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June, 2003

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The sweet smile of success
AFCC team removes oral teratoma

Miracles do happen—just ask Tricia and Matt Coblentz.

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

Tricia and Matt were referred to the Advanced Fetal Care Center (AFCC), where mom and fetus underwent a series of diagnostic tests to establish the cause of the mass. A magnetic resonance imaging (MRI) scan confirmed the baby's 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 shed some light on 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, as the longer the baby remained in her mother's womb, the greater her chance for survival," says Reza Rahbar, DMD, MD, associate in Otolaryngology, who managed much of the baby's care.

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 completely 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 Dr. 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 Dr. Rahbar with only a few moments to perform a tracheotomy so Gracie could breathe on her own.

She was then fully delivered and taken to a separate operating room where the majority of the teratoma was removed from her mouth. Photographs taken immediately after the delivery show that the mass extending from Gracie's mouth had grown to nearly half the size of her body. Following the removal of the mass, she weighed just two pounds, nine ounces and remained in the Neonatal Intensive Care Unit for three months.

"I only saw Gracie for a moment after she was born," recalls Tricia. "But right away she had so much spirit and fight in her. She was definitely 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 diagnosis remains. "Gracie has been tremendously successful thanks to the dedicated efforts of countless physicians, nurses and social workers," says Dr. Rahbar.

Today, the 1-year-old is thriving, with smiles for everyone she meets. "Gracie is really doing wonderfully," says Tricia. "She is eating normally and making all kinds of noises. 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 Dr. Rahbar. "Gracie truly is a miracle baby."

 


For more information on the Advanced Fetal Care Center, visit www.childrenshospital.org/afcc.