Tiffany L. Kruger, D.O. Children s Hospital of Michigan Wayne State University/Kresge Eye Institute

Transcription

1 Pediatric Cases Nt Not To Be Missed Tiffany L. Kruger, D.O. Pediatric Ophthalmology Fellow Children s Hospital of Michigan Wayne State University/Kresge Eye Institute

2 Case Presentation CC: Left eye turns in and eyes don t move together HPI 9-month old healthy h female, fraternal twin A Parents and pediatrician noticed left eye crossing since 3 months old Pediatrician will watch at interval well-child visits Now others are seeing eye cross, mom requests ophthalmology consult Twin B no signs/symptoms of pathology ROS/PMH Negative ROS, no developmental or growth delays, 6 lbs, 1 oz at birth, 38 w gestation, uncomplicated (requested) C-section; no medications; NKDA FH Mother myopic Aunt lazy eye

3 Physical Exam VA: CSM OU with vertical prism IOP: STP OU RAPD: Negative OU Pupils dim: 4mm OU, light: 2mm OU Motility: EX = 0 (PACT), appears ET EOM: Full OU (After dilation, LET 8pd) PLE: Wide nasal bridge with epicanthal folds OU, otherwise normal Dilated Posterior Exam: Fundus OD poor view, but appears normal Fundus OS retinal/subretinal white lesion including macula and extending temporally, satellite lesion above nerve, possible calcific changes over retinal lesions CRx OD: x 090 OS: x unsure of axis, difficult reflex

4 I/P: Probable Retinoblastoma, follow-up EUA with Rb specialist in 1 day

5 Alignment Normals Conjugate horizontal gaze: birth Eyes appear straight around 2-3 months old Accommodation and fusional convergence: 3 months old Vertical gaze fully functional by 6 months old

6 Retinoblastoma Modified from Abramson, DH et al.

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8 Treatment Options Intravenous chemotherapy Intra-arterial chemotherapy Intravitreal chemotherapy Sunconjunctival chemotherapy Sub-Tenon s chemotherapy Thermotherapy Cryotherapy Focal laser photocoagulation Plaque radiotherapy Enucleation External beam radiation

9 International Classification of Retinoblastoma A: Small intraretinal tumors (< 3mm) away from foveola and disc B: Tumors > 3mm, macular or juxtapapillary location, or with subretinal fluid C: Tumor with focal subretinal or vitreous seeding within 3mm of tumor D: Tumor with diffuse subretinal or vitreous seeding > 3mm from tumor E: Extensive retinoblastoma occupying >50% of the globe with or without neovascular glaucoma, hemorrhage, extension of tumor to optic nerve or anterior chamber

10 Treatment Chemoreduction (typically Vincristine, i Carboplatin, and Etoposide) Most preferred method 85% globe salvage in Reese Ellsworth groups I-IV, 47% in group V Complications: Beware of side effects: Etoposide AML, optic neuritis Carboplatin ototoxicity Recurrence of vitreous/subretinal seeds

11 Treatment Laser photocoagulation small tumors; can seed if direct treatment Cryotherapy small tumors, especially for subretinal seeds near ora; fails if overlying vitreous seeds Thermotherapy synergistic effect, small or large tumors, tumors adjacent to fovea or nerve; v; tedious Plaque radiotherapy <16mm base x 8mm thick, primary or secondary treatment; maculopathy in 25% by 5 years

12 Treatment External beam radiation vitreous seeds, advanced tumor presentation Complications: recurrence usually within 4 years, formation of secondary cancers, amblyopia 35% incidence of secondary tumor formation in 30 years in bilateral cases Greater risk of secondary cancers if <1 year old Least preferred treatment Enucleation advanced or group E retinoblastoma, risk of orbital invasion, vitreous seeding, AC invasion Complications: small risk of recurrent orbital tumor, orbit and prosthetic management

13 Treatment Intravitreal Melphalan: January 2013, FDA approved intraocular injection for retinoblastoma; good results in cases of vitreous seeding (recurrent or resistant) Ocular survival rate at 2 years, 84%* All cases in remission at 22 months* Complications: Risk of vitreous seeding or extraocular spread Multiple injections Vitreous hemorrhage, retinal vasculitis, RPE changes *N=23 eyes

14 Intra-arterial arterial Chemotherapy

15 Treatment Primary therapy, including advanced disease (groups D and E) Bilateral cases 80.5% ocular survival at 2 years, groups D & E Recurrent tumors Complications: Vascular obstruction Choroidal atrophy Side effects of chemotherapy agents

16 Before Our patient t After 3 sessions

17 p g Shields Treatment Cocktail 10 Unilateral: consider intra-arterial chemotherapy (IAC) or chemoreduction Unilateral groups D and E: globe-conserving chemoreduction and/or IAC or enucleation Intravitreal chemotherapy is reserved for recurrent vitreous seeds following other therapies Bilateral: l chemoreduction Bilateral groups D and E: additional subtenons carboplatin for local l control

18 Summary Control of alignment occurs between 3 6 months old 20% of retinoblastoma cases present with strabismus Don t ignore recurrent anterior uveitis in older children Many yglobe salvage therapies are being used with great results Before After 6 sessions

19 References 1. FDA clears orphan retinoblastoma drug. One Network. Date: January 03, Abramson DH, Frank CM, Susman M, et al. Presenting signs of retinoblastoma. Journal of Pediatrics 1998; 132(3 Pt 1): Shields, CL and Shields, JA. Diagnosis and Management of Retinoblastoma. Cancer Control 2004; 11(5): BCSC: Pediatric Ophthalmology and Strabismus, Section 6. Retinoblastoma : , Shields CL, Shields JA, Cater J, et al. Development of new retinoblastomas after 6 cycles of chemoreduction for retinoblastoma in 162 eyes of 106 consecutive patients. Archives of Ophthalmology 2003;121: Heo JW, et al. Inhibitory Activity of Bevacizumab to Dff Differentiation of fretinoblastoma Cells. Pbl Public Library of Science One 2012; 7(3): e doi: /journal.pone Ramasubramanian, A. and Shields, CL. Retinoblastoma. Jaypee-Highlights Medical Publishers. New Delhi, India; Shields CL, et al. Retinoblastoma frontiers with intravenous, intra-arterial, periocular, and intravitreal chemotherapy. Eye 27, February 2013 doi: /eye /y