The sweet smile of success

In the spring of 2002, with Tricia Coblentz just four months into her second pregnancy, an ultrasound revealed a sizable, solid mass growing from her baby girl's mouth. Scared and uncertain, Tricia and her husband, Matt, turned to Children's Hospital Boston to determine what this meant for their unborn child.

The Coblentz's were referred to the Advanced Fetal Care Center (AFCC), where mom and baby underwent a series of diagnostic tests to establish the cause of the mass. A magnetic resonance imaging (MRI) scan confirmed the diagnosis of a congenital oral teratoma-a rare type of germ cell tumor that can hold serious implications for both the baby and mother.

The couple met with a multidisciplinary team of AFCC specialists, who explained the diagnosis, as well as treatment options for the baby. "The team determined that it was critical for Tricia to carry the baby until she reached a viable stage for delivery. The longer the baby remained in her mother's womb, the greater her chance for survival," says Dr. Reza Rahbar, an otolaryngologist at Children's, who managed much of the baby's care.

The Coblentzes put their faith in the AFCC team, deciding to name their daughter Gracie. "Everyone kept saying that our little girl was in God's grace," says Tricia. "We had every hope she would survive, and we wanted her name to reflect that hope."

A little more than seven months into Tricia's pregnancy, another MRI revealed that the mass had grown, extending down into Gracie's throat and obstructing her airway. Fearing that her windpipe would be blocked if the mass grew any larger, the team performed an ex utero intrapartum treatment (EXIT) procedure just two days later.

"An EXIT procedure is a special method of delivery in which the head and shoulders of the baby are extracted." Explains Rahbar. "The lower body remains in the womb so the baby can continue to receive oxygen through the placenta until the windpipe has been cleared and an effective airway established."

Clinicians usually have up to 45 minutes to create an airway for the baby. However, Tricia's placenta ruptured, leaving Rahbar with only a few moments to perform a tracheotomy-a small incision in the throat to insert breathing tube-so Gracie could breathe on her own.

Gracie was then fully delivered and taken to a separate operating room where the majority of the teratoma was removed from her mouth. She weighed just two pounds, nine ounces following the removal of the mass and remained in Children's Neonatal Intensive Care Unit (NICU) for three months. "Gracie had so much spirit and fight in her," recalls Tricia. "I knew she was a survivor from that very first day."

Gracie has since undergone 11 operations , including removal of the remaining teratoma from her mouth, reconstruction of her windpipe to remove her tracheotomy tube, and repair of her cleft palate, but only minor evidence of her once-serious condition remains. "Gracie has been tremendously successful thanks to the dedicated efforts of countless physicians, nurses, and social workers at Children's," says Rahbar.

Today, the one-year-old is thriving, with smiles for everyone she meets. "Gracie is eating normally and making all kinds of noises," says Tricia. "She is such a happy baby. We can't thank Dr. Rahbar and all the amazing caregivers at Children's enough."

"This is one of those once-in-a-lifetime cases," adds Rahbar. "Gracie truly is a miracle baby." To learn more about Children's Hospital Boston's Advanced Fetal Care Center, please visit www.childrenshospital.org/afcc.