Rare Eye Muscle Syndrome Treated
Kellogg Expert Takes Eye Care to Guatemala
Among the many surprising moments for Monte Del Monte, M.D., on a recent trip to Guatemala was his encounter with a family who had a rare hereditary syndrome. Dr. Del Monte, the Skillman Professor of Pediatric Ophthalmology at the U-M Kellogg Eye Center, traveled under the auspices of the World Eye Mission for a week of teaching, examining patients, and performing strabismus surgery to correct vision problems related to abnormal eye alignment. He visited two hospitals in Guatemala City and one in the Petén, a remote jungle region of northern Guatemala.
The family had a hereditary syndrome known as congenital fibrosis of the extraocular muscles type 1. The mother and son had severely limited visual function due to restrictive strabismus, in which the already misaligned eyes have such limited movement that affected individuals must assume abnormal and uncomfortable head positions in order to see. Because corrective surgery is complex and not available in Central America, the family accompanied Dr. Del Monte to the Petén, where he performed the complicated procedure. The surgery was successful in establishing eye alignment and in improving the range of eye movement—and thus vision—in both patients.
In a fortuitous coincidence, Dr. Del Monte has been involved with a research project that isolated the gene for one form of the syndrome. He sent blood samples to the laboratory of his long-time collaborator Dr. Elizabeth Engle at Harvard Medical School. If the gene affecting this family can be identified, Dr. Del Monte will have contributed useful knowledge about a region where little is known about genetic patterns of this disorder.
The entire trip was, according to Dr. Del Monte, "not only gratifying but enlightening." At the Visualiza Clinic in Guatemala City, Dr. Del Monte examined 15–20 clinic patients, and performed or assisted in half a dozen eye muscle procedures in a surgical suite within the clinic.
At his second stop in Guatemala City, Roosevelt Hospital, Dr. Del Monte spent a great deal of time working with the local residents. They presented complicated cases at a special Grand Rounds and also assisted Dr. Del Monte in the surgical procedures performed at that hospital. In the evenings, Dr. Del Monte led teaching conferences and presented several lectures each night, which often led to lengthy question-and-answer sessions, especially concerning those procedures that were new to this part of the world.
After leaving Guatemala City, Dr. Del Monte traveled to the remote northern province of Petén to visit the state-of- the-art Vincent Pescatore Eye Clinic, a unique clinical model for developing countries. The clinic has two fee structures: the revenue from "private" patients helps fund care for "social" patients who are unable to pay. The care, of course, is the same. This model is being duplicated in other countries in Central America, including Nicaragua and El Salvador.
At the Pescatore Clinic, Dr. Del Monte examined strabismus patients, children and adults, and performed surgeries assisted by one of his hosts, Dr. Edwin Arias. Despite being the only eye clinic in this poor jungle area, the rural Pescatore Eye Clinic was observed by Dr. Del Monte to be "well equipped with most modern instruments and supplies so I needed to bring only a few specialized instruments and sutures." He added, "This clinic really did have almost anything you would need."
Board members of the World Eye Mission were instrumental in arranging Dr. Del Monte's travels. They are President Jeevak Lal, M.D., an Albion, Michigan ophthalmologist and Bartley R. Frueh, M.D., eye plastics specialist at the Kellogg Eye Center.
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