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Chemoreduction for nonvisualized retinoblastoma hidden behind dense cataract
CORRESPONDENCE
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Chemoreduction for nonvisualized retinoblastoma hidden behind dense cataract
A
21-month-old female was noted to have a gray-white reflex in her left eye. A cataract was diagnosed (Fig. 1A) and ocular ultrasonography disclosed an intraocular mass. There was no history of direct ocular trauma, infection, inflammation, or metabolic disorder, but the patient had sustained a fall from bed 4 months previously. On examination, the patient fixed and followed with her right eye, but not with her left. There was 15 prism diopters of left esotropia and a dense intumescent white cataract in the left eye with no view of fundus. The right fundus was normal. Ultrasonography OS revealed a hyperreflective intraocular mass in the nasal quadrant measuring 5 mm in thickness and 8 mm in base, with a few echoes in the overlying vitreous (Figs. 2A, B). Computed tomography disclosed a hyperdense mass nasally with no sign of orbital trauma or bone abnormality (Figs. 2C, D). Magnetic resonance imaging showed an enhancing retinal mass (Figs. 2E, F). The imaging features were consistent with retinoblastoma, astrocytic hamartoma, or foreign body, despite the lack of direct visualization. Whether to proceed with cataract surgery for better visualization or chemoreduction to treat presumed retinoblastoma was discussed. Because surgery in an eye with active retinoblastoma can lead to tumour spread, especially if vitreous seeds escape through an open posterior capsule, the decision was made to initiate chemoreduction prior to cataract surgery. The patient was treated with 1 round of chemoreduction using vincristine, etoposide, and carboplatin.1 The
Fig. 1—(A) A 21-month-old child with unilateral dense cataract and no view of the fundus. (B) Following 1 cycle of chemoreduction and cataract surgery, the regressed retinoblastoma with persistent dilated blood vessels is seen.
thickness of the retinal mass as measured by ultrasonography decreased from 5 mm to 3.5 mm after 1 cycle of chemotherapy. Cataract surgery was subsequently performed successfully, with preservation of the posterior capsule and no intraocular lens implantation. Immediately postoperatively, the left eye showed a partially calcified retinoblastoma superonasal to the optic disc, measuring 3.5 mm in thickness (Fig. 1B). Partially calcified overlying vitreous seeds were noted. Chemoreduction and thermotherapy were continued for 6 months. Isolated cases of cataract in an eye with retinoblastoma have been described in congenital bilateral cataracts, trauma, and zonular necrosis.2 Bindlish and LaRoche2 described an infant with bilateral retinoblastoma in which 1 eye had cataract, and chemotherapy before cataract extraction was employed. Kase et al.3 found that 2 of 20 patients with unilateral, newly diagnosed retinoblastoma managed with enucleation demonstrated cataract (posterior subcapsular and anterior polar cataract). Both eyes had prominent cytoplasmic immunoreactivity for transforming growth factor–beta (TGF-b) in retinoblastoma cells, whereas the remainder of the globes showed minimal expression of TGF-b. They speculated that TGF-b produced in retinoblastoma cells might induce cataract formation,3 suggesting that cataract in an eye with retinoblastoma could be related and not coincidental.
Fig. 2—Imaging studies showing hyperreflective intraocular mass. (A) B-scan and (B) A-scan ultrasonography. Coronal computed tomography using (C) bone window and (D) soft-tissue window. Axial magnetic resonance imaging using (E) T1-weighted image showing a bright-signal intraocular mass and (F) T2-weighted image showing a low-signal mass. CAN J OPHTHALMOL—VOL. 45, NO. 5, 2010
539
Correspondence Honavar et al.4 demonstrated that the approach to cataract surgery, intraocular lens placement, and posterior capsule status did not seem to impact ocular or systemic outcome in patients with a mean interval of 26 months from completion of treatment to time of intraocular surgery.4 However, violation of the posterior capsule and disturbance of the vitreous is inadvisable in patients with partially treated tumors or vitreous seeds, such as ours. In our case, the cataract surgery was performed early in the chemoreduction course so that appropriate consolidation with thermotherapy could be performed. This case demonstrates the importance of fundus examination and ultrasonography in a child with cataract. Confirmation of suspected retinoblastoma with computed tomography and magnetic resonance imaging was employed. The occurrence of cataract could be attributed to either the history of fall or the presence of retinoblastoma. We suggest a conservative approach, with treatment of presumed tumor prior to proceeding with cataract surgery. REFERENCES 1. Shields CL, Mashayekhi A, Au AK, et al. The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology 2006;113:2276–80. 2. Bindlish R, LaRoche GR. Successful treatment of neonatal trilateral retinoblastoma. J AAPOS 1999;3:376–8. 3. Kase S, Parikh JG, Youssef PN, et al. Transforming growth factor ß in retinoblastoma-related cataract. Arch Ophthalmol 2008;126:1539–42. 4. Honavar SG, Shields CL, Shields JA, Demrci H, Naduvilath TJ. Intraocular surgery after treatment of retinoblastoma. Arch Ophthalmol. 2001;119:1613–21.
Sudha Nallasamy, Aparna Ramasubramanian, Carol L. Shields, Richard Tipperman, Jerry A. Shields Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pa. Correspondence to Carol L. Shields, MD: [email protected] Can J Ophthalmol 2010;45:539–40 doi:10.3129/i10-025
540
CAN J OPHTHALMOL—VOL. 45, NO. 5, 2010
––––––––––––––––––––––––––––––––
Chemoreduction for nonvisualized retinoblastoma hidden behind dense cataract
A
21-month-old female was noted to have a gray-white reflex in her left eye. A cataract was diagnosed (Fig. 1A) and ocular ultrasonography disclosed an intraocular mass. There was no history of direct ocular trauma, infection, inflammation, or metabolic disorder, but the patient had sustained a fall from bed 4 months previously. On examination, the patient fixed and followed with her right eye, but not with her left. There was 15 prism diopters of left esotropia and a dense intumescent white cataract in the left eye with no view of fundus. The right fundus was normal. Ultrasonography OS revealed a hyperreflective intraocular mass in the nasal quadrant measuring 5 mm in thickness and 8 mm in base, with a few echoes in the overlying vitreous (Figs. 2A, B). Computed tomography disclosed a hyperdense mass nasally with no sign of orbital trauma or bone abnormality (Figs. 2C, D). Magnetic resonance imaging showed an enhancing retinal mass (Figs. 2E, F). The imaging features were consistent with retinoblastoma, astrocytic hamartoma, or foreign body, despite the lack of direct visualization. Whether to proceed with cataract surgery for better visualization or chemoreduction to treat presumed retinoblastoma was discussed. Because surgery in an eye with active retinoblastoma can lead to tumour spread, especially if vitreous seeds escape through an open posterior capsule, the decision was made to initiate chemoreduction prior to cataract surgery. The patient was treated with 1 round of chemoreduction using vincristine, etoposide, and carboplatin.1 The
Fig. 1—(A) A 21-month-old child with unilateral dense cataract and no view of the fundus. (B) Following 1 cycle of chemoreduction and cataract surgery, the regressed retinoblastoma with persistent dilated blood vessels is seen.
thickness of the retinal mass as measured by ultrasonography decreased from 5 mm to 3.5 mm after 1 cycle of chemotherapy. Cataract surgery was subsequently performed successfully, with preservation of the posterior capsule and no intraocular lens implantation. Immediately postoperatively, the left eye showed a partially calcified retinoblastoma superonasal to the optic disc, measuring 3.5 mm in thickness (Fig. 1B). Partially calcified overlying vitreous seeds were noted. Chemoreduction and thermotherapy were continued for 6 months. Isolated cases of cataract in an eye with retinoblastoma have been described in congenital bilateral cataracts, trauma, and zonular necrosis.2 Bindlish and LaRoche2 described an infant with bilateral retinoblastoma in which 1 eye had cataract, and chemotherapy before cataract extraction was employed. Kase et al.3 found that 2 of 20 patients with unilateral, newly diagnosed retinoblastoma managed with enucleation demonstrated cataract (posterior subcapsular and anterior polar cataract). Both eyes had prominent cytoplasmic immunoreactivity for transforming growth factor–beta (TGF-b) in retinoblastoma cells, whereas the remainder of the globes showed minimal expression of TGF-b. They speculated that TGF-b produced in retinoblastoma cells might induce cataract formation,3 suggesting that cataract in an eye with retinoblastoma could be related and not coincidental.
Fig. 2—Imaging studies showing hyperreflective intraocular mass. (A) B-scan and (B) A-scan ultrasonography. Coronal computed tomography using (C) bone window and (D) soft-tissue window. Axial magnetic resonance imaging using (E) T1-weighted image showing a bright-signal intraocular mass and (F) T2-weighted image showing a low-signal mass. CAN J OPHTHALMOL—VOL. 45, NO. 5, 2010
539
Correspondence Honavar et al.4 demonstrated that the approach to cataract surgery, intraocular lens placement, and posterior capsule status did not seem to impact ocular or systemic outcome in patients with a mean interval of 26 months from completion of treatment to time of intraocular surgery.4 However, violation of the posterior capsule and disturbance of the vitreous is inadvisable in patients with partially treated tumors or vitreous seeds, such as ours. In our case, the cataract surgery was performed early in the chemoreduction course so that appropriate consolidation with thermotherapy could be performed. This case demonstrates the importance of fundus examination and ultrasonography in a child with cataract. Confirmation of suspected retinoblastoma with computed tomography and magnetic resonance imaging was employed. The occurrence of cataract could be attributed to either the history of fall or the presence of retinoblastoma. We suggest a conservative approach, with treatment of presumed tumor prior to proceeding with cataract surgery. REFERENCES 1. Shields CL, Mashayekhi A, Au AK, et al. The International Classification of Retinoblastoma predicts chemoreduction success. Ophthalmology 2006;113:2276–80. 2. Bindlish R, LaRoche GR. Successful treatment of neonatal trilateral retinoblastoma. J AAPOS 1999;3:376–8. 3. Kase S, Parikh JG, Youssef PN, et al. Transforming growth factor ß in retinoblastoma-related cataract. Arch Ophthalmol 2008;126:1539–42. 4. Honavar SG, Shields CL, Shields JA, Demrci H, Naduvilath TJ. Intraocular surgery after treatment of retinoblastoma. Arch Ophthalmol. 2001;119:1613–21.
Sudha Nallasamy, Aparna Ramasubramanian, Carol L. Shields, Richard Tipperman, Jerry A. Shields Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pa. Correspondence to Carol L. Shields, MD: [email protected] Can J Ophthalmol 2010;45:539–40 doi:10.3129/i10-025
540
CAN J OPHTHALMOL—VOL. 45, NO. 5, 2010