Ablasio Retina

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.
AMERICAN ACADEMY OF OPHTHALMOLOGY
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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
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Examination
Right Eye
Visual acuity (Baseline VA) (Baseline MRx) Extraocular motility Confrontational visual fields Pupils Intraocular pressures Light perception (20/400) (+5.25 sphere) Grossly full Unable to cooperate Round, no APD < 4 mm Hg
Left Eye
20/25 (20/40) (+1.75 sphere) Grossly full Unable to cooperate Round, no APD 15 mm Hg
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External Examination
Right facial hemangioma involving the right upper lid
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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
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Examination Under Anesthesia
Retrolental retinal tissue, close to, but not making contact with the lens
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Funnel-shaped bullous total exudative retinal detachment extending to the ora

Normal left fundus

B-scan Ultrasonography
Diffuse choroidal thickening OD with thickest area 5.8 mm, located temporally Total retinal detachment OD
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A-scan Ultrasonography
High internal reflectivity

MRI
Details of the eye on the next slide Status post hemispherectomy
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MRI
Vitreous Funnel-shaped retinal detachment Subretinal fluid Mass
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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

Diagnosis
Unilateral exudative total retinal detachment Associated with a diffuse choroidal hemangioma In a boy with SturgeWeber syndrome
Sturge-Weber Syndrome
Encephalotrigeminal angiomatosis
Etiology Sporadic No underlying genetic defect identified yet The Syndrome Diffuse choroidal hemangiomas
Hyperopia, anisometropic amblyopia, exudative retinal detachment High internal reflectivity on A-scan
Leptomeningeal angiomas
Seizures
Facial hemangiomas Glaucoma

Increased episcleral venous pressure Angle malformation
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Sturge and Weber

William Allen Sturge (18501919)
British neurologist/pathologist Also was an archeologist with collections in museums Served as Queen Victorias 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
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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, but may be suboptimal for tumors this diffuse Propranolol: Definitely an option, but the patient has a history of asthma, and we have seen patients fail propranolol therapy Anti-VEGF injection: Not safe for this bullous detachment Proton radiotherapy
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, after which there is a sudden drop off, to avoid radiation exposure of tissues beyond the target (like the brain/contralateral orbit).
Proton Radiotherapy
Protons Photons
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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, but increased the fractionation to 10 fractions of 2 Gy(RBE)
Concern #2
A 4 year old with ADHD and developmental delay cannot maintain fixation for the radiation session
To get around this unique issue, we placed the patient under general anesthesia
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Concern #3
But then, how do we control eye positioning? Suction cups!
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Treatment Plan
Radiation field
Suction cup contact lens to move the eye for proper positioning
Lens avoided Tumor
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Initial presentation
4 months after proton therapy
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6 months
Just a sliver of subretinal fluid
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12 months
Completely attached retina

Tumor Response
Initial 5.4 mm 4 mo 3.2 mm
7 mo 3.3 mm
12 mo 3.0 mm
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Follow-up
The most recent visual acuity was 20/300 (back to baseline) No recurrence of subretinal fluid No radiation complications
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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, but proton radiotherapy offers an effective option
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, we devised a technique to perform the treatments under general anesthesia, and using a suction cup contact lens to control the eye position
References
Witschel H, Font RL. Hemangioma of the choroid. A clinicopathologic study of 71 cases and a review of the literature. Surv Ophthalmol 1976;20:415-31.
Chan RV, Yonekawa Y, Lane AM, et al. Proton beam irradiation using a light-field technique for the treatment of choroidal hemangiomas. Ophthalmologica 2010;224:209-16. Zografos L, Egger E, Bercher L, Chamot L, Munkel G. Proton beam irradiation of choroidal hemangiomas. Am J Ophthalmol 1998;126:261-8. Schilling H, Sauerwein W, Lommatzsch A, et al. Long-term results after low dose ocular irradiation for choroidal haemangiomas. Br J Ophthalmol 1997;81:267-73.
Acknowledgements
Shizuo Mukai, M.D.
Pediatric vitreo-retinal surgeon Massachusetts Eye & Ear Infirmary
Shannon MacDonald, M.D.

Pediatric radiation oncologist Massachusetts General Hospital
Eugene Shildkrot, M.D.

Mass Eye & Ear retina fellow 10-12 Currently University of Virginia attending
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Yoshihiro Yonekawa, M.D.
Yoshi is a second year ophthalmology resident at the Massachusetts Eye and Ear Infirmary. He has published over 25 papers, reviews and book chapters, and serves as a reviewer for several journals. He has received several awards, including those from Fight for Sight, ARVO/NEI, and AAO EyeWiki. He hopes to pursue a career in adult and pediatric vitreo-retinal surgery, and appreciates the guidance he has been fortunate to receive from his mentors.

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Retinal Detachment in a 4 Year Old Boy

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

Anterior Segment Examination

AMERICAN ACADEMY OF OPHTHALMOLOGY

Examination Under Anesthesia

Examination Under Anesthesia

Funnel-shaped bullous total exudative retinal detachment extending to the ora

Examination Under Anesthesia

Normal left fundus

AMERICAN ACADEMY OF OPHTHALMOLOGY

High internal reflectivity

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

Choroidal hemangioma Retinoblastoma Other tumors including:

Vascular diseases including:

Inflammatory conditions including:

Missed rhegmatogenous retinal detachment

Facial hemangiomas Glaucoma

AMERICAN ACADEMY OF OPHTHALMOLOGY

Sturge and Weber

Frederick Parkes Weber (18631962)

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

Lens avoided Tumor

AMERICAN ACADEMY OF OPHTHALMOLOGY

4 months after proton therapy

AMERICAN ACADEMY OF OPHTHALMOLOGY

Just a sliver of subretinal fluid

AMERICAN ACADEMY OF OPHTHALMOLOGY

Completely attached retina

AMERICAN ACADEMY OF OPHTHALMOLOGY

AMERICAN ACADEMY OF OPHTHALMOLOGY

Take Home Points 1

Take Home Points 2

Shannon MacDonald, M.D.

Eugene Shildkrot, M.D.

AMERICAN ACADEMY OF OPHTHALMOLOGY

Yoshihiro Yonekawa, M.D.

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