*2.6.3.1 Photography*

It is important to obtain photographs of both the anterior segment as well as the vitreous cavity and posterior segment for documentation. The most useful method is using a wide-angle fundus camera. Photographs should be obtained during every EUA to help document response to therapy (**Figures 4**, **5A**, **B**, and **6A**, **B**). The clinician should try to standardize the photographs, so comparisons to each exam can be readily performed. As an example, start with a posterior pole photograph centered on the fovea then sequentially take peripheral photos superiorly, inferiorly, temporally and nasally keeping the optic nerve visible at the respective edge of the photograph to use as a point of reference for peripheral lesions. Then any other areas of special interest can be photographed separately.

**33**

**Figure 5.**

*chemotherapy.*

**Figure 3.**

**Figure 4.**

*2.6.3.2 Fluorescein angiography*

Thanks to advances in photography as described in the above section, fluorescein angiography (FA) can be readily performed during an EUA and can help one

*(A) RetCam photograph of class C retinoblastoma of the right eye prior to intra-arterial chemotherapy and (B) RetCam photograph of the right eye showing class C retinoblastoma after treatment with intra-arterial* 

*Retinoblastoma: Presentation, Evaluation, and Diagnosis DOI: http://dx.doi.org/10.5772/intechopen.85744*

*RetCam photography of the left eye with defocusing to document free-floating vitreous seeding.*

*A retinoblastoma of the left eye with tumor scar showing type 3 regression.*

*Retinoblastoma: Presentation, Evaluation, and Diagnosis DOI: http://dx.doi.org/10.5772/intechopen.85744*

#### **Figure 3.**

*Retinoblastoma - Past, Present and Future*

prematurity rather than RB.

existing classification schemes at a later time.

areas of special interest can be photographed separately.

*2.6.3 Ancillary testing*

*2.6.3.1 Photography*

both eyes.

**Figure 2.**

extending towards the periphery of the lens which could be secondary to exophytic tumor, whereas the persistent tunica vasculosa lentis in PFV, vessels should be noted to be growing towards the center of the posterior lens in a disorganized fashion. Retrolental membrane without vascular pattern is suggestive of retinopathy of

*External photograph of young child with leukocoria of the left eye secondary to intraocular retinoblastoma.*

Binocular indirect ophthalmoscopy should then be performed in a step by step fashion. Initially the vitreous should be examined in both eyes for presence of seeding, vitreous hemorrhage, fibrous membranes, or inflammatory debris. If the posterior pole is visible, optic disc and macula should be examined and abnormal findings documented. Examination of the periphery should be performed with scleral depression of the ora serrata in a clockwise or counter-clockwise fashion, with examination of the midperipheral retina and posterior pole for 360 degrees in

Small RB lesions can be difficult to detect as there can be poor contrast between the small translucent tumor and the surrounding fundus. Meticulous examination and depression is needed to observe these tumors stereoscopically. Medium sized tumors become more opaque and start appearing white. Large tumors (>4–5 mm) in diameter will begin to have visually obvious blood supply and visually significant feeder vessels should be noted. Many very large lesions (>6 mm) will develop chalky-white calcifications within the body of the tumor. The size and number of all tumors should be noted, with evaluation of subretinal fluid, retinal detachment, and presence of seeding (subretinal or vitreous) (**Figure 3**), and should all be incorporated into a detailed retinal drawing along with fundus photography [12]. Drawing and photography can help the clinician classify the tumor according to

It is important to obtain photographs of both the anterior segment as well as the vitreous cavity and posterior segment for documentation. The most useful method is using a wide-angle fundus camera. Photographs should be obtained during every EUA to help document response to therapy (**Figures 4**, **5A**, **B**, and **6A**, **B**). The clinician should try to standardize the photographs, so comparisons to each exam can be readily performed. As an example, start with a posterior pole photograph centered on the fovea then sequentially take peripheral photos superiorly, inferiorly, temporally and nasally keeping the optic nerve visible at the respective edge of the photograph to use as a point of reference for peripheral lesions. Then any other

**32**

*RetCam photography of the left eye with defocusing to document free-floating vitreous seeding.*

#### **Figure 4.** *A retinoblastoma of the left eye with tumor scar showing type 3 regression.*

#### **Figure 5.**

*(A) RetCam photograph of class C retinoblastoma of the right eye prior to intra-arterial chemotherapy and (B) RetCam photograph of the right eye showing class C retinoblastoma after treatment with intra-arterial chemotherapy.*

#### *2.6.3.2 Fluorescein angiography*

Thanks to advances in photography as described in the above section, fluorescein angiography (FA) can be readily performed during an EUA and can help one

**Figure 6.**

*(A) RetCam photograph of the left eye showing class D retinoblastoma prior to intra-arterial chemotherapy and (B) RetCam photograph of the left eye showing class D retinoblastoma after intra-arterial chemotherapy.*

differentiate RB from simulating lesions. Firstly, subclinical neovascularization of the iris (NVI) can be distinguished using Retcam FA. In a recent study, eyes with advanced retinoblastoma, NVI was documented appearing as placoid or patchy areas of hyper fluorescence involving one or more sectors of the iris [19]. This finding typically occurred between 1 and 2 min. Retinoblastoma in the fundus should show dilated and tortuous vessels, with retinal arteries which "feed" the tumor of the largest caliber. Also, microaneurysms, retinal hemorrhages, and arteriovenous shunts can also be noted. In the same study, as the tumors enlarged, the abnormal vascularization was no longer consistent with normal retinal anatomy, and were contained entirely within the tumor itself. Intrinsic vessels of the tumor had disorganized and complex branching patterns, irregular caliber, and terminated early within the body of the tumor. This multi-level involvement of vascular abnormality helps the clinician readily distinguish RB from Coat's disease which has large dilated vessels which remain within one level of the retina and show extensive peripheral non-perfusion (**Figure 7A** and **B**). Lastly, after 3 min diffuse leakage from retinal vessels can lead to inability to discern fine details of the fundus, so the clinician should try to obtain all valuable information within the first 3 min of the study.
