Treacher Collins syndrome ups risk of dental complications

A 10-year-old Texas boy with a genetic disorder that affects the structure of the face died two days after being anesthetized for a routine dental procedure.

Marcos Tirado received the anesthesia at Harlingen Family Dentistry prior to having a tooth filled, according to a news report by KGBT TV.

Tirado suffered from Treacher Collins syndrome, also known as mandibulofacial dysostosis. His family said they informed the dental team about the boy's condition, and that the staff assured them it was "perfectly safe" for him to be given the anesthesia.

But 10 minutes into the procedure, Tirado stopped breathing and was rushed to a local hospital, according to KGBT TV. Doctors were able to revive him, but he slipped into a coma and died two days later.

In 2005, a 10-year-old Florida girl who also had Treacher Collins syndrome died after being given nitrous oxide prior to a tooth extraction, according to Nicole Cunha, executive director of the Raven Maria Blanco Foundation.

"Treacher Collins is a huge deal that trained pediatric dentists and dental anesthesia providers should be aware of," said James Tom, DDS, an associate clinical professor at the Hermann Ostrow School of Dentistry and secretary of the American Society of Dentist Anesthesiologists. "I would only treat a Treacher Collins child in a hospital setting with a good amount of backup, as getting into trouble with them anesthetically is highly probable. Their airways are so tenuous and compromised because of their small mandibles that even breathing normally is a huge problem for these kids. It is very hard to rescue a child from any sort of respiratory depression when they have Treacher Collins."

Some get an elective tracheostomy so they can breathe normally through a small stoma in their necks, "but even the procedure to place the tracheostomy is complex as there can be complications that can be life-threatening," Dr. Tom added.

A paper published earlier this year in the Journal of Dentistry for Children noted that, for patients with Treacher Collins syndrome, "even simple dental restorative procedures can be challenging" due to associated medical conditions such as congenital heart defects, decreased oropharyngeal airways, hearing loss, and anxiety toward treatment (January-April 2012, Vol. 79:1, pp. 5-21).

"These patients often require a multidisciplinary treatment approach, including: audiology; speech and language pathology; otorhinolaryngology; general dentistry; orthodontics; oral and maxillofacial surgery; and plastic and reconstructive surgeries to improve facial appearance," the authors wrote.