title: Thoracic ganglioneuromas resulting in nonimmune hydrops fetalis.
abstract: Introduction Most often, ganglioneuromas affect older pediatric and adult patients. They are typically slow growing tumors that remain clinically silent until they become large enough to cause symptoms by compression of adjacent structures. Case We report a case of a 22-year-old Hispanic gravida 2 para 1 female patient who was found to have massive hydrops fetalis at 20 completed gestational weeks. Fetal echocardiography revealed a narrowed distal ductal arch and proximal descending aorta. Cesarean delivery was undertaken at 29 completed gestational weeks for refractory labor and nonreassuring fetal status. The neonate expired at 47 minutes of life despite aggressive resuscitation. At autopsy, multiple thoracic masses were found adjacent to a compressed proximal descending aorta. Histological and immunohistochemical analysis confirmed the diagnosis of a ganglioneuroma, a rare type of neural crest tumor. Discussion A variety of intrathoracic masses have previously been reported to cause hydrops fetalis including teratomas, fibrosarcomas, and lymphangiomas. To our knowledge, this case is the first description of hydrops fetalis caused by ganglioneuromas. We propose that multiple thoracic ganglioneuromas led to biventricular distal outflow tract obstruction and hydrops fetalis.
authors.affiliation: Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri Kansas City School of Medicine, Kansas City, Missouri.
authors.forename: Paul
authors.initials: P
authors.lastname: Singh
authors.affiliation: Division of Maternal Fetal Medicine and Staff Physician, Department of Obstetrics and Gynecology, Children's Mercy Hospital, Fetal Health Center, University of Missouri Kansas City School of Medicine, Kansas City, Missouri.
authors.forename: Cristiano
authors.initials: C
authors.lastname: Jodicke
authors.affiliation: Division of Pediatric Cardiology, Department of Pediatrics, Children's Mercy Hospital, University of Missouri Kansas City School of Medicine, Kansas City, Missouri.
authors.forename: Tara
authors.initials: T
authors.lastname: Swanson
authors.affiliation: Department of Obstetrics and Gynecology, UMKC School of Medicine, Kansas City, Missouri ; Department of Obstetrics and Gynecology, Truman Medical Center, Kansas City, Missouri ; Department of Maternal Fetal Medicine, Children's Mercy Hospital, Kansas City, Missouri.
authors.forename: Dev
authors.initials: D
authors.lastname: Maulik
keywords: ganglioneuroma; hydrops fetalis; neuroblastoma; thoracic