Nuchal cystic hygroma associated with massive lymphangiomatosis of the upper extremity and myocardium in a stillborn fetus: a case report.
Nuchal cystic hygromas may be encountered in stillborn fetuses as part of a spectrum of a more generalized abnormality of the lymphatic system. These lymphatic system abnormalities may be accompanied by various visceral anomalies, especially in Ullrich-Turner Syndrome (UTS) (45,X0) but also in non-45,XO fetuses. We present unique autopsy findings in the case of a stillborn female fetus with a nuchal cystic hygroma associated with massive lymphangiomatosis of the upper extremity as well as myocardium. While previous descriptions of abnormal cardiac lymphatics associated with nuchal cystic hygroma have focused on dilated lymphatics surrounding the root of the aorta and pulmonary artery, the present case extends the spectrum of possible findings to include subtotal replacement of the ventricular myocardium by dilated lymphatics.
Walker
W P
WP
Department of Surgical Pathology, University of Iowa Hospitals and Clinics, Iowa City.
Landas
S K
SK
D006332:Cardiomegaly; D005260:Female; D005313:Fetal Death; D006258:Head and Neck Neoplasms / Q000473:pathology*; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D007231:Infant, Newborn; D008202:Lymphangioma / Q000473:pathology*; D008208:Lymphatic System / Q000002:abnormalities; D008210:Lymphocele / Q000473:pathology; D009206:Myocardium / Q000473:pathology; D011247:Pregnancy