ABSTRACT
Primary idiopathic chylopericardium is an extremely rare condition especially in children and young adults. Although the exact pathophysiology of primary chylopericardium has not been established, the reflux of chylous fluid into the pericardial space was suggested as the etiology. Damage to the thoracic duct valves and the communication of the thoracic duct to the pericardial lymphatics or abnormally elevated pressure in the thoracic duct could cause chylous fluid reflux. In this report, we described the case of a 4-year-old boy with primary idiopathic chylopericardium presenting as cardiac tamponade who was treated with video-assisted thoracoscopic window and then surgical duct ligation.
Subject(s)
Cardiac Tamponade/diagnosis , Pericardial Effusion/diagnosis , Cardiac Tamponade/surgery , Child, Preschool , Echocardiography , Humans , Ligation , Male , Pericardial Effusion/surgery , Pericardiocentesis , Recurrence , Thoracic Duct/surgerySubject(s)
Cardiac Catheterization/methods , Ductus Arteriosus, Patent/complications , Ductus Arteriosus, Patent/surgery , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/surgery , Plastic Surgery Procedures/methods , Child , Ductus Arteriosus, Patent/diagnosis , Female , Humans , Hypertension, Pulmonary/diagnosis , Treatment OutcomeABSTRACT
Arterial tortuosity syndrome (ATS; OMIM 208050) is a rare autosomal recessive condition characterized by dysmorphic features, elongation, tortuosity, and aneurysm of the large and middle sized arteries. We report on a 13-year-old boy who presented with a malformed ascending aorta mimicking coarctation of aorta and a cutis laxa-like facial dysmorphia. Based on angiogram, a diagnosis of ATS was made and subsequently confirmed by a homozygous one base-pair deletion at position g.318 of SLCA10. We stress similarities (facial appearance, inguinal herniae, ..) between ATS and autosomal recessive cutis laxa, both being connective tissue disorders disorganizing the elastin network.
Subject(s)
Aorta, Thoracic/abnormalities , Joint Instability/diagnosis , Skin Diseases, Genetic/diagnosis , Vascular Malformations/diagnosis , Abnormalities, Multiple/diagnosis , Abnormalities, Multiple/genetics , Adolescent , Aorta, Thoracic/pathology , Aortography/methods , Arteries/abnormalities , Diagnosis, Differential , Facies , Genetic Predisposition to Disease/genetics , Glucose Transport Proteins, Facilitative/genetics , Humans , Joint Instability/genetics , Magnetic Resonance Imaging/methods , Male , Skin Diseases, Genetic/genetics , Vascular Malformations/geneticsABSTRACT
A 3-month-old girl presented with myocarditis owing to brucellosis. Her mother had been diagnosed with brucellosis at 28 weeks gestation but did not receive treatment until after delivery. The infant had intrauterine retardation and had gained little weight since birth. It is considered likely that the brucellosis was transmitted transplacentally.
