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Kelly Nordin was pregnant with her third child when an ultrasound revealed her baby had a congenital pulmonary airway malformation / CPAM. To her and husband Lewis’ great disappointment, their doctors were pessimistic that anything could be done to help the baby. Rather than accepting this prognosis, Kelly and Lewis began researching their options and, after researching several institutions, decided that the Cincinnati Fetal Center gave their baby the best chance for survival.
CPAM (until recently, the condition was termed congenital cystic adenomatoid malformation – CCAM) is a rare pulmonary maldevelopment that is usually restricted to one lobe of the lung. It typically appears on an ultrasound as a solid or cystic mass of pulmonary tissue that has an abundance of bronchial structures.
The indications for fetal intervention depend on the type of lesion, whether solid or cystic, and the development of hydrops. In some cases, open fetal surgery may be indicated when patients present with a solid CPAM with associated hydrops. Cases in which the malformations are large but have not resulted in hydrops may require removal during an EXIT (ex utero intrapartum treatment) procedure at the time of delivery.
The best prognostic indicator for a CPAM is the CPAM volume ratio (CVR). The CVR is a calculated volume based on ultrasound measurements obtained in three dimensions of the mass at presentation. The volume is then divided by the head circumference so the CPAM volume is corrected for gestational age.
If the CVR is < 1.6, this is a CPAM with a favorable prognosis. The risk of developing hydrops is less than 2 percent in these cases. A CPAM with a CVR > 1.6 shows a higher risk for the development of hydrops and fetal demise, which occurs in up to 80 percent of cases in this category. Such malformations should be followed with twice-weekly ultrasound scans so that fetal surgery can be undertaken at the earliest signs of hydrops.
Kelly and Lewis Nordin came to the Fetal Center for Kelly’s initial consultation on April 9, 2008. Doctors at the Fetal Center, led by Timothy Crombleholme, MD, FAACS, FAAP, diagnosed their baby − who would be named Joshua − with a CPAM of the left upper lobe with a CVR of 1.66. Kelly responded well to an initial steroid treatment and was able to return home to Pennsylvania, where she was monitored weekly by ultrasound.
At 32 weeks gestation, however, Joshua’s CVR was up to 1.8 and, a week later, Kelly relocated to Cincinnati for the remainder of her pregnancy. Because the CPAM was found to be high risk, Kelly received an additional course of the steroid betamethasone. Remarkably, the growth of the CPAM stabilized, and Joshua began growing around the lung mass. Kelly was closely monitored by weekly ultrasounds to follow the growth of the CPAM. With steroids and time, the mediastinal shift caused by the CPAM improved and, because the intrathoracic tracheal compression had improved, an EXIT-to-resection was not necessary.
Joshua was born on July 3, 2008, at 38 weeks’ gestation, weighing 2.795 kilos. Joshua had significant pulmonary hypertension requiring treatment with sildenafil. Treating the pulmonary hypertension enabled Crombleholme to successfully resect Joshua’s CPAM. Joshua was discharged within 48 hours of surgery, and the Nordins returned home. Joshua continues to thrive and is being weaned off sildenafil.
If you would like to request an appointment or get more information about the Cincinnati Fetal Center, call us at 1-888-338-2559 (1-888-FETAL59).
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