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AJP Rep 2014; 4(01): 049-054
DOI: 10.1055/s-0034-1371751
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Thoracic Ganglioneuromas Resulting in Nonimmune Hydrops Fetalis

Paul Singh
1   Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, University of Missouri Kansas City School of Medicine, Kansas City, Missouri
,
Cristiano Jodicke
2   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
,
Tara Swanson
3   Division of Pediatric Cardiology, Department of Pediatrics, Children's Mercy Hospital, University of Missouri Kansas City School of Medicine, Kansas City, Missouri
,
Dev Maulik
4   Department of Obstetrics and Gynecology, UMKC School of Medicine, Kansas City, Missouri
5   Department of Obstetrics and Gynecology, Truman Medical Center, Kansas City, Missouri
6   Department of Maternal Fetal Medicine, Children's Mercy Hospital, Kansas City, Missouri
› Author Affiliations
Further Information

Publication History

30 November 2013

20 January 2014

Publication Date:
15 April 2014 (online)

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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.

Keywords

ganglioneuroma - hydrops fetalis - thoracic - neuroblastoma
 

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