EXIT Procedure for Cervical Teratoma
Smith and Matt Sherrer were shocked when Smith’s 20-week anatomy ultrasound revealed that their second son, Drayton, had a large neck mass. Their obstetrician, Paige Kessler-Johnson, MD, referred them to a local high-risk obstetrician, Mark Hennessy, MD, at the University of Tennessee Medical Center in Knoxville. Hennessy made the diagnosis of a cervical teratoma.
A cervical teratoma is an exceedingly rare tumor of the neck made up of a variety of tissues (nerves, cartilage, skin and thyroid, among others) that come from more than one embryonic layer. Although the tumors are typically benign, if a fetus with a cervical teratoma is delivered by standard delivery, it is at high risk of asphyxiation due to tracheal compression by the tumor. An EXIT procedure is required, which involves partial delivery of the baby and preservation of uteroplacental blood flow, allowing time for the airway to be secured prior to clamping the umbilical cord.
The Sherrers were referred to the Cincinnati Children's Fetal Care Center for Smith and Drayton’s care. At the urging of the Fetal Care Center team, the Sherrers moved to a hospital-appointed short-stay apartment in suburban Cincinnati at approximately 26 weeks’ gestation because of the high likelihood of preterm labor.
For the next month, nearly every day was filled with doctor’s appointments, sonograms and echocardiograms under the guidance of Jeffrey Livingston, MD, then associate director of the Fetal Care Center and maternal-fetal medicine specialist. As expected, Smith developed significant polyhydramnios due to esophageal compression of the cervical teratoma and twice underwent amnioreduction as a result. The teratoma was successfully aspirated, significantly decreasing the size of the cystic component of the mass. Unfortunately, this rapidly reaccumulated as the mass continued to grow.
Drayton’s heart seemed to be tolerating the stress well until late in the 30th week of gestation. A unilateral pleural effusion was noted on ultrasound, and by the next day bilateral pleural effusions were present, as well as ascites, scalp edema and scrotal edema. Livingston quickly made the diagnosis of hydrops fetalis, and the EXIT team was assembled. The family was informed of the grim prognosis of hydrops at 31 weeks, with mortality approaching 98 percent.
Drayton was successfully delivered by EXIT procedure in the fetal surgery unit of the Fetal Care Center on Oct. 28, 2007, by Livingston and Timothy Crombleholme, MD, director of the Fetal Care Center and experienced fetal surgeon. He was orally intubated by Crombleholme and Ravi Elluru, MD. Surfactant was administered, and umbilical arterial and venous cannulas were placed.
Drayton was transported to the Level III newborn intensive care unit at Cincinnati Children’s Hospital Medical Center for ongoing resuscitation. His respiratory distress syndrome resolved as well as his capillary leak phenomenon. On his fifth day of life, Drayton was taken back to the OR and the tumor was resected by Crombleholme and his surgical team. He was extubated in the OR 12 days later.
During the next few months, Drayton’s right hemidiaphragm became significantly elevated, presumably due to phrenic neuropraxia, causing weakness of the diaphragm. Right recurrent laryngeal neuropraxia also led to right vocal cord paralysis. Drayton was re-intubated on three occasions due to respiratory distress and required nasal CPAP for approximately four months postoperatively. Gastroesophageal reflux was found to be a contributing factor, and Nissen fundoplication and gastrostomy tube placement were performed.
After five months in the NICUs in Cincinnati and later at University of Tennessee Medical Center in the Sherrers’ hometown of Knoxville, Drayton was discharged. His mother, Smith, is now his loving nurse, administering medications and formula via his gastrostomy. To date, Drayton has not required a tracheostomy or any reconstructive facial or jaw surgery, both of which are common with cervical teratomas. His parents are thrilled with the cosmetic result. Drayton receives physical therapy and speech therapy at home and is making great strides with feeding therapy. At his six-month follow-up, Drayton requires no supplemental oxygen, his phrenic neuropraxia had resolved, but right vocal cord paralysis persists.
Drayton will require radiographic monitoring and serial physical examination to monitor for tumor recurrence in the form of “benign metastases,” a paradoxical term used to identify lymphatic rests of teratoma. Otherwise, his long-term prognosis is excellent. His mother and father are extraordinarily grateful for the gift that the Fetal Care Center has given them and the excellent care they received in Cincinnati.
Contact the Fetal Care Center
If you would like to request an appointment or get more information about the Cincinnati Children's Fetal Care Center, call us at 1-888-338-2559.
