA. Review of spectral domain-enhanced depth imaging optical coherence tomography of tumors of the retina and retinal pigment epithelium in children and adults. Indian J Ophthalmol 2015;63:128-32.  Back to cited text no. 2
[PUBMED]  [Full text]  
Bremner R. Retinoblastoma, an inside job. Cell 2009;137:992-4.  Back to cited text no. 3
Dimaras H, Corson TW, Cobrinik D, White A, Zhao J, Munier FL, et al. Retinoblastoma. Nat Rev Dis Primers 2015;1:15021.  Back to cited text no. 4
Rootman DB, Gonzalez E, Mallipatna A, Vandenhoven C, Hampton L, Dimaras H, et al. Hand-held high-resolution spectral domain optical coherence tomography in retinoblastoma: Clinical and morphologic considerations. Br J Ophthalmol 2013;97:59-65.  Back to cited text no. 5
Soliman SE, VandenHoven C, MacKeen LD, Héon E, Gallie BL. Optical coherence tomography-guided decisions in retinoblastoma management. Ophthalmology 2017;124:859-72.  Back to cited text no. 6
Mallipatna A, Vinekar A, Jayadev C, Dabir S, Sivakumar M, Krishnan N, et al. The use of handheld spectral domain optical coherence tomography in pediatric ophthalmology practice: Our experience of 975 infants and children. Indian J Ophthalmol 2015;63:586-93.  Back to cited text no. 7
[PUBMED]  [Full text]  
Saktanasate J, Vongkulsiri S, Khoo CT. Invisible retinoblastoma. JAMA Ophthalmol 2015;133:e151123.  Back to cited text no. 8
Berry JL, Cobrinik D, Kim JW. Detection and intraretinal localization of an 'invisible' retinoblastoma using optical coherence tomography. Ocul Oncol Pathol 2016;2:148-52.  Back to cited text no. 9
Palazzi MA, de Abreu HFH, de Freitas ACLH, Quagliato LB, de Freitas JAH. Optical coherence tomography in the evaluation of macular retinoblastoma. Rev Bras Oftalmol 2015;74:275-8.  Back to cited text no. 10
Hasanreisoglu M, Dolz-Marco R, Ferenczy SR, Shields JA, Shields CL. Spectral domain optical coherence tomography reveals hidden fovea beneath extensive vitreous seeding from retinoblastoma. Retina 2015;35:1486-7.  Back to cited text no. 11
Fuller TS, Alvi RA, Shields CL. Optical coherence tomography of cavitary retinoblastoma. JAMA Ophthalmol 2016;134:e155355.  Back to cited text no. 12
Yousef YA, Shroff M, Halliday W, Gallie BL, Héon E. Detection of optic nerve disease in retinoblastoma by use of spectral domain optical coherence tomography. J AAPOS 2012;16:481-3.  Back to cited text no. 13
Lee H, Proudlock FA, Gottlob I. Pediatric optical coherence tomography in clinical practice-recent progress. Invest Ophthalmol Vis Sci 2016;57:OCT69-79.  Back to cited text no. 14
Gonzalez-Montpetit ME, Samara WA, Magrath GN, Shields CL. Detection of minimally visible recurrent retinoblastoma by hand-held spectral-domain optical coherence tomography. J Pediatr Ophthalmol Strabismus 2017;54:e6-8.  Back to cited text no. 15
Park K, Sioufi K, Shields CL. Clinically invisible retinoblastoma recurrence in an infant. Retin Cases Brief Rep 2017. [Epub ahead of print].  Back to cited text no. 16
Berry JL, Anulao K, Kim JW. Optical coherence tomography imaging of a large spherical seed in retinoblastoma. Ophthalmology 2017;124:1208.  Back to cited text no. 17
Samara WA, Pointdujour-Lim R, Say EA, Shields CL. Foveal microanatomy documented by SD-OCT following treatment of advanced retinoblastoma. J AAPOS 2015;19:368-72.  Back to cited text no. 18
Hasanreisoglu M, Saktanasate J, Schwendeman R, Shields JA, Shields CL. Indocyanine green-enhanced transpupillary thermotherapy for retinoblastoma: Analysis of 42 tumors. J Pediatr Ophthalmol Strabismus 2015;52:348-54.  Back to cited text no. 19
Francis JH, Abramson DH, Brodie SE, Marr BP. Indocyanine green enhanced transpupillary thermotherapy in combination with ophthalmic artery chemosurgery for retinoblastoma. Br J Ophthalmol 2013;97:164-8.  Back to cited text no. 20
Al-Haddad CE, Abdulaal M, Saab RH, Bashshur ZF. Indocyanine green-enhanced thermotherapy for retinoblastoma. Ocul Oncol Pathol 2015;1:77-82.  Back to cited text no. 21


  [Figure 1]


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
   Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded107    
    Comments [Add]    

Recommend this journal