ORBIS International, a non-profit humanitarian organization, teaches surgical techniques for retinoblastoma in developing countries. Retinoblastoma is a cancerous tumor of the eye. The tumor is located within the retina — the light-sensitive tissue located in the back of the eye. Retinoblastoma is passed on as a dominant hereditary condition or results from a spontaneous genetic mutation occurring on its own.

Retinoblastoma is usually apparent in children by the time they’re 5 years old, although it’s most often diagnosed within the first year or two. If the disease is not treated promptly and effectively, it results in death in nearly every case.

Sol Mendoza Corrales was diagnosed with retinoblast- oma using an ORBIS-donated  retinal camera.

Signs of retinoblastoma include:

• A white dot filling the pupil in flash photography
• A white spot in the pupil in ordinary illumination
• One eye being a different color than the other
• Misaligned or crossed eyes
• Red, painful eyes
• Poor vision

The cure rate for retinoblastoma that is diagnosed early can be over 90 percent. For those who aren’t diagnosed and treated early, though, the disease is usually fatal. 

How common is retinoblastoma?

Although retinoblastoma is relatively rare, comprising of 2-3 percent of all childhood cancers, it is the most common eye cancer in children. It can affect only one eye, although nearly half the time it affects both. Retinoblastoma can spread, and in fatal cases that is usually the case.

Risk factors in developing countries

The toll of retinoblastoma is greater in developing countries than it is in industrialized nations, especially in areas where marriage between blood relatives commonly occurs. In developing countries, successful treatment is more difficult to accomplish for several reasons, among them delayed diagnosis, inadequate treatment facilities, financial barriers and a lack of trained doctors.

Because children in developing countries are typically diagnosed at a later stage of the disease, tumors have often already spread outside the eye. As treatment entails a multi-stage approach, many families cannot see treatment through to completion. Fatality rates in developing countries may be 60 percent or higher, whereas in the United States the death rate has been reported to be as low as 1 or 2 percent.

Prevention of retinoblastoma

No preventive measures can be taken to avoid retinoblastoma. However, with advanced training in pediatric ophthalmology, doctors can save the sight — and the lives — of many children with this disease.  

Treatment of retinoblastoma

Because retinoblastoma is a type of cancer, many of the standard cancer therapies are employed when treating it. These include:

• Surgery to remove a tumor, including the entire eyeball
• Laser therapy to destroy abnormal tissue by burning it or by sealing off blood vessels that feed the tumor
• Cryotherapy, which uses a small probe to freeze and kill the abnormal tissue
• Chemotherapy, which uses drugs to reduce the size of tumors and in some cases to kill them entirely, to halt the spread of cancer cells, and to improve the effectiveness of other treatment
• Radiation to kill cancer cells

If eyesight has not been severely affected by the retinoblastoma, doctors will try to destroy the cancer without removing the eyeball (enucleation). If vision loss is severe or complete, and the tumor is large, doctors will usually remove the eye and part of the optic nerve behind the eye. Enucleation carried out when the cancer has not spread beyond that eye will cure retinoblastoma.

What ORBIS is doing about retinoblastoma in developing countries

In 2003, ORBIS partnered with St. Jude Children’s Research Hospital and the University of Tennessee to initiate a retinoblastoma diagnosis, treatment and training program in Guatemala City. This program serves patients from all over Central America and several clinics worldwide, where the fatality rate for retinoblastoma had been said to be approximately 60 percent.

Through this partnership, ORBIS has:

• Trained pediatric ophthalmologists in diagnosing and treating retinoblastoma
• Provided ongoing consultation for patient care through ORBIS telemedicine (Cyber-Sight)
• Supplied a retinal digital camera for recording retinoblastoma tumors
• Supplied cryotherapy and laser treatment equipment to doctors in Guatemala City
• Helped the Guatemala City facility receive radiation equipment and chemotherapy drugs through St. Jude Children’s Research Hospital

Through Cyber-Sight, ORBIS’s telemedicine program, ORBIS volunteer medical faculty have provided more than 320 retinoblastoma consultations for the Guatemala City program as well as for several other facilities, including the King Hussein Hospital in Amman, Jordan.

ORBIS’s work in retinoblastoma is part of its overall strategy to reduce the incidence of childhood blindness. ORBIS has shown through this program that with the proper training and equipment, the lives of patients with retinoblastoma can be saved and their eyesight maintained in developing countries.