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Retinal Detachment in a 4 Year Old Boy

Yoshihiro Yonekawa, PGY3 Massachusetts Eye and Ear Infirmary Harvard Medical School

Financial Disclosure
I have no financial interest, affiliation, or other relationships with the manufactures of any commercial products discussed or with the manufactures of any competing commercial products.

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History
Chief Complaint “My eye hurts”
History of Present Illness: A 4 year old boy with a history of Sturge-Weber syndrome presented with 4 days of “pain” of his right eye. He could not verbalize any other symptoms, but his mother indicated that his right eye had been tearing. Review of Systems: Otherwise negative

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History Past Medical History: Sturge-Weber syndrome s/p hemispherectomy for seizures facial hemangioma s/p laser ADHD Developmental delay Asthma Choroidal hemangioma OD Amblyopia OD Hyperopia OD Allergies: None Medications: Dextroamphetamine for ADHD Social History: Lives at home Family History: Unremarkable AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .AAO.

25 sphere) Grossly full Unable to cooperate Round. no APD 15 mm Hg AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.Examination Right Eye Visual acuity (Baseline VA) (Baseline MRx) Extraocular motility Confrontational visual fields Pupils Intraocular pressures Light perception (20/400) (+5.75 sphere) Grossly full Unable to cooperate Round. no APD < 4 mm Hg Left Eye 20/25 (20/40) (+1.ORG .AAO.

External Examination Right facial hemangioma involving the right upper lid AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .AAO.

ORG .Anterior Segment Examination Right Eye Lids/Lashes Conjunctiva Hemangioma of right upper lid Clear and quiet Left Eye Normal Clear and quiet Cornea Anterior Chamber Iris Lens Clear Deep and quiet Round and flat Clear Clear Deep and quiet Round and flat Clear AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.

Examination Under Anesthesia Retrolental retinal tissue.ORG . but not making contact with the lens AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO. close to.

Examination Under Anesthesia Funnel-shaped bullous total exudative retinal detachment extending to the ora AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.ORG .

ORG .AAO.Examination Under Anesthesia Normal left fundus AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.

AAO.B-scan Ultrasonography Diffuse choroidal thickening OD with thickest area 5.8 mm. located temporally Total retinal detachment OD AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .

A-scan Ultrasonography High internal reflectivity AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .AAO.

MRI Details of the eye on the next slide Status post hemispherectomy AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .AAO.

ORG .MRI Vitreous Funnel-shaped retinal detachment Subretinal fluid Mass AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.

ORG .Differential Diagnosis Pediatric exudative retinal detachment Choroidal hemangioma Retinoblastoma Other tumors including: Medulloepithelioma Retinal capillary hemangioma Vascular diseases including: Coats’ disease Familial exudative vitreoretinopthy Predominantly effusive stage 4/5 ROP Inflammatory conditions including: Intermediate uveitis Posterior scleritis Choroiditis Missed rhegmatogenous retinal detachment AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.

Diagnosis Unilateral exudative total retinal detachment Associated with a diffuse choroidal hemangioma In a boy with SturgeWeber syndrome AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .AAO.

AAO. anisometropic amblyopia.Sturge-Weber Syndrome Encephalotrigeminal angiomatosis Etiology Sporadic No underlying genetic defect identified yet The Syndrome Diffuse choroidal hemangiomas Hyperopia.ORG . exudative retinal detachment High internal reflectivity on A-scan Leptomeningeal angiomas Seizures Facial hemangiomas Glaucoma Increased episcleral venous pressure Angle malformation AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.

AAO.ORG AMERICAN ACADEMY OF OPHTHALMOLOGY .Sturge and Weber William Allen Sturge (18501919) British neurologist/pathologist Also was an archeologist with collections in museums Served as Queen Victoria’s physician Frederick Parkes Weber (18631962) British dermatologist Wrote over 1200 papers Also described: Klippel-Trenaunay-Weber syndrome Osler-Weber-Rendu disease Parkes Weber syndrome Weber-Christian disease Weber-Cockayne syndrome WWW.

but the patient has a history of asthma. but may be suboptimal for tumors this diffuse Propranolol: Definitely an option.Treatment Treatment options are limited and prognosis is poor for large detachments External beam radiation: Many radiation side effects Photocoagulation/Photodynamic therapy: This tumor/detachment is too large Brachytherapy: Definitely an option.ORG .AAO. and we have seen patients fail propranolol therapy Anti-VEGF injection: Not safe for this bullous detachment Proton radiotherapy ✓ AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.

after which there is a sudden drop off. to avoid radiation exposure of tissues beyond the target (like the brain/contralateral orbit).Proton Radiotherapy Conventional photon radiation Radiation Dose Proton therapy Tissue Depth The Bragg peak of proton radiation allows the highest radiation doses at the tip of the beam.ORG . AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.

AAO.ORG .Proton Radiotherapy Protons Photons AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.

but increased the fractionation to 10 fractions of 2 Gy(RBE) AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.Concern #1 Radiation in a 4 year old Choroidal hemangiomas have been recently treated with 4 fractions of 3.75 Gy(RBE) when using proton therapy But that resulted in radiation complications for several patients We therefore lowered the dose.AAO.ORG .

we placed the patient under general anesthesia AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.Concern #2 A 4 year old with ADHD and developmental delay cannot maintain fixation for the radiation session To get around this unique issue.ORG .AAO.

how do we control eye positioning? Suction cups! AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.ORG .Concern #3 But then.

Treatment Plan Radiation field Suction cup contact lens to move the eye for proper positioning Lens avoided Tumor AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.ORG .

AAO.Initial presentation 4 months after proton therapy AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .

ORG .6 months Just a sliver of subretinal fluid AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.AAO.

AAO.12 months Completely attached retina AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.ORG .

AAO.4 mm 4 mo 3.0 mm AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.3 mm 12 mo 3.2 mm 7 mo 3.Tumor Response Initial 5.ORG .

ORG .AAO.Follow-up The most recent visual acuity was 20/300 (back to baseline) No recurrence of subretinal fluid No radiation complications AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.

but proton radiotherapy offers an effective option AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.Take Home Points 1 Sturge-Weber patients can have diffuse choroidal hemangiomas that may lead to exudative retinal detachment Treatment of total retinal detachments is challenging.ORG .AAO.

Take Home Points 2 We lowered the dose and increased the fractionation to avoid radiation complications for the 4 year old boy For patients who cannot maintain fixation.AAO.ORG . we devised a technique to perform the treatments under general anesthesia. and using a suction cup contact lens to control the eye position AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.

A clinicopathologic study of 71 cases and a review of the literature. Egger E. Long-term results after low dose ocular irradiation for choroidal haemangiomas.ORG . et al. Yonekawa Y. Zografos L. Font RL. Ophthalmologica 2010. Proton beam irradiation of choroidal hemangiomas. AMERICAN ACADEMY OF OPHTHALMOLOGY WWW.81:267-73. Surv Ophthalmol 1976.20:415-31. Proton beam irradiation using a light-field technique for the treatment of choroidal hemangiomas.224:209-16. Chamot L. Lane AM.126:261-8. Am J Ophthalmol 1998.AAO. Chan RV. Munkel G. Bercher L. et al.References Witschel H. Schilling H. Sauerwein W. Hemangioma of the choroid. Br J Ophthalmol 1997. Lommatzsch A.

D. M.ORG .AAO. M. Pediatric vitreo-retinal surgeon Massachusetts Eye & Ear Infirmary Shannon MacDonald. Pediatric radiation oncologist Massachusetts General Hospital Eugene Shildkrot. Mass Eye & Ear retina fellow ‘10-’12 Currently University of Virginia attending AMERICAN ACADEMY OF OPHTHALMOLOGY WWW. M.Acknowledgements Shizuo Mukai.D.D.

Yoshi is a second year ophthalmology resident at the Massachusetts Eye and Ear Infirmary. AMERICAN ACADEMY OF OPHTHALMOLOGY WWW. and AAO EyeWiki. He has published over 25 papers. M. He hopes to pursue a career in adult and pediatric vitreo-retinal surgery.ORG . and serves as a reviewer for several journals.D. including those from Fight for Sight.AAO. He has received several awards. reviews and book chapters. and appreciates the guidance he has been fortunate to receive from his mentors. ARVO/NEI.Yoshihiro Yonekawa.