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Bright Futures

What You Need to Know About Pediatric Retinoblastoma

May 12, 2020, Aparna Ramasubramanian
What You Need to Know About Pediatric Retinoblastoma

This week is World Retinoblastoma Week, which gives us a great opportunity to raise awareness of this type of cancer and to highlight the importance of early detection and treatment on a global scale and right here in Arizona.

Retinoblastoma is a rare type of eye cancer that usually develops in early childhood, typically before the age of 5. It is diagnosed in 250-350 children each year in the United States and makes up about 4% of all cancers in children under 15. While retinoblastoma was once quite deadly, medical advances have dramatically improved outcomes. Now, the five-year survival rate for children with the disease is higher than 95%.

Retinoblastoma develops in the retina, the specialized, light-sensitive tissue at the back of the eye that detects light and color. The disease most often affects only one eye. However, one out of three children will develop it in both eyes.

Signs & Symptoms

The most common first sign of retinoblastoma is a visible whiteness in the pupil called “cat’s eye reflex” or leukocoria. This unusual whiteness is particularly noticeable in dim light or in photographs taken with a flash. Upon examination of the eye, the retina of the eye appears to have a whitish elevated mass.

Other signs and symptoms include crossed eyes or eyes that do not point in the same direction, which can cause squinting; a change in the color of the colored part of the eye; redness, soreness, or swelling of the eyelids; and blindness or poor vision in the affected eye or eyes.

Phoenix Children’s Becomes Retinoblastoma ‘Center of Excellence’

Recognizing the opportunity to expand and enhance the care we provide to children with cancer and to continue leading and pioneering in pediatric specialty care, Phoenix Children’s became a destination medical center with the hire of Aparna Ramasubramanian, MD, as medical director of our retinoblastoma program. Highly sought-after as an expert in ocular oncology in the United States, Dr. Ramasubramanian offers highly unique expertise as a dual fellowship-trained physician in both pediatric ophthalmology and ocular oncology.

We offer individualized care plans customized for each patient. This is important, as the progression of the disease and the needs of each child are different. Our goals of treatment are first to save the child’s life, second to save their eyes, and third to preserve and maximize their vision. Our multidisciplinary team of pediatric specialists including an ocular oncologist (ophthalmologist), pediatric oncologist, geneticist, radiation oncologist, interventional radiologist and other support staff offer state-of-the-art diagnoses and a tailored mix of treatments, including:

  • Pre-implantation genetic diagnosis: Because retinoblastoma is a genetic disorder, Phoenix Children’s Genetics & Metabolism Department offers pre-conception genetic counseling and screening.
  • Prenatal diagnosis: Our special obstetric ultrasound can identify retinoblastoma before a baby is born.
  • Secondary cancer screening: Retinoblastoma increases the risk of other cancers. At Phoenix Children’s, we offer streamlined cancer surveillance for adolescents and adults.
  • Intra-arterial chemotherapy: This specialized procedure means chemotherapy is given only to the artery supplying the eye, minimizing any toxicity to the rest of the body.
  • Intra-vitreal chemotherapy: Medication is injected directly in the eye.

We encourage you to “Know the Glow” because early detection and treatment greatly improve a child’s prognosis and can help us save lives and eyes.

For more information or to schedule an appointment at Phoenix Children’s, please call (602) 933-EYES (3937), or visit us at: http://www.phoenixchildrens.org/centers-programs/ophthalmology.

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